Hematology

Anatomy and Physiology

• Blood – transports cellular requirements and products from one part of the body to another; composed of plasma (55%) and cellular component (45%)– slightly alkaline (ph 7.35-7.4)– 5-6 liters or 70-75 ml/kg BW (average

volume)• Pulmonary circulation = 1300 cc

– arterial (400cc) + capillary (60cc) + venous (840cc)

• Systemic circulation = 3000 cc– arterial (550cc) + capillary (300cc) +

venous (2150cc)

• Hematopoiesis – blood cell production; done in the bone marrow (red), pelvis, sternum, ribs, epiphysis of long bones

• Erythropoiesis – red blood cell production in the liver in utero (2 to 5 months old) then in bone marrow.– needs iron, protein, pyridoxine (B6),

cyanocobalamine (B12), folic acid, and copper

• Reticuloendothilial System – mononuclear phagocyte system or macrophage (spleen, liver, lymphatic system, lungs)

Components

PLASMA(55%)

FormedElements

(45%)

Proteins 7%

Water 91%

Other solutes2%

Plasma (% by weight)

Formed Elements

(number per mm3)

Platelets250-400 thou

Erythrocytes2-5.8 million

Leukocytes5-9 thou

Albumin58%

Globulin38%

Fibrinogen4%

IonsNutrients

Waste ProductsGases

Regulatorysubstances

Neutrophils60-70%

Lymphocytes20-25%

Monocytes3-8%

Eosinophils2-4%

Basophils0.5-1%

Components of the BloodCell Structural

CharacteristicsNormal amounts

Function Life Span

Erythrocyte(RBC)

Non-nucleated cytoplasmic disk

4.2-6.2m/mm3

Gas Transport

80-120 days

Reticulocyte

0.5-2% of erythrocytes

Leukocyte(WBC)

Nucleated cell 5000-1000/mm3

Body defense mechanism

Lymphocyte

Mononuclear immunocyte

25%-36% of leukocytes

Humoral and cell mediated

Days or years

Natural Killer cell

Large granular

5-10% Early response to viral infection

Unknown

Monocyte and Macrophage

Large mononuclear

3-8% Phagocytosis

Months or years

Components of the BloodCell Structural

CharacteristicsNormal amounts

Function Life Span

Eosinophil Segmented polymorphonuclear granulocyte

1-4% Phagocytosis, antibody mediated response against paracites, allergies

Unknown

Neutrophil Segmented polymorphonuclear granulocyte

54-67% Phagocytosis, early phase of inflammation

4 days

Basophil Segmented Polymorphonuclear granulocyte

0-.75% Unknown, associated with allergies and mechanical irritation

Unknown

Platelet Irregularly shaped cytoplasmic fragment

140k-340k/mm3

Hemostasis after vascular injury; normal coagulation and clot formation

T-Cell subsetsCell Functions

Helper T-cells Secretes cytokines for immune response. Traget of HIV. These cells are also called CD4 cells

Cytotoxic T-cells Destroy virally infected cells and tumor cells, and are also implicated in transplant rejection. These cells are also known as CD8 cells

Memory T-cells Are a subset of antigen-specific T cells that persist long-term after an infection has resolved. They quickly expand to large numbers of effector T cells upon re-exposure to their cognate antigen, thus providing the immune system with "memory" against past infections.

Regulatory T-cells

Their major role is to shut down T cell mediated immunity towards the end of an immune reaction and to suppress auto-reactive T cells that escaped the process of negative selection in the thymus.

Natural Killer T-cells

These cells can perform functions ascribed to both Th and Tc cells (i.e. cytokine production and release of cytolytic/cell killing molecules)

B-Cell subsetsCell Functions

Plasma B-cells Are large B cells that have been exposed to antigen and are producing and secreting large amounts of antibodies, which assist in the destruction of Microbes by binding to them and making them easier targets for Phagocytes and activation of the compliment system.

Memory B-cells Are specific to the antigen encountered during the primary immune response. These cells are able to live for a long time, and can respond quickly following a second exposure to the same antigen.

B-1 Cells Express IgM in greater quantities than IgG and its receptors show polyspecificity, meaning that they have low affinities for many different antigens, but have a preference for other immunoglobulins, self antigens and common bacterial polysaccharides.

Stem Cell Pool Bone Marrow Pool Peripheral Blood

ProliferatingAnd maturing Storage

Bone Marrow 50% 50%

Storage Functional

30%

70%

0% 100%

Granulocyte

Thrombocyte

Erythrocyte

Nutritional requirements for Erythropoiesis

Nutrient Role Deficiency

Protein Structural Component of plasma membranes

↓strength,elasticity and flexibility of membranes; Hemolytic Anemia

Intrinsic Factor G.I. absorption of B12 Pernicious Anemia

Cobalamin (B12) RNA & DNA synthesis, maturation of erythrocytes

Megaloblastic Anemia

Folate RNA & DNA synthesis, maturation of erythrocytes


Pyridoxine (B6) Heme synthesis, ↑folate metabolism

Hypochromic Microcytic Anemia

Riboflavin (B2) Oxidative Reactions Normochromic-normocytic anemia

Nutritional requirements for Erythropoiesis

Nutrient Role Deficiency

Vitamin C Iron metabolism, maintains iron in ferrous(Fe) form

Normochromic-normocytic anemia

Panthothenic Acid Heme synthesis unknown

Niacin Respiration in mature erythrocytes

unknown

Vitamin E Heme synthesis, possible protection against oxidative rxns in mature erythrocytes

Hemolytic anemia with ↑cell membrane fragility, ↓lifespan or erythro in cystic fibrosis

Iron Hemoglobin Synthesis Iron Deficiency Anemia

Copper Mobilization of tissue iron to plasma

Hypochromic-microcytic anemia

• Nursing Assessment:– Pallor – conjunctiva– Jaundice (hemolytic) – sclera; palms of

hands; soles of feet– Signs of bleeding such as petechiae,

ecchymosis, hematoma, epistaxis– Lymph nodes enlargement– Limited joint range of motion– Splenomegaly or hepatomegaly

– Physical Assessment:•Auscultate – heart murmur, bruits•Inspect – above assessment•Palpate – lymph nodes, location, size, bone tenderness

•Percuss for lung excursion, splenomegaly, hepatomegaly

•Evaluate joint ROM and tenderness

Normal Laboratory Values for Red Blood Cells and PlateletsLaboratory Test Normal Value

Red blood cell count

Men 4.2 to 5.4 million/mm3

Women 3.6 to 5.0 million/mm3

Reticulocytes 1.0% to 1.5% of total RBC

Hemoglobin

Men 14 to 16.5 g/dL

Women 12 to 15 g/dL

Laboratory Test Normal Value

Hematocrit

Men 40% to 50%

Women 37% to 47%

Mean Corpuscular

Volume (MCV) 85 to 100 fL/cell

Mean corpuscular

Hemoglobin concentration

(MCHC) 31 to 35 g/dL

Mean corpuscular

Hemoglobin (MCH) 27 to 34 pg/cell

Platelet count 250,000 to 400,000/mm3

Normal Laboratory Values for White Blood Cells

Laboratory Test Value

WBC count 5000 to 10,000/mm3

Differential Neutrophils 60 to 70% or 3000 to

7000/mm3

Eosinophils 1-3% or 50 to 400/mm3

Basophils 0.3 to 0.5% or 25 to 200/mm3

Lymphocytes 20 to 30% or 1000 to 4000/mm3

Monocytes 3 to 8% or 100 to 600/mm3

Diagnostic Assessment• Complete Blood Count

– Red Blood Cell Count• Circulating RBCs in 1 cubic mm of blood

– White Blood Cell Count• All leukocytes present in 1 cubic mm of blood

– Mean Corpuscular Volume• Measures the average volume or size of a

single RBC• Useful for classifying anemias

– Mean Corpuscular Hemoglobin Concentration• Measures the average amount of hemoglobin

by percentage in a single RBC– Hematocrit

• Calculated as the percentage of red blood cells in the total blood volume

– Hemoglobin• Total amount of hemoglobin

• Reticulocyte Count– Helpful in determining bone marrow function– A reticulocyte is an immature RBC that still has

its nucleus– An elevated reticulocyte count is desirable in an

anemic client or after hemorrhage• Indicates the bone marrow is responding

appropriately to a decrease in the total RBC mass

• Elevated reticulocyte count without a precipitating cause indicates polycythemia vera

• Hemoglobin Electrophoresis– Detects abnormal forms of hemoglobin– Such as Hemoglobin S in Sickle cell disease

• Serum ferritin, transferrin, and total iron-binding capacity (TIBC)- Used to evaluate iron levels.

Ferritin measures the iron in plasma, which is also a direct reflection of total iron stores.

Transferrin is the major iron-transport protein

Total Iron-Binding Capacity– The amount of iron that can be bound to serum

transferrin indirectly

• Reticulocyte Count– Helpful in determining bone marrow

function– A reticulocyte is an immature RBC that

still has its nucleus– An elevated reticulocyte count is desirable

in an anemic client or after hemorrhage• Indicates the bone marrow is responding

appropriately to a decrease in the total RBC mass

• Elevated reticulocyte count without a precipitating cause indicates polycythemia vera

• Hemoglobin Electrophoresis– Detects abnormal forms of hemoglobin– Such as Hemoglobin S in Sickle cell

disease

• Leukocyte Alkaline Phosphatase– An enzyme produced by normal mature

neutrophils• Elevated levels occur during episodes of

infection or stress• If elevated neutrophil count without increase

in LAP indicates chronic myelogenous Leukemia

• Coomb’s test– Used for blood typing– Direct test

• Detects the presence of antibodies against RBCs that may be attached to a person’s RBC

– Indirect test• Detects the presence of circulating

antiglobulins

• Capillary Fragility Test– Rumpel-Leede test– Measures the vascular hemostatic

function•When the number of petechiae that

form increases, the cause of excessive bleeding or bruising is capillary fragility rather than poor platelet action

• Bleeding Time Test– Evaluates vascular and platelet

activities during hemostasis– Normal bleeding time ranges from 1-9

minutes

• Prothrombin Time– Measures how long blood takes to clot– Reflects how much of the clotting factors

II,V and X is present and how well they are functioning

• Prolonged when one of the clotting factors is deficient such as

– Liver disease– Warfarin therapy is considered when PT is

prolonged by one and a half to two times the normal value

• International Normalized Ratio– Measures the same process as the PT in a

slightly different way– Establishing a normal mean or standard for

PT– Calculated by dividing the client’s PT by the

established standard PT

• Partial Thromboplastin Time– Assess the intrinsic clotting cascade and evaluates the

action of factors II,V,VIII,IX,XI and XII– Prolonged when any of these factors is deficient such as

• Hemophilia• Disseminated Intravascular disease

– Factors II,IX and X are Vitamin K dependent and are produced in the liver

• Liver disease can decrease their levels and prolong PTT

– Heparin therapy is monitored

• Platelet Agglutination/Aggregation– The ability to clump– Tested by mixing the client’s plasma with a substance

called Ristocetin• During use of drugs such as

– Aspirin– Anti-inflammatory agents– Psychotropic drugs– Platelet inhibitors

• Aggregation can be impaired in – Von Willebrand’s disease

• Coagulation studies

Fibrinogen normal range is 150-400 mg/dl . It is a soluble plasma protein that is decreased in DIC

Fibrin degradation products (FDP): normal value is < 10 micrograms/ml. FDP is increased in fibrinolysis, thrombolytic therapy, and DIC.

Fibrin D-dimer - normal is 0 - 0.5 micrograms/ml. D-dimer is the most sensitive indicator to differentiate DIC from primary fibrinolysis. It is elevated in DIC.

Radiographic Examinations

• Radioisotopic Imaging– Isotopes are used to evaluate the

bone marrow for sites or active blood cell formation and sites of iron storage

– Client is given a radioactive isotope intravenously about 3 hours before the procedure

– No special client preparation or follow-up care is needed

Bone Marrow Aspiration and Biopsy

• Assess the cellularity and morphology of the bone-marrow cells.

• Most valuable diagnostic procedures for the diagnosis and staging of haematological disorders

• They can also be used to assess the cause of bone-marrow failure in patients with solid tumours

• Investigate pyrexia of unknown origin • Examination of the aspirate or biopsy specimen

may reveal infections such as – tuberculosis – Mycobacterium avium-intracellulare (MAI)

infections– histoplasmosis – leishmaniasis – other disseminated fungal infections.

• Useful in establishing the diagnosis of storage diseases such as – Niemann-Pick disease – Gaucher disease.

• Preferred sites for bone-marrow aspiration and bone-marrow biopsy are: – 1. Posterior iliac crest and

anterior iliac crest (both aspiration and biopsy)

– 2. Sternum (aspiration only in adults)

• INDICATIONS:

Unexplained anemia. Decreased presence of red blood cells, the cause of which may be a variety of reasons, the most common being deficiency of iron stores and Myelodysplasia.

Thrombocytopenia. Decreased production of platelets, I.T.P. (or Idiopathic Thrombocytopenia) being most common. Physicians determine if adequate number of platelet producing cells (Megakaryocytes) are present in the bone marrow.

Pancytopenia. Decreased production of all three cell lines, differentiates relative involvement of red cell, white cell and platelet lines in a disease.

Leukemia, Lymphoma, or Myeloma (helpful in diagnoses, staging and determining results of the treatment)

Lympho and Myeloproliferative disorders. Metastatic disease. To determine if bone marrow is

involved with cancers from other sites. Chromosomal analyses Unusual infection. Such as Tuberculoses, Fungi and

Fevers of Unknown Origin

• Preparation– Allay anxiety and fear– Secure consent– Stay with the patient during the procedure– Provide emotional support by having to hold the

client’s hands

• Procedure– Lasts from 5 to 15 minutes– Amount of anesthesia or sedation depend on the

physician’s preference– Skin over the site is cleaned with a disenfectant

solution– Needle is inserted in a twisting motion and the

marrow is aspirated by pulling back the plunger of the syringe

– Pressure and several twisting motions are needed to ensure coring and loosening of a adequate amount of marrow tissue

– Pressure dressing or sandbags may be applied to reduce bleeding at the site

• CONTRAINDICATIONS:– Hemophilia – Other Clotting disorders. (If you are

taking a blood thinner your doctor needs to be informed before the procedure).

– Previous Radiation Therapy site.

• PRE- and POST-PROCEDURE PATIENT EDUCATION:

Procedure is performed under local anesthesia, therefore patient can eat or drink any time before or after the

procedure. The patient will need to remain in a supine position (lay on the

back) for about an hour after the procedure to help keep pressure on the biopsy site.

The urinary bladder should be emptied before the procedure. The bandage over the biopsy site may be removed 24 hours after

the procedure. Ask the patient to notify the physician of any

Pain Drainage fever spreading redness around the biopsy site area.

A standard consent form should be signed by all patients before the procedure.

• Follow-Up Care– cover site with a dressing after a

bleeding is controlled– Closely observe for 24 hours for signs

of bleeding and infection– A mild analgesic may be given for

discomfort– Ice packs can be placed over the site to

limit bruising– Advise client to avoid contact sports or

any activity that might result in trauma to the site for 48 hours

The Bone Marrow• Sponge-like tissue found in the center of certain

bones-contains stem cells that are the precursors of – white blood cells– red blood cells– platelets.

• These blood cells are vital for normal body functions such as – oxygen transport– defense against

• infection • Disease• Clotting

• Blood cells have a limited lifespan and are constantly being replaced; therefore, healthy stem cells are vital

Bone Marrow Transplantation

• Involves extracting bone marrow containing normal stem cells from a healthy donor, and transferring it to a recipient whose body cannot manufacture proper quantities of normal blood cells

• The goal of the transplant is – to rebuild the recipient's blood cells and

immune system – hopefully cure the underlying ailment

• Precautions Accompanied by a risk of infection Transplant rejection by the recipient's immune system Other complications Complications are exacerbated for people whose health is

already seriously impaired as in late-stage cancers.

Person's age or state of health may prohibit use of a bone marrow transplant.

The procedure has a lower success rate the greater the recipient's age

Typical cut-off age ranges from 40 to 55 years a person's general health is usually the more important

factor. Are debilitating Person's ability to withstand the rigors of the transplant is

a key consideration in deciding to use this treatment.

• Autologous transplant– It is typically used in cases in which a person's bone

marrow is generally healthy but will be destroyed due to medical treatment for diseases such as

• breast cancer and Hodgkin's disease. – Most bone marrow transplants are autologous.

• Allogeneic transplants – Are more complicated – Proteins called human lymphocyte antigens (HLA) that

are on the surface of bone marrow cells – If the donor and the recipient have very dissimilar

antigens, the recipient's immune system regards the donor's bone marrow cells as invaders and launches a destructive attack against them.

• Such an attack negates any benefits offered by the transplant.

• The treatment of choice for a client with leukemia

• Also used for lymphoma, aplastic anemia, sickle cell disease and many solid tumors

• Bone Marrow is the actual site of production of leukemic cells– Additional chemotherapy treatments– Total body irradiation

• HLA matching is more likely if the donor and recipient are related – particularly if they are siblings

• an unrelated donor may be a potential match– Only in rare cases is matching HLA

types between two people not an issue

• if the recipient has an identical twin – Identical twins carry the same genes

and the same antigens

• Once a patient has been identified as a candidate for bone marrow transplant (BMT)– Arrangements will be made for scheduling

the procedure

• During this period, patients are encouraged to build up their strength with safe and suitable activities recommended by their physicians

• Appropriate exercise and nutrition can play an important role in preparing for and recovering from BMT

• Before undergoing BMT, patients have a series of test and procedures for screening and preparation

• The testing schedule is based on the patient’s disease process and medical history

• These tests and procedure may include:– Blood tests to measure

kidney liver heart lung hormone function

– Blood tests to screen for infections– Bone marrow evaluation– X-rays and computer-assisted tomography (CT) scans– Lumbar puncture (spinal tap)– Physical examination– Dental examination– Psychological evaluation– Placement of a central venous catheter

• Pre-transplant Education– Patients and their caregivers will meet with a

transplant physician and BMT nurse coordinator

• To discuss the results of the testing, treatment options and the treatment plan

– Touring areas where BMT patients are treated – Familiarize themselves with these areas,

patients and their caregivers may tour• The hospital bone marrow transplant unit• The outpatient chemotherapy unit• The apheresis unit• The radiation oncology area

– During this tour, staff from each area wil explain their role in the treatment plan

– Patients and caregivers are encouraged to ask questions

• Transplant Conditioning– The goal of transplant conditioning is to

destroy abnormal cells or cancer cells throughout the patient’s body

– The conditioning regimen is based on the• Type of disease• Previous treatment• Clinical trial participation

– It may consist of• Chemotherapy• Radiation therapy• both

– Radiation may be given before the transplant as part of the conditioning regimen, or it may be given following recovery from transplant

• TransplantationBlood and Marrow Stem Cell donation– The harvest procedures for autologous or

allogeneic BMT are similar– The timing of the blood or bone marrow

harvest depends on factors such as• The patient’s physical condition• Donor availability• Insurance approval

– Peripheral blood stem cells are obtained through a process called Apheresis, which separates blood into its different components

– Before and during Apheresis• The donor will receive daily injections of protein

growth factors to help stimulate bone marrow to make new white blood cells

• Stem cells collected by Apheresis can then be frozen for later use

• Donors of bone marrow typically enter the hospital the morning of the donation

• The donation is taken in the operating room under general anesthesia

• Because general anesthesia is used, donors may remain in the hospital overnight for observation

• Most donor’s bodies will replace the donated marrow within two or three weeks

• Healthy bone marrow may be frozen for storage and transplanted later

• Length of Stay• How long the patients need to

remain near the Clinic depends on a variety of factors which include:– When the transplanted marrow “engrafts”

and begins producing healthy blood cells– Patient’s need for red blood cell and platelet

transfusions– Their calorie intake– The level of independence and ability to

perform daily self care– The level of support from caregivers at

home

• Follow-up– After a patient has returned home,

follow-up appointments are needed periodically

– These visits will take from one to four days to complete

– During these follow-up visits, the staff will perform tests to evaluate how well the treatment is working

Common nursing procedures of the

hematologic system • Protocol for the administration of

blood and blood products1. Check the agency’s policy and procedure.2. Verify the physician’s order3. Consent Typing and crossmatch4. Obtain blood intravenous access line is

available. 5. IV with normal saline6. Blood may not be returned to the blood

bank after 20 minutes.

7. Do not keep blood or blood products in the nursing unit refrigerator.

8. Validate data of blood product with another nurse. Validate with another nurse that the client’s name, ID number, blood type and Rh matches the unit of blood to be transfused.

9. Note the expiration date indicated on the blood product Observe the unit of blood for bubbles or discoloration.

10. Pre-transfusion vital signs, 15 minutes after the transfusion is initiated and immediately after the completion of the transfusion.

11. *A pre-transfusion temperature of 100 F should be reported before initiating the transfusion. Any increase in 2 degrees F in the client’s temperature may be an indication of a transfusion reaction

12. *Start blood transfusion slowly (25-50 mL during the first 15 minutes). Stay with the client during first 15 min.

13. Do not administer any medications through the blood transfusion tubing.

14. Use only agency- approved blood- warming devices.

15. Blood products should not be infused longer than 4 hours

16. Discard tubings and bags used in the transfusion in a biohazard receptacle.

17. Follow protocol for transfusion reactions.

Common nursing procedures of the

hematologic system• Protocol for suspected blood

transfusion reaction1. Check agency protocol.2. Stop the infusion immediately.

Change IV tubing and keep vein open with normal saline.

3. Assess the client for other signs and symptoms of transfusion reaction.

4. Notify the physician and the blood bank.

5. Send the unit of blood and tubing used to the blood bank.

6. Urine and blood samples will be required.

7. Administer prescribed drugs8. Document the reaction and

interventions.

Signs and Symptoms of Blood Transfusion Reaction

• Hemolytic reaction

•Chills

•Fever

•Urticaria

•Tachycardia

•chest pain or complaints of chest tightness

•shortness of breath

•dyspnea

•lumbar pain

•Nausea

•Rales

•Vomiting

•hematuria,

•Hypotension

•Wheezing


• Bacterial (Pyrogenic) reaction

•Hypotension

•Fever

•Chills

•Flushed skin

•Abdominal pain

•Pain in extremities

•Vomiting

•Diarrhea


• Allergic reaction

•Urticaria

•Pruritus

•Swelling of the tongue

•Swelling of the face

•Difficulty of breathing

•Pulmonary edema

•Shock


• Circulatory Overload

•Chest pain,

•Tightness of the chest

•Cough

•Rales

•Pulmonary edema

•Tachycardia

•Elevated blood pressure

Disordersof the

Hematologic System

Erythrocyte Disorders• Anemia

– reduction below normal level in number of erythrocytes, quantity of hemoglobin and volume of packed RBC’s.

– Basic underlying – tissue hypoxia– Signs and Symptoms – depends upon severity and

chronicity and age.• Mild

– hemoglobin 10-14 gms; asymptomatic; palpitations, dyspnea and diaphoresis following strenuous exertion.

• Moderate– increased palpitations, dyspnea, and diaphoresis; fatigue at

rest or during activity.• Severe

– pale and exhausted all the time, sever palpitations, sensitivity to cold, loss of appetite, profound weakness, angina.

Microcytic Anemia• Iron Deficiency Anemia

– Supply of iron is inadequate for optimal formation of RBCs related to:

• excessive iron loss due to bleeding• decreased dietary intake• malabsorption

– Causes:• Inadequate absorption – increased requirement• Inadequate intake of iron rich foods• Physiologic need – more in children and pregnant

women• Physiologic loss – menstruation• Blood loss – trauma, GI bleeding

– Etiology and Pathophysiology• Accounts for 60% of anemias in clients over

age 65.• Most common cause -blood loss from

gastrointestinal or genitourinary system.• Normal iron excretion is less than 1 mg/day

through the urine, sweat, bile, feces, and from desquamated cells of the skin

• 0.5 mg of iron daily or 15 mg monthly during menstruation

• Reduced oxygen carrying capacity of the blood, producing tissue hypoxia. Iron is stored in the body as ferritin

• It is formed in the intestinal mucosa, when ferritin iron joins with the protein apoferritin

• *ferritin is stored in the tissues, primarily in the reticuloendothelial cells of the liver, spleen, and bone marrow.

• Develops slowly through three phases: – Body’s stores of iron depleted– insufficient iron is transported to the bone

marrow and iron deficient erythopoiesis begins

– small hemoglobin deficient cells enter the peripheral circulation in large numbers

• iron is needed on the hemoglobin so the oxygen molecule will attach.

• Adequate iron in the RBC is essential since the oxygen molecule attaches to it.

• An average diet supplies the body with 12 to 15 mg/day of iron, of which only 5-10% is absorbed.

– Signs and Symptoms• PalpitationsPalpitations• DizzinessDizziness• Easy fatigabilityEasy fatigability• Cold sensitivityCold sensitivity• Pallor Pallor • Brittle nails and hairBrittle nails and hair• Plummer-vinsons syndromePlummer-vinsons syndrome

– soreness and inflammation of mouth and soreness and inflammation of mouth and tongue (stomatitis and glossitis)tongue (stomatitis and glossitis)

– Nursing management:• Oral iron

– route of choice; given after meals; liquid iron intake with straw because it stains; mixed with 1 glass cold H2O, best absorbed with Vitamin C; stool becomes tarry and constipation may occur.

• Parenteral iron– avoid tissue staining by using separate

aspiration injection needles; Z-tract method and deep IM; do not massage but encourage ambulation.

• Dietary– increase in iron and roughage

• Blood transfusion

Megaloblastic Anemia• Pernicious anemia

– Vitamin B12 (cyanocobalamine) deficiency of intrinsic factor in the gastric mucosa which is necessary for absorption of Vitamin B12.

– Signs and Symptoms:• Hemolytic jaundice

– macrolytic hypochromic

• Tingling sensations, paresthesias• Beefy red tongue• Deficiency or absence of hydrochloric

acid in the stomach

– Etiology and Pathophysiology• Inevitably develops after total

gastrectomy or gastrojejunostomy.• Lack of vitamin B12 alters the structure

and disrupts the function of the peripheral nerves, spinal cord and brain.

• Lack of Vitamin B12 impairs cellular division and maturation especially in rapidly proliferating RBC's.

• Pernicious anemia is the body’s inability to absorb Vitamin B12 due to a lack of intrinsic factor, a substance secreted by the parietal cells of the gastric mucosa.

– Signs and Symptoms• pallor or slight jaundice• complaint of weakness• smooth sore beefy red tongue (glossitis)• Diarrhea• Paresthesias• difficult prioception• fair haired or prematurely gray.

– Nursing management:• Drug therapy

– Vit B12 injections (monthly) for life– Folic acid – reverses anemia, decreases

neurological symptoms

• Transfusion therapy

– Diagnostic assessment:• Schilling’s test• Gastric analysis


• Folic Acid Deficiency Anemia– Etiology and Pathophysiology

• poor nutrition• malabsorption syndrome• medications that impede the absorption

(oral contraceptives, anticonvulsants, methotrexate [MTX])

• alcohol abuse• anorexia.• Lack of folic acid causes the formation of

megaloblastic cells. These cells are fragile.

– Risk Factors• Pregnancy• Alcoholism• Hemodialysis

– Signs and Symptoms• Pallor• progressive weakness• Fatigue• shortness of breath• cardiac palpitations• GI symptoms are similar to B12 deficiency, but

usually more severe (glossitis, cheilosis, and diarrhea);

**Neurological symptoms seen in B12 deficiency are not seen in folic acid deficiency and therefore assist in the differentiation of these two types of anemia.

– Diagnostic and Laboratory tests• Macrocytic (megaloblastic) anemia

– RBC diameter > 8

• MCV high with low hemoglobin• Low serum folate level

– Nursing Management• Energy saving techniques

– e.g., shower chair, sitting to perform tasks

• Monitor for dizziness, suggest position changes be made slowly.

• Encourage/assist with good oral hygiene before and after meals, using soft bristled toothbrush for gentle brushing of fragile gums.

– Medical Management• Oral folate, 1-5 mg/day for 3-4 months.• Folate should be given along with vitamin

B12 when both are deficient.

– Client Education• Dietary sources of folic acid - green leafy

vegetables, fish, citrus fruits, yeast, dried beans, grains, nuts, and liver.

• Teach clients at risk to increase their dietary intake through diet selection and supplementation.

• Strategies to decrease pain associated with glossitis such as eating bland and soft foods.

Aplastic Anemia• Aplastic anemia

– depressed bone marrow activity secondary to:• Antineoplastics• Radiation• Insecticide• drugs and chemical toxins.

– Laboratory Assessment:• Pancytopenia• Erythrocytopenia• Leukocytopenia • Thrombocytopenia

– Etiology and Pathophysiology• Affects all age groups and gender.• Two classifications

– Congenital aplastic anemia is caused by a chromosomal alteration.

– Acquired form may be caused by radiation, chemical agents and toxins, drugs, viral and bacterial infections, pregnancy, and idiopathic.

• In about 50% of cases, the cause is unknown.

• There is a decrease or cessation of production of:

– RBCs (anemia)– WBCs (leukopenia)– platelets (thrombocytopenia).

• Decrease may result from damage to bone marrow stem cells, the bone marrow itself, and the replacement of bone marrow with fat.

• Condition may be acute or chronic.

– Clinical Manifestations• Pallor• Fatigue palpitations• Exertional dyspnea• Infections of the skin and mucous membranes• Bleeding from gums, nose, vagina or rectum• Purpura (bruising)• Retinal hemorrhage

– Diagnostic and Laboratory Tests• Blood counts reveal pancytopenia (decreased

RBC, WBC, and platelets).• Decreased reticulocyte count• Bone marrow examination reveals decrease in

activity of the bone marrow or no cell activity.

– Therapeutic management• Identification of the cause of bone

marrow suppression • Bone marrow transplantation.• Immunosuppression• Transfusion of leukocyte-poor RBCs.• Spleenectomy• Blood transfusion• Prevent and treat infections• Drugs

– Corticosteroids– estrogen

• Identify and withdraw offending agent

Hemolytic Anemia• Sickle Cell

– hereditary, chronic form of hemolytic anemia.

– Etiology and pathophysiology• 8% of African-Americans are

heterozygous (carriers) for sickle cell anemia thereby inheriting one affected gene or the sickle cell trait

• 1% of African-Americans are homozygous (identical genes) for the disorder, thereby inheriting a defective gene from both parents or sickle cell anemia and are likely to experience sickle cell crisis.

• Sickle cell trait (heterozygous state) is a generally mild condition that produces few, if any manifestations.

• Sickle cell anemia is caused by an autosomal genetic defect (one gene affected) that results in the synthesis of hemoglobin S.

• Produced by a mutation in the beta chain of the hemoglobin molecule though a substitution of the amino acid valine for glutamine in both beta chains.

• During decreased oxygen tension in the plasma, the hemoglobin S causes the RBCs to elongate, become rigid, and assume a crescent sickled shape. Cells clump, obstruct capillary blood flow causing ischemia and possible tissue infarction.

– Conditions likely to trigger a sickle cell crisis include:• Hypoxia• low environmental and/or body temperature• excessive exercise• high altitudes• inadequate oxygen during anesthesia.

– Other causes of sickle cell crisis include:• elevated blood viscosity/decreased plasma

volume• Infection• Dehydration• increased hydrogen ion concentration

(acidosis).

• With normal oxygenation, the sickled RBCs resume their normal shape. Repeated episodes of sickling and unsickling weaken the cell membrane, causing them to hemolyze and be removed

• Crisis is extremely painful and can last from 4-6 days.

– Clinical manifestations

• Pallor

• Jaundice

• Fatigue

• Irritability

• Priapism

• Large joints and surrounding tissue may become swollen during crisis

– Diagnostic and Laboratory Tests• Anemia with sickled cells noted on a

peripheral smear.• Hemoglobin electrophoresis to determine

the presence and percentage of hemoglobin S is used for a definitive diagnosis.

• Elevated serum bilirubin levels. • Elevated reticulocyte count.

– Therapeutic Management• Bone marrow transplantation• Blood transfusions • Management of pain • Use of chemotherapy drug hydroxyurea

(Droxia) to increase hemoglobin F and decrease sickling

– Nursing Management• Teach prevention of sickle cell crisis. • Referral to appropriate agency for

genetic counseling and family planning.• Clients who are in crisis should have the

following included• Management of infection

– Management of pain. – Administration of oxygen. – Promoting hydration to decrease blood

viscosity. The client in crisis should have an oral intake of at least 6 to 8 quarts per day or IV fluids of 3 liters per day.

• Monitor for complications such as:– vaso-occlusive disease (thrombosis)– Hypoxia– CVA– renal dysfunction– priapism leading to impotence, acute chest

syndrome (fever, chest pain, cough, pulmonary infiltrates, and dyspnea)

– substance abuse. • Teach client ways to prevent sickle cell

crisis – Maintaining adequate fluid intake. Clients with

sickle cell disease should maintain an oral intake of at least 4 to 6 quarts per day. Avoid conditions that might predispose them to dehydration.

– Avoiding high altitudes – Prevention of and prompt treatment of infections – Stress reduction strategies– Importance of regular medical follow-up.

– Avoid exposure to cold. – Avoid overexertion. – Adhere to vaccination schedules for

pneumococcal pneumonia, haemophilus influenza type B, and hepatitis B.

– Importance of regular medical follow-up.

– Medication Therapy• Nifedipine (Procardia) may be used for

priapism. • Hydroxyurea (Droxia) to increase

hemoglobin F and decrease sickling• Narcotic analgesics during the acute

phase of sickle cell crisis. • Broad spectrum antibiotics to manage

acute chest syndrome. • Folic acid supplements

Polycythemia Vera

• An increase in the number of circulating erythrocytes and the concentration of hemoglobin in the blood

• Also known as polycemia, PV, or Myeloproliferative red cell disorder.

• Can be primary or secondary

• Etiology and Pathophysiology– Primary

• Common in men of European Jewish descent.

• Neoplastic stem cell disorder characterized by increased production of RBCs, granulocytes, and platelets.

• With the over production of erythrocytes, there is increased blood viscosity resulting in congestion of blood in tissues, the liver, and spleen.

• Thrombi form, acidosis develops, and tissue infraction occurs as a result of the diminished circulatory flow of blood due to the increased viscosity.

– Secondary• Most common form of polycythemia vera. • The disturbance is not in the

development of red blood cells but in the abnormal increase of ertythropoietin, causing excessive erythropoiesis.

• The increase in red blood cell production due to increased erythropoietin release is a physiologic response to hypoxia. Hypoxia stimulates the release of erythropoietin in the kidney.

• Chronic hypoxic states may be produced by prolonged exposure to high altitudes, pulmonary diseases, hypoventilation, and smoking.

• The results of an increased RBC production include the increased viscosity of blood, which alters circulatory flow.

• Clinical Manifestations– Plethora

• a ruddy (dark, flushed) color of the face, hands, feet, ears, and mucous membranes resulting from the engorgement or distention of blood vessels.

– Symptoms associated with increased blood volume including:• Headaches• Vertigo• blurred vision• tinnitus.

– Distended superficial veins.

– Itching unrelieved by antihistamines. – Symptoms associated with impaired

tissue oxygenation including:• Angina• Claudication• Dyspnea

– Erythromyalgia– burning sensation of the fingers and

toes.– Splenomegaly in majority of those

with primary polycythemia vera. – Epistaxis, GI bleeding

• Diagnostic and Laboratory Tests– Elevated hemoglobin and erythrocyte

count– Decreased MCHC – Increased WBC and basophilia – Increased platelets – Elevated leukocyte alkaline phosphatase – Elevated uric acid– Elevated cobalamin levels – Increased histamine levels – Bone marrow examination shows

hypercellularity.

• Therapeutic Management– Management of the underlying

condition (such as COPD) causing the chronic hypoxia.

– Repeated phlebotomy to decrease blood volume. The goal is to keep the hematocrit less than 45 to 48%.

– Hydration to decrease blood viscosity.

• Nursing Management– Assist in phlebotomy – Measures to relieve pruritus including

cool and tepid baths – Accurate monitoring of fluid intake

and output – Nursing measures to prevent

thrombotic events including:• early ambulation• passive leg exercises when on bed rest• avoid crossing legs• maintaining adequate hydration

– Administration of medications for the prevention of complications including anticoagulants.

• Medication Therapy– Myelosuppressive agents to inhibit

bone marrow activity including:• hydroxyurea (Hydrea)• melphalan (Alkeran)• radioactive phosphorous

– Allopurinol – Antiplatelet agents

• Client Education– Importance of maintaining good

hydration.• 3 liters of fluid per day.

– Facts about the disease and ways in which it can be controlled• smoking cessation.

– Discuss signs and symptoms of complications associated with the disorder

– Prevent bleeding states – Importance of a regular medical check up.– Avoid products that contain iron. – Ways of preventing thrombosis.

Thrombocytopenia

• A decrease in the number of circulating platelets or a platelet count of less than 100,000 platelets per milliliter of blood resulting in problems of hemostasis.

• Etiology and Pathophysiology– The decrease in the number of

circulating platelets may be a result of three mechanisms:• decreased production• increased destruction• increased consumption.

– The cause of decreased production of platelets may be inherited or acquired.

– Increased destruction of platelets may be caused by an immune system defect.• The platelets become coated with an

antibody.

– When these antibody coated platelets reach the spleen, they are recognized as foreign and are destroyed.

– Platelets normally have a circulating life of 8 to 10 days but because of this immune response their life cycle is shortened. This condition is referred to as Immune Thrombocytopenic Purpura (ITP)

– The acute form of ITP is more common in children whereas the chronic form is more common in women between the ages of 20 to 50.

– Other causes of increased destruction of platelets include non -immune related factors such as infection or drug induced effects.

– A decrease in the number of functional platelets leads to bleeding disorders. Cerebral and pulmonary hemorrhage can occur when platelet counts drop below 10,000/mm3.

• Clinical Manifestations– Petechiae and purpura

• anterior thorax• Arms• neck)

– Epistaxis– Gingival bleeding– Menorrhagia– Hematuria– Gastrointestinal bleeding.– Signs of internal hemorrhage

• Diagnostic and laboratory tests

– Decreased hemoglobin and hematocrit if bleeding is present.

– Decreased platelet count.

– Prolonged bleeding time.

– Bone marrow examination to determine the etiology.

• May reveal decreased platelet activity or increased megakaryocytes.

• Therapeutic management

– Treatment of the underlying cause or removal of the causative agent.

– Use of immunosuppressive and chemotherapeutic agents in cases of ITP

– Platelet transfusions if there is active bleeding; little benefit in ITP.

– Splenectomy in ITP

• Nursing Management– Institute bleeding (thrombocytopenic)

precautions:• Avoid intramuscular or subcutaneous injections• Avoid indwelling catheters • If absolutely necessary use smallest gauge

needles for injections or venipunctures.• Apply pressure on injection sites for 5 minutes or

until bleeding stops.• Discourage straining at stool, vigorous coughing

and nose blowing.• Avoid rectal manipulation such as rectal

temperatures, suppositories, or enemas. • Discourage the use of razors. • Use only electric shavers. • Use soft-bristled toothbrush or toothettes and

avoid flossing.• Pad side rails if necessary and avoid tissue

trauma • Avoid the use of aspirin and drugs that interfere

with blood coagulation.

– Monitor for signs of bleeding.

– Test stools for occult blood.

– Monitor CBC and platelet counts.

– Administer platelets as ordered.

– Monitor response to therapy.

• Medication therapy

– Steroids and immunoglobulins may be used to suppress the immune response in ITP

– Immunosuppressive agents may be used such as: • vincristine (Oncovin)

• cyclophosphamide (Cytoxan).

– Platelet growth factor such as oprelvekin (Neumega).

• Client education

– The client and family should be taught to monitor for signs of bleeding and when to contact the primary care provider.

– Instructions on bleeding precautions including:

• use of soft-bristled toothbrush

• avoidance of flossing

• prevention of tissue trauma and injury including vigorous sexual intercourse

• using an electric razor for shaving.

– Teach client to avoid drugs that contain aspirin and others that interfere with coagulation.

– Discuss medication dosing, schedule, and side effects.

– Teach the importance of regular medical follow up and platelet monitoring.

Hemophilia

• A group of hereditary clotting factor disorders characterized by prolonged coagulation time that results in prolonged and sometimes excessive bleeding.

• Etiology and Pathophysiology– Hemophilia A and B are X-linked

recessive disorders transmitted by female carriers, displayed almost exclusively in males.

– Hemophilia A (classic hemophilia) is a deficiency in Factor VIII . Most common form of hemophilia.

– Hemophilia B (Christmas disease) is a deficiency in Factor IX.

– Hemophilia A & B are clinically identical. - In clients with hemophilia A &B, platelet plugs are formed at the site of bleeding, but the clotting factor impairs the coagulation response and the capacity to form a stable clot.

– Von Willebrand’s Disease• a related disorder caused by a deficiency

of the von Willebrand’s factor (vWF), which is necessary for factor VIII activity and platelet adhesion.

• This disorder affects men and women equally

• Clinical Manifestations– Persistent and prolonged bleeding

from small cuts and injuries.– Delay of onset of bleeding after an

injury.– Subcutaneous ecchymosis and

subcutaneous hematomas.– Gingival bleeding– Gastrointestinal bleeding.– Hematuria– Pain, paresthesias, or paralysis

resulting from nerve compression of the hematomas.

– Hemarthrosis


– Specific factor assays to determine the type of hemophilia present

– APTT is increased in all types of hemophilia

– Bleeding time is prolonged in von Willebrand disease

– Decreased factor VIII in hemophilia A, vWF disease, and factor IX in hemophilia B.


– Treatment is the replacement of the deficient coagulation factor(s).

– Hemophilia A

• cryoprecipitate containing 8-100 units of factor VIII per bag at 12- hour intervals until bleeding ceases. Freeze-dried concentrate of Factor VIII may also be given.

– Hemophilia B:

• plasma or factor IX concentrate given Q 24 hours or until bleeding ceases.

– Von Willebrand’s disease

• cryoprecipitate containing 8-100 units of factor VIII per bag at 12-hour intervals until bleeding ceases. Desmopressin (DDAVP) given intravenously may also be used.

– Supportive treatment for hemarthrosis including arthrocentesis and physiotherapy.

– Control of topical bleeding with hemostatic agents, pressure, and application of ice. - Management of complications associated with hemorrhage.

• Nursing Management– Teach the client and family about the

disease and therapeutic regimen– Refer for genetic counseling and family

planning.– Refer to the National Hemophilia

Foundation for support and counseling– Monitor for signs of complications

including hemarthrosis and intracranial bleeding

– Assist in the management of pain associated with hemarthrosis

– Control bleeding and maintain hemostasis

– Administer medications as prescribed.

• Client Education– Signs and symptoms requiring

immediate medical attention– Precautions to prevent bleeding.– Medic Alert bracelet indicating about

the hemophilia.– Maintain good dental hygiene to

decrease the necessity of invasive dental procedures.

– Adhering to scheduled visits and follow up care

Disseminated Intravascular Coagulation• Also known as Consumption

coagulopathy• a syndrome characterized by

abnormal initiation and acceleration of clotting and simultaneous hemorrhage.

• The paradoxical bleeding that occurs is a result of the consumption of clotting factors and platelets.

• The syndrome is usually precipitated by an underlying pathologic condition.

• Etiology and Pathophysiology– Mortality rate associated with DIC is as high

as 80% with the most frequent sequela being hemorrhage.

– The syndrome is precipitated by conditions such as widespread tissue damage, hemolysis, hypotension, hypoxia, and metabolic acidosis.

– The underlying condition causes initiation and widespread formation of clots in the vascular system either through the activation of factor XII, factors II and X, or the release of tissue thromboplastin. Substances necessary for clotting are used at a more rapid rate than it can be replaced.

– As the clotting continues, the fibrinolytic pathway is activated to dissolve the clots formed. Clotting factors become depleted while fibrinolysis continues. Platelets decrease, clotting factors II, V, VIII, and fibrinogen are depleted

– Fibrin degradation products (FDP) are released as a result of fibrinolysis. Fibrin degradation products (FDP), which are potent anticoagulants used to lyse the clots further increases the bleeding state.

– With the depletion of clotting factors and the increase in fibrin degradation products stable blood clots no longer form and hemorrhage occurs.

• Risk factors– Venomous snake bite– Sepsis– Trauma– Obstetric complications– Neoplasms– Vascular disorders– Hypoxia– Drug reactions– Liver disease– Acute hemolysis– Extensive burns– Prosthetic devices

• Clinical Manifestations– Integumentary

• Decreased skin temperature• Pallor• Purpura• Ecchymoses• Hematomas• Acral cyanosis• Superficial gangrene• Altered sensation• Gingival bleeding• Bleeding from puncture sites

– Gastrointestinal• Hemoptysis• Melena• Occult blood in stool or vomitus• Abdominal distention• Abdominal pain

– Respiratory• Dyspnea• Orthopnea• Tachypnea• Decreased breath souns• Chest pain

– Genitourinary• Hematuria• Oliguria

– Nervous system• Vision changes• Dizziness• Headache• Irritability• Anxiety• Confusion• Seizures

– Cardiovascular• Decreased pulses• Decreased capillary filling time• Tachycardia• Venous distention

– Musculoskeletal• Joint pain• Bone pain• Weakness


– Prothrombin time - prolonged

– Partial thromboplastin time – prolonged

– Thrombin time – prolonged

– Fibrinogen – decreased

– Platelets- decreased

– Fibrin split (degradation) products – elevated

– Factor assays (factors V, VII, VIII, X, XIII)- reduced

– D-dimers- elevated

• Therapeutic Management-– The priority of therapeutic management is

to initiate treatment of the underlying medical condition that precipitated DIC.

– Life-threatening hemorrhage accomplished by administering specific blood components based on the identified deficiency:

• Platelets for thrombocytopenia• cryoprecipitate to replace fibrinogen, and factors

V and VII• fresh frozen plasma to replace all clotting factors

except platelets.

– Use of Heparin or Antithrombin III (AT-III) to control intravascular clotting. Their use is controversial.

• Nursing Management– Assess client carefully for evidence of

bleeding and altered tissue oxygenation– Institute thrombocytopenic precautions– Monitor intake and output hourly.– Administer blood products as indicated.– Monitor for signs of complications such

as:• renal failure• pulmonary embolism• cerebrovascular accident• acute respiratory distress syndrome

– Monitor effectiveness of therapy and pharmacologic interventions.

– Provide emotional support to client and family.

• Medication therapy

– Heparin

– Antithrombin III

– Epsilon aminocaproic acid (Amicar)

• to inhibit fibrinolysis

– Blood products

• FFP

• Platelets

• cryoprecipitate

• Client education

– Regarding the syndrome and explain treatments and interventions

– To report symptoms of complications including:

• abdominal pain

• Headache

• visual disturbances

• pain

– Thrombocytopenic precautions

Neutropenia

• Refers to a decrease (less than 2000/mm3) in the neutrophil count either as a result of decreased production or increased destruction.

• The neutrophil plays a major role in phagocytosis of disease-producing microorganisms. Consequently, a decrease in their numbers increases the individual’s risk for infection.

• Etiology and Pathophysiology

– Neutropenia is not a disease but a syndrome.

– May occur as a primary hematologic disorder but may also be caused by drugs, autoimmune disorders, infections, and other medical conditions such as severe sepsis and nutritional deficiencies.

– If the leukocyte count is decreased, or if immature white blood cells predominate in the circulation, the normal phagocytic function of these cells is impaired.

– Neutrophils constitute about 70% of the total circulating white blood cells. Normally, the neutrophil count is above 2000/mm3.

• Signs and Symptoms– There are no real symptoms

associated with neutropenia.

• Diagnostic and laboratory tests– Neutrophil count less than 1000 to

1500.– Bone marrow examination to examine

cell morphology helps distinguish the etiologic factor causing the neutropenia.


– If the etiology of neutropenia is drug-induced, discontinuation of the medication is indicated.

– Corticosteroids are used if the etiology is immunologic

– If the etiology is decreased production, growth factors (granulocyte/macrophage colony stimulating factor or GM-CSF) may be used.

– If client develops a fever, identification and treatment of the infection is instituted

• Nursing Management– Monitor for signs of infection.– Monitor temperature elevations– Obtain cultures suspected as sites of infection.– Administer antibiotics as prescribed and

evaluate their effectiveness.– Administer medications that stimulate the

production of neutrophils.– Enforce strict hand washing by all individuals

in contact with the client.– Institute reverse isolation.– Use private room with HEPA filtration if

possible.– Avoid invasive procedures. – Fresh flowers and fruits should not be

permitted in the client’s room.

• Client education– Teach client and family to report

signs of fever – Teach the client and individuals who

come in contact with the client about strict hand washing and reverse isolation procedure.

– Teach client methods to maintain good personal hygiene.

– Explain to the client and family about the condition and the rationale of therapeutic interventions.

Leukemia

• A malignant disorder of the blood-forming tissues of the bone marrow, spleen, and lymph system characterized by unregulated proliferation of WBCs and their precursors.

• Etiology and Pathophysiology

• Basis of the classification of the different types of leukemia

– The type of WBC affected (granulocyte, lymphocyte, monocyte)

– The duration of the disease (acute or chronic)

• If the majority of the leukemia cells are primitive, the leukemia is classified as acute; if the leukemic cells are mostly mature (well differentiated), the leukemia is classified as chronic.

• Classifications– Acute lymphocytic/lymphoblastic

leukemia (ALL) • Peak incidence at 2 to 4 years of age.• Immature granulocytes proliferate and

accumulate in the marrow

– Chronic lymphocytic leukemia (CLL) • More common in men and mainly

between the ages of 50 and 70.• Abnormal and incompetent lymphocytes

proliferate and accumulate in the lymph nodes and spreads to other lymphatic tissues and the spleen. Most of the circulating cells are mature lymphocytes.

– Acute myelogenous/myelocytic leukemia (AML)• All age groups are affected with a peak

incidence at age 60.• There is uncontrolled proliferation of

myeloblasts, which are the precursors of granulocytes. They accumulate in the bone marrow.

– Chronic myelogenous leukemia (CML)• Uncommon in people under 20 years of

age. The incidence rises with age. - There is uncontrolled proliferation of granulocyte resulting in increased circulating blast (immature) cells.

• The marrow expands into long bones because of this proliferation and also extends into the liver and spleen. - In most cases the Philadelphia chromosome, a characteristic chromosomal abnormality, is present.

• Abnormal cells can continue to multiply, infiltrate, and damage the bone marrow, spleen, lymph nodes, liver, kidneys, lungs, gonads, skin, and central nervous system (CNS)

• The bone marrow becomes functionally incompetent with resulting bone marrow suppression

Acute leukemia

has a rapid onset, progresses rapidly, with a short clinical course

If left untreated, death will result in days or months.

The symptoms of acute leukemia relate to a depressed bone marrow, infiltration of leukemic cells into other organ systems, and hypermetabolism of leukemia cells.

Chronic leukemia

has a more insidious onset with a more prolonged clinical course.

Clients with the chronic form of leukemia are usually asymptomatic early in the disease.

The life expectancy may be more than five years.

Symptoms of chronic leukemia relate to hypermetabolism of leukemia cells infiltrating other organ systems.

The cells in this type of leukemia are more mature and function more effectively.

• Clinical Manifestations

•Fever

•Night sweats

•Bleeding

•Ecchymoses

•Lymphadenopathy

•Weakness

•Fatigue

•Pruritic vesicular lesions

•Anorexia

•Weight loss

•Shortness of breath

•Decreased activity tolerance

•Bone or joint pain

•Visual disturbances

•Gingival bleeding

•Epistaxis

•Pallor

•Splenomegaly

•hepatomegaly

• Diagnostic and Laboratory Tests– Increased WBC (in CLL and CML) – A normal, decreased or increased WBC

(in ALL and AML). – Decreased reaction to skin sensitivity

tests (anergy). – Bone marrow tests reveal excessive

blast cells in AML – Philadelphia chromosome found in 90%

to 95% in clients with CML. BCR/ABL gene is present in virtually all clients with CML.

– Bone marrow biopsy and aspirate is the definitive diagnostic test.


– Induction of remission with chemotherapy and radiation therapy.

– Bone marrow and stem cell transplantation.

• Nursing Management

– Provide for diversionary activities.

– Maintain good nutrition. Enlist the assistance of a dietician in maximizing and meeting the nutritional needs of the client.

– Assist the client in maintaining good personal hygiene. Measures to promote oral hygiene should be instituted.

– Refer client and family to appropriate agencies such as Meals on Wheels, American Cancer Society, and the Leukemia Society.

– Provide emotional support to the client and family. Refer to appropriate agency, organization, or professional for counseling and support.

– Administer drugs that are prescribed and monitor for side effects. - Monitor laboratory results to evaluate effectiveness of interventions and therapy

– Prepare the client for bone marrow transplantation if this is included in the treatment plan.

– Review and institute the care of a client receiving chemotherapy

– Review and implement the nursing care of a client undergoing radiation therapy

– Assist in bone marrow biopsy. Apply pressure on the site for five minutes or until bleeding stops. Frequently assess the site for signs of bleeding up to four hours after the procedure.

– Plan activities to prevent fatigue. Provide measures for uninterrupted rest and sleep.

– Institute neutropenic and bleeding precautions

• Medication Therapy– alkylating agents

• Busulfan [Myleran]• Anthracyclines Doxorubicin [Adriamycin]

– Antimetabolites• Fludarabine [Fludara]

– corticosteroid • Prednisone

– plant alkaloids• Vincristine [Oncovin]

Malignant Lymphomas

• Lymphoma is a group of malignant neoplasms that affects the lymphatic system resulting in the proliferation of lymphocytes.

• Lymphomas can be classified as:

– Hodgkin’s diease

– Non-Hodgkin’s lymphoma

Hodgkin’s Disease• Etiology and Pathophysiology

– More common in men and has two peaks ; 15-35 years of age and 55 to 75 years of age.

– Incidence is higher in whites than in African Americans

– Cause unknown– Several factors have been identified to

contribute:• infection with the Epstein-Bar virus (EBV)• familial pattern• exposure to toxins

– Characterized by the presence of Reed-Sternberg cell

– The tumor originates in a lymph node (in majority of cases from the cervical nodes) and infiltrates the spleen, lungs, and liver

• Clinical Manifestations– Usually begins with a firm and painless

enlargement of one or more lymph nodes on one side of the neck.

– Fatigue– Weakness– Anorexia– Dysphagia– Dyspnea– Cough– Jaundice– Abdominal pain– Bone pain– PruritusDevelopment of severe but brief

pain at the site of Hodgkin’s after ingestion of alcohol.

• B Symptoms– Fever without chills– Night sweats– Unintentional 10% weight loss– Enlarged lymph nodes– Splenomegaly– Hepatomegaly

• Diagnostic and Laboratory Tests– Normocytic, normochromic anemia -

Neutrophilia, monocytophilia, and lymphopenia

– Presence of Reed-Strenberg cells in excisional bone biopsy

– Mediastinal lymphadenopathy revelaed by chest x-ray, CT scan, and radioisotope studies.

– Mediastinal mass and pulmonary infiltrates may be seen on chest x-ray

– Absent or decreased response to skin sensitivity testing known as anergy.

• Therapeutic Management– Lymphangiography is used to evaluate

abdominal nodes. – Staging laparotomy is performed to obtain

specimen of retroperitoneal lymph nodes and to remove the spleen.

– Stage I indicates involvement of a single lymph node region

– Stage IV indicates diffuse or disseminated involvement of 1 + extralymphatic organs, with or without lymph node involvement (liver, lung, marrow, skin).

– Radiation therapy for stages IA, IB. IIA, and IIB. - Combination chemotherapy for stages III, IV and all B stages.

– Combination radiation and chemotherapy for stages IA and IB.

• Nursing Management– Institute nursing interventions for clients on

chemotherapy or radiation therapy.– Assist in balancing activity with periods of

rest - Provide and assist in maintaining good nutritional state

– Provide measures to diminish the discomfort associated with pruritus

– Provide interventions to enable client to deal with body image changes such as alopecia, weight loss, and sterility.

– Refer client and family to appropriate agencies for support.

– Plan interventions for the prevention of infection.

• Medication therapy– Chemotherapeutic agents – Biologic therapy agents

• Client Education– nature of the disease, the course of

therapy and associated interventions.– medications prescribed, preacautions,

and side effects.– symptoms necessitating immediate

medical intervention such as the occurrence of bleeding, infection, or fever

Non-Hodgkin Lymphoma

• Etiology and Pathophysiology– Most common form of lymphoma.– Affects usually adults from 50 to 70

years old. – More common in men than women

and in whites – No known cause but is linked to viral

infections, immune disorders, genetic abnormalities, exposure to chemicals, and infection with Helicobacter pylori

– Has a similar pathophysiology to Hodgkin’s disease although Reed-Sternberg cells are absent and the method of lymph node infiltration is different.

– In majority of cases the disease involves malignant B cells

– Lymphoma usually originates outside the lymph nodes.

– The lymphoid tissues involved become infiltrated with malignant cells.

– The cells that make up the lymphoid tissue become abnormal and crowd out normal cells.

• Clinical Manifestations– Oainless lymoh node enlargement– B symptoms– Hematuria– Nausea– Vomiting– Abdominal pain– Peripheral neuropathy– Cranial nerve palsies– Headaches– Visual disturbances– Changes in mental status and seizures– Shortness of breath, cough and chest

pain

• Diagnostic and Laboratory Tests– Lymphocytopenia– X-ray may reveal pulmonary

infiltrates.– Lymph node biopsy helps to identify

the cell type and pattern

• Therapeutic Management– Staging of the disease is undertaken.

This is based on data obtained from CT scans and bone marrow bipsies

– Combination chemotherapy

– Radiation alone or in combination with chemotherapy for stage I and II.

– Biologic therapy with alpha interferon, interleukin-2, and tumor necrosis factor.

– Administration of rituximab (Rituxan), a monoclonqal antibody against the CD20 of malignant B lymphocytes, which causes cell lysis and death

• Nursing Management and Client Education same as Hodgkin’s disease.

Documents

Hematology