Management of Clients with Hematologic Disorders

NRS 108

Majuvy L. Sulse RN, MSN, CCRN

HEMATOLOGIC DISORDERS

Disorders associated with ErythrocytesDisorders of bleedingDisorders associated with white blood

cellsLymphomas

ANEMIA(decreased in number of RBCs)

Low Hgb

Less 02 carrying capacity

Hypoxia

H/H, Bone marrow aspiration, Peripheral smear

( Diagnosis)

Abnormal & deficient production, blood loss, destruction of RBC

( Causes)

Pallor, Fatigue, Palpitation, Low BP, SOB, DOE, MI, Renal failure

Management of Anemia

Medical: Identify cause Treat cause Relieve

symptoms Prevent

complications

Nursing: Assess Educate

Classification of Anemia

Based on the size of the RBCNormocyticMicrocyticMacrocytic

Iron Deficiency Anemia

Causes: Inadequate iron supply Chronic blood loss without iron replacement Decreased iron absorption in the intestines

Signs/ Symptoms: Hypochromic (low MCH), microcytic ( low MCV) Elevated serum binding capacity Brittle, spoon-shaped nails with longitudinal ridges Cheilosis (painful mouth cracks/ sores) Red shiny tongue Insidious development of fatigue

Diagnosis/Treatment

Treatment: Identify cause

Diagnosis: Laboratory values Gastroscopy Sigmoidoscopy Occult blood in stools Radiographic studies

of GI Iron supplement Nutritional/dietary

Megaloblastic Anemia

Predominance of megaloblasts & lack of normoblasts Includes pernicious anemia, Vit. B12 & Folic acid

deficiencies Related to surgery particularly of terminal ileum where

B12 is absorbed; vegetarian diet; prolonged exposure to nitrous oxide

Related to aging & long term gastritis Related to alcohol malnutrition & malabsorption (Folic

acid deficiency)Macrocytic, normochromic RBC

Lack of Intrinsic factor Autoimmune response

Diagnosis & Treatment: (Megaloblastic Anemia)

Schilling test- definitive dx of pernicious anemia

Gastric secretion analysis- pH, free HCL, low gastric secretion

Diagnosis & Treatment :(Megaloblastic Anemia)

Management- Lifelong tx with Vit B12 injection Iron & Folic acid supplement Nutritional/ dietary changes

Foods that are rich in folic acid and vitamin B12 include the following:

eggs meat poultry milk shellfish fortified cereals

Aplastic Anemia

Low Hb & pancytopenia Unknown etiology / autoimmune disturbance Direct injury by Myelotoxins- agents causing bone marrow

damage when received in large doses Medication induced:

Chemo, chloramphenicol, sulfonamides, mephenytoin, quinine

Exposure to environmental hazards: Benzene, insecticides, radiation & radioactive

materials Infections

Hepatitis, miliary TB, EPBV Congenital/hereditary causes

Aplastic Anemia-Manifestations & Dx

Manifestations- Exertional dyspnea, fatigue, pallor, infections Bleeding (nasal,oral, rectal,vaginal) ecchymosis,

petechiae, pupura Low platelets (less 30, 000), RBCs, WBCs Dry bone marrow on aspiration

Diagnosis- Differential count Manifestations History of exposure to myelotoxins

Aplastic Anemia

Medical Remove causative

Idiopathic cause- treat with steroids, hormone therapy

Autoimmune cause- bone marrow transplants (younger than 30 or those who have not received transfusions yet

Antithymocyte & cyclosporine therapy- skin testing & watch for allergic reactions

Blood transfusions Frequent CBCs esp for those on radiation therapy

Nursing management Infection control Client education

Anemia from Blood Loss

Types Acute-trauma, complications from surgery

Hypovolemia, hypotension, hypoxemia, weakness, tachycardia, stupor

Chronic- bleeding ulcer, hemorrhoidsGradual and vague symptoms as fatigue, pallor,

dyspnea Medical/ Nursing Interventions

Identify source of bleeding Control through medical or surgical interventions Blood transfusions

Hemolytic Anemia

Abnormal destruction of RBCs by Intrinsic-defective RBCs, enzyme deficit(G6PD) extrinsic factors- toxins, injury as in prosthetic heart

valves Failure of bone marrow to replace destroyed RBCs Sites of hemolysis:

Intravascular-circulation Extravascular- macrophages of spleen, liver & bone

marrow

Findings/ Treatment

Findings Normocytic

anemia Reticulocytosis as

compensatory mechanism

Increased RBC fragility

Short lifespan Hyperbilirubinemia

- blood, urine, stools

S/S as in Anemia

Treatment Identify/Treat

cause IV fluids to flush

kidneys NAHCO3 or Na

Lactate to alter urine pH (decrease) precipitation in renal tubules

Splenectomy

Sickle Cell Anemia and Sickle Cell Trait

Inherited, autosomal, recessive disorders of HB synthesis resulting in decrease O2 to the tissues (hypoxia) and obstruction of blood vessels

Substitution of valine for glutamic acid in B-globin gene

Primarily seen in the black population Sickle cell trait is a mild form of the disease and it is

the commonest Sickle cell trait is prevalent in Africa Resistant to the parasite that causes malaria Genetically inherited from each parent

Inheritance

Pathophysiology

Sickling of RBC-triggered by Hypoxia- low O2 tension in blood

High altitude Emotional/ physical

stress Surgery Blood loss Infection-bacterial & viral

Dehydration Acidosis Decreased plasma volume-

Increased blood viscosity Hypothermia Stress

Pathophysiology

Sickled cell-rigid & elongated causes tissue injury (as cells can’t pass through small vessels) results in local hypoxia anemia as more cells are hemolyzed by spleen

Initially reversible with re-oxygenation then becomes irreversible due to cell membrane damage from recurrent sickling

Sickle Cell crisis

Exacerbation of RBC sickling Vaso- occlusion (Vaso-occlusive crises) -

vasospasm Changes in membrane permeability plasma loss

& hemoconcentration development of thrombi, tissue ischemia, infarction, necrosis

Shock is a life threatening consequence Frequency, extent, & severity of episode is

dependent on percentage of HbS present

Causes of Sickle Cell Crisis

Vaso - OcclusionAplastic crisisHemolytic crisisSequestration crisisMixed crisis

Types of Sickle Cell Disease

Sickle cell anemiaSickle cell ThalassemiaSickle cell HbC-

Clinical Manifestations

Cardiovascular changesSkin changesAbdominal changesMusculoskeletal changesCentral nervous system changes

Clinical Manifestation

Noticed after 6 months when fetal Hb is no longer present

Improper growth related to anemia Retarded growth and delay in sexual maturity Hand- foot syndrome- edema of hands and

feet Pain from tissue ischemia-hands, feet , joints

Clinical Manifestation

Weakness and fatigue-exercise intolerance

Jaundice- development of gallstonesPallor-of mucous membrane-grayish

cast on skinPriapism-occlusion of penile veinsInfarct on the spleen - small and scarredLeg ulcers in about 75% of cases

Complications

CHF from ischemia & heart enlargement

Acute Chest syndrome-fever, chest pains, cough, dyspnea

Pulmonary infarct- pulmonary HTN, MI, Cor Pulmonale

Blindness from retinal obstruction-hemmorhage-detachment

Renal failure- hemoconcentration

Autosplenectomy-shrinking of spleen from repetitious scarring

CVA- thrombus formation

Osteoporosis/ Osteosclerosis

Leg ulcers esp. at ankles-tissue hypoxia

Infection- absence of phagocytic activity by spleen

Diagnostic findings

Peripheral SmearSickling TestDNAElevated serum bilirubin levelsSkeletal xrays-reveal bone/joint

deformities & flatteningMRI-check for clots (CVA)

Medical/ Nursing Management

No cureSupportive interventions: pain relief,Hydration, O2, rest, blood transfusionsPatient Teaching to avoid factors that cause

crisisAssessment of family understanding of the

disease & coping mechanismLinks for supportGenetic counseling

Get Involved Class!

What nursing diagnosis should receive the highest priority in a client with sickle cell crisis?

A nurse is preparing a teaching plan for a sickle cell client, what should the nurse emphasize on to prevent sickle cell crisis?

Polycythemia Vera

Excessive production of erythrocytes, leukocytes, platelets due to excessive activation of pluripotent stem cells

ManifestationsHTN- headache, vertigo, tinnitus, dizziness,

visual disturbancesCHF- angina- hypervolemia & viscosity Intermittent claudication- thrombophlebitis

Polycythemia Vera

Stroke- thrombi formationPruritus- histamine release from basophilsHemmorrhage- vessel rupture from tissue

distentionHepatomegaly & Slenomegaly from organ

engorgementPlethora- Ruddy complexionGout—hyper uric acid production from RBC

destruction

Diagnosis

RBC=8-12 millionHgb=18-25 gmIncreased platelet countHCT= >54% in men; 49% in womenElevated WBCs with basophiliaIncreased serum uric acid, B12SplenomegalyHyperplastic bone marrow

Medical/Nursing Management

Goal of treatment isReduce blood volume & viscosity

Phlebotomy to Hct levels of 45-48% (about 300-500ml/day)

Reduce bone marrow activity- myelosuppressive agents & radioactive phosphorous

Hydration therapy with I & O

DISORDERS OF BLEEDING- THROMBOCYTOPENIA

Decreased production of platelets below 150,000/uL

Manifests as bleeding- skin bruises easily Maybe

acquired –food, drugs, infections, aplastic anemia

inherited- pancytopenia, hereditary thrombocytopenia

Diagnosis/ Management

CBC shows low platelet & Hb count Assess for hx of NSAIDS Avoid injury Good oral hygiene & skin care Rectal enemas & suppository-avoid constipation Avoid IM, SC injections, rectal temperatures Apply pressure on any bleeding source Monitor signs of bleeding

Immune Thrombocytopenia Purpura- ( ITP)

Autoimmune bleeding disorder Platelets coated with antibodies Destroyed by macrophages in liver & spleen Survival is 1-3 days instead of 8-10 Gradual onset & transient remissions occur

Clinical Manifestations

Petecchiae- small, flat, pinpoint red Purpura-numerous petecchiae Ecchymosis-large purplish lesions Epistaxis- Bleeding gums Heavy menses Complication=hemorrhage

Diagnosis

Platelet Ct<100, 000Prolonged bleeding time with normal

coagulation timeIncreased capillary fragilityPositive platelet antibody testBone marrow aspirate contains normal

or increased megakaryocytes

Management

Cortecosteroids (prednisone)-suppreses the phagocytic response of Spleenic macrophages, depress antibody formation, reduce capillary permeability & bleeding time

IV immunoglobulin (IVIG) Immunosuppressive therapy Splenectomy- Platelet transfusions

Hemophilia

Characterized by prolonged bleeding after surgical/ dental or small trauma or cuts

Types Hemophilia A- factor Vlll-most common-80% cases Hemophilia B- factor lX deficiency-inherited gene Von Willebrand’s disease- deficient Vlll & platelet

dysfunction

Etiology

Sex linked genetic disorderTransmitted by females but males

express the disorderCarriers transmit the gene to half their

daughters and the disorder to half their sons

Males transmit the gene to all their daughters but none to their sons

Clinical Manifestations

Slow persistent bleeding from cuts/scratches Delayed hemorrhage-hours/days after injury Severe bleeding after dental surgery GI bleed Nosebleed Hematoma Prolonged APTT

Treatment

Goal is to stop bleedingIncrease anti-hemophilic factor-give

factor Vlll, lXPrevent complicationsSupport therapy

LEUKEMIA

DESCRIPTION Malignant exacerbation in the number of

leukocytes, usually at an immature stage, in the bone marrow

May be acute, with a sudden onset and short duration, or chronic, with a slow onset and persistent symptoms over a period of years

Affects the bone marrow, causing anemia, leukopenia, the production of immature cells, thrombocytopenia, and a decline in immunity

LEUKEMIA

DESCRIPTIONThe cause is unknown and appears to

involve gene damage of cells leading to the transformation of cells from a normal state to a malignant state

Risk factors include genetic, viral, immunological, and environmental factors and exposure to radiation, chemicals, and medications

CLASSIFICATION OF LEUKEMIA

ACUTE LYMPHOCYTIC LEUKEMIA (ALL) Mostly lymphoblasts present in bone marrow Age of onset is less than 15 years CNS manifestation common-leukemic meningitis Normally, the lymphocytes fight infection by making

antibodies that attack harmful elements. But, in ALL, the cells are immature and overabundant. They crowd out other blood cells, and may collect in the blood, bone marrow, and lymph tissue.

CLASSIFICATION OF LEUKEMIA

ACUTE MYELOGENOUS LEUKEMIA (AML) affects the young blood cells (called blasts) that

develop into a type of white blood cell (called granulocytes). The main function of granulocytes is to destroy bacteria. The blasts, which do not mature and become too numerous, remain in the bone marrow and blood.

Age of onset is between 15 and 39 years

Classification

CHRONIC MYELOGENOUS LEUKEMIA (CML)Mostly granulocytes present in bone marrowAge of onset is after 50 years of ageCML occurs mainly in adults and is rare in

children CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)

Mostly lymphocytes (B cells) present in bone marrow

Age of onset is after 50 years of age,

Diagnosis

CBC-WBC may be normal, decreased or elevated

Decreased plateletDecreased HbBone marrow aspiration- increase in

marrow cells

LEUKEMIA

ASSESSMENTAnorexia, fatigue, weakness, weight lossAnemiaBleeding (nosebleeds, gum bleeding, rectal

bleeding, hematuria, increased menstrual flow)

PetechiaeProlonged bleeding after minor abrasions or

lacerations

Assessment

Elevated temperatureLymphadenopathy and splenomegalyPalpitations, tachycardia, orthostatic

hypotensionPallor and dyspnea on exertionHeadacheBone pain and joint swelling

Leukemia

ASSESSMENTNormal, elevated, or reduced white blood

cell (WBC) countDecreased hemoglobin and hematocrit

levelsDecreased platelet countPositive bone marrow biopsy identifying

leukemic blast phase cells

Leukemia

INFECTIONA major cause of death in the

immunosuppressed clientCan occur through autocontamination or

cross-contaminationCommon sites of infection are the skin,

respiratory tract, and gastrointestinal (GI) tract

Leukemia

IMPLEMENTATION: INFECTION

Initiate protective isolation procedure-also called Neutropenic Precautions or Reverse isolation

Ensure frequent and thorough hand washing Reduce exposure to environmental organisms by

eliminating raw fruits and vegetables (low-bacteria) from the diet, fresh flowers from the client’s room, and by not leaving standing water in the client’s room

Avoid invasive procedures such as injections, rectal temperatures, and urinary catheterization

Leukemia

CLIENT EDUCATION: INFECTION Avoid crowds and those with infections Consume a low-bacteria diet and to avoid drinking

water that has been standing for longer than 15 minutes

Avoid activities that expose the client to infection such as changing a pet’s litterbox or working with houseplants or in the garden

Neither the client nor their household contacts should receive immunization with a live virus

Leukemia

BLEEDING During the period of greatest bone marrow

suppression (the nadir), the platelet count may be extremely low, less than 10,000/mm3 (bleeding risk)

Examine the client for signs and symptoms of bleeding; examine all body fluids and excrement for the presence of blood

Handle the client gently; use caution when taking blood pressures to prevent skin injury

Measure abdominal girth, which can provide an indication of internal hemorrhage

Provide safety-pad side rails and sharp corners of the bed and furniture

Leukemia

IMPLEMENTATION: BLEEDING Provide soft foods that are cool to warm in

temperature Avoid injections if possible to prevent trauma to the

skin and bleeding; apply firm, gentle pressure to a needle stick site for at least 10 minutes

Avoid rectal suppositories, enemas, and thermometers

If the female client is menstruating, count the number of pads or tampons used

Administer blood products as prescribed

Leukemia

FATIGUE AND NUTRITION Assist the client in selecting a well-balanced diet Provide small, frequent meals (high calorie, high-

protein, high-carbohydrate) that require little chewing

Assist the client in self-care and mobility activities Allow adequate rest periods during care; do not

perform activities unless they are essential Administer blood products for anemia as prescribed

Leukemia

CHEMOTHERAPY Induction therapy: Aimed at achieving a rapid,

complete remission of all manifestations of the disease

Consolidation therapy: Administered early in remission with the aim of cure

Maintenance therapy: May be prescribed for months or years following successful induction and consolidation therapy; the aim is to maintain remission

Leukemia

IMPLEMENTATIONAdminister antibiotic, antibacterial, antiviral,

and antifungal medications Blood replacements as neededPrepare the client for transplantationAdminister colony-stimulating factors as

prescribedMaintain infection and bleeding precautions

Leukemia

IMPLEMENTATIONProvide an adequate dietProvide an activity schedule that will

conserve energy Instruct the client in appropriate home care

measuresProvide psychosocial support and support

services for home care

Leukemias

AcuteCombination ChemoInductionRest- 3 weeksConsolidationMaintenace follows remission- lasts 2-3 yearsBone marrow transplant

ChronicTreatment based on symptomsRadiation to spleen, nodesInterferonAutologous stem cell transplantBlood transfusions: RBCs, platelets, WBCs

HODGKIN’S DISEASE

DESCRIPTION A malignancy of the lymph nodes that originates in

a single lymph node or a single chain of nodes; metastasis occurs to other adjacent lymph structures and eventually invades nonlymphoid tissue

Usually involves lymph nodes, tonsils, spleen, and bone marrow and is characterized by the presence of the Reed-Sternberg cell in the nodes

Possible causes include viral infections and previous exposure to alkalyting chemical agents

Prognosis is dependent on the stage of the disease

Etiology & Pathophysiology

Unknown cause Linked to Epstein-Barr Virus Genetics- high among Jews Mechanism of growth & spread- unknown Cancerous cells transforms in lymph and

progresses to other nodes or direct infiltration of blood vessels

Clinical Manifestations

Enlargement of cervical, axillary, inguinal lymph nodes may be painless

B symptoms-Weight loss, Fever, night sweats Cough, stridor, dyspnea Hepatomegaly Splenomegaly SVC syndrome from intra thoracic involvement

Diagnosis & Staging

Peripheral blood analysis-microcytic, hypochromic anemia, leukocytosis

Lymph node biopsy-definitive dxBone marrow examination-staging( l-lV)CXR, CT Scan, radioisotope studies-

define sites Lymphangiography-assess nodes &

vessels

Hodgkins Disease

IMPLEMENTATIONFor stage 1 and 2 without mediastinal node

involvement, the treatment of choice is extensive external radiation of the involved lymph node regions

With more extensive disease, radiation along with multi agent chemotherapy is utilized

HODGKIN’S DISEASE

Implementation Monitor for side effects related to chemo or radiation Monitor for signs of infection & bleeding Maintain infection and bleeding precautions Discuss possibility of sterility with male client

receiving radiation & inform of options related to sperm banks

Pain management Client & family support If terminal- ensure client has dignified death

NHLNON- HODGKIN’S LYPHOMA

Group of malignancies with origins in lymphoid cells affecting all ages

Most common occurring cancer & 5th leading cancer causing death

Men are more affected than women; survival better for women than in men

Incidence higher in whites than other races Painless lymph node enlargement is primary

manifestation Symptoms will present on where disease has

spread

Diagnosis & Treatment

Diagnostic tests are same as for Hodgkin’s disease-Lymph node biopsy done

Treatment-radiation & chemotherapy Nursing assessment for nodes characteristics,

B symptoms appearance Support for client & family Manage risk for infection, bleeding, sexual &

reproductive dysfunction Nutritional management

Multiple Myeloma

Cancer of the plasma cell, an important part of the immune system that produces immunoglobulins (antibodies) to help fight infection and disease.

Hypercalcemia, anemia, renal damage, increased susceptibility to bacterial infection, and impaired production of normal immunoglobulin are common clinical manifestations of multiple myeloma. It is often also characterized by diffuse osteoporosis, usually in the pelvis, spine, ribs, and skull.

Diagnostics/ Management

Diagnostics Radiologic studies- lesions in bones,

demineralization, osteoporosis Bone marrow biopsy- immature plasma

cells Abnormal immunoglobulin Monoclonal immunoglobulin chains in

blood & urine exam

Multiple Myeloma

ManagementChemotherapy, radiation txAutologous bone marrow transplantPlasmapheresisHydration, diuretics, phosphate for treatment

of HypercalcemiaPain managementSupportive for anemia, leukopenia,

thrombocytopenia