Transfusion Disorders

8/19/2019 Transfusion Disorders

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Haematological Disorders

B. Pimentel, M.D.University Of MakatiCollege of Nursing


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The Red Blood Cell

•Transports oxygen, called oxyhemoglobin, when it gives upits oxygen it is deoxyhemoglobin.

•Also binds and transports carbon dioxide,

carbaminohemoglobin.

•Makes up ± 97 % of RBC→ ± 250 million Hb molecules perRBC

•Men: 14-18 mg/dl blood

•Women: 12-16 mg/dl blood


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The Red Blood Cell

Types of Hemoglobin (Hb)

•Hb A

–96% of adult Hb (α2 β2)

•Hb A2

–3% of adult Hb (α2 σ2)

•Hb F

–1 % of adult Hb (α2 γ2)


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The Red Blood Cell


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The Red Blood Cell


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The Red Blood Cell


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Transfusion Therapy

Blood

group

Antigen Antibody Donor to Recipient

from

A A Anti-B A A, O

B B Anti-A B B, O

AB AB Neither AB A, B, AB, O

O Neither Anti-

A/Anti-B

O, A, B, AB O

Universal donor "O"Universal recipient "AB"


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Adverse Transfusion Reactions

Acute Hemolytic Reaction•Symptoms–Fever, chills and fever, the feeling of heat along thevein in which the blood is being transfused–Pain in the lumbar region–Constricting pain in the chest, tachycardia, hypo-tension–Hemoglobinemia with subsequent hemoglo-binuria

and hyperbilirubin-emia.•"feeling of impending doom"


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Acute Hemolytic Reaction

•Causes

–Human error!

•Transfused red cells react with circulating antibody in therecipient with resultant intravascular hemolysis


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Acute Hemolytic Reaction

•Frequency

–Rare

•Prevention

–Proper identification of patients, pretransfusion bloodsamples and blood components at the time oftransfusion


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Delayed Hemolytic Reaction•Falling hematocrit–due to extravascular destruction of the transfused red bloodcells)

•Positive direct antiglobulin (Coombs) test (DAT)

•Occurs about 4-8 days after blood transfusion

•Patients may manifest fever and leukocytosis–Appearing to have an occult infection.


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Febrile Transfusion Reaction

•Fever or chill fever

–temperature rise of 1.5 F or 1.0 C from the baseline

•Cytokines and antibodies to leukocyte antigensreacting with leukocytes or leukocyte fragments

•1 in 8 transfusions


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Allergic – urticaria

•Laryngeal edema and bronchospasm

•1% of recipients –If coupled with another sign, such as fever, evaluationfor a hemolytic reaction may be indicated.


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Allergic – Anaphylaxis

•Anaphylactic or anaphylactoid

–Respiratory involvement with dyspnea or stridor

•Cardiovascular instability

–hypotension, tachycardia, loss of consciousness,

cardiac arrhythmia, shock and cardiac arrest


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Volume Overload •Transfusion-related volume overload

•Infuse smaller volumes more slowly


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Bacterial Contamination

•Hypotension, shock, fever and chills, nausea andvomiting, and respiratory distress

•Gram stain and blood culture


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IF A TRANSFUSION REACTION ISSUSPECTED

Follow protocol for transfusion

reactions implemented by theinstitution


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•Stop the transfusion immediately!

•Disconnect the intravenous line from theneedle.

•Seek medical attention immediately. If the

patient is suffering cardiopulmonary collapse,and medical attention is not immediatelyavailable, press for “Code"


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•Check to ensure that the patient name andregistration number on the blood bag labelexactly with information on the patient's

identification

•Do not discard the unit of blood that has been

discontinued because it may be necessary for theinvestigation of the transfusion reaction.


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Treatment for Transfusion Reactions

Reaction Type Treatment - Adult Pediatric Follow-up

Acute

HemolyticReactions

Diuretic therapy: Initially, give 40-80mg Furosemide (Lasix)intravenously. This dose can ere!eated once. Lac" o# res!onse to#urosemide in $-% hours indicatesthe !resence o# acute renal #ailure.

&ediatric dose' -$mg"gdose.*ay re!eat onceat $-4 mg"g.

Treat shoc" and disseminatedintravascular coagulation +itha!!ro!riate measures i# and +henthey a!!ear.

Water loading: The !atient should ehydrated to maintain urinaryout!ut o# at least 00 mLhr untilurine is #ree o# hemogloin.

In#use a loading dose o# 0. sodiumchloride or dextrose in 0.4sodium chloride. /hart hourlyurine out!ut. *aintain the urine

out!ut y administeringintravenous #luid at 00 mLhouruntil the urine is #ree o#hemogloin. I# the !atientsurinary out!ut does not increase,+ith this hydration any additional#luids should e in#used +ithcaution.

&ediatric !atientsshould receive asmaller loadingvolume o# #luidin !ro!ortion totheir odysur#ace area.


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DelayedHemolytic

TransfusionReactions

1!eci#ic treatment generally is notnecessary

1u!!lemental trans#usion o# lood lac"ingthe antigen corres!onding to theo##ending antiody may e necessaryto com!ensate #or the trans#used cells

that have een removed #rom thecirculation.



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AllergicTransfusion

Reactions

Antihistamines(e.g., 2enadryl). 3ive0-00 mg orally or intravenously. I#urticaria develo!s slo+ly,antihistamines may e given orally.

&ediatric dose' -$mg"g intramuscularlyor intravenously #or$-0 mg !er averagedose.

outine use o# 2enadryl as !remedication#or all trans#usions, regardless o# a historyo# allergic reactions, is discouraged.

Aminophylline #or +hee5ing, at a doseo# $-$0 mg intravenously slo+lyover a !eriod o# aout #ive minutes

&ediatric dose' %mg"gdose inintravenous dri! overo# $0 minutes.

Epinephrine #or severe, acute reactionsincluding laryngeal edema or

ronchos!asm 3ive 0.-0. mg (0.-0.mL o# a '000 solution)sucutaneously. 1ucutaneous dosemay e re!eated at 0- minuteintervals. The total sucutaneous dosein a $4-hour !eriod, +ith rareexce!tion, should not exceed mg.

&ediatric dose' 0.0%mL*$ (0.0% mg*$

o# a '000 solution)given sucutaneously.6 single !ediatricdose should notexceed 0.% mg.


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FerileTransfusion

Reactions

Premedicate the !atient +ithacetamino!hen or otheranti!yretic agents +hen !reviousreactions have een extremely

othersome. &ediatric dose' 0mg"g to a maximum o# 700 mg.

6s!irin +ill adversely a##ect the !atients !latelet #unction, so non-as!irinanti!yretic agents are !re#erale.

!e"ere sha#ing$hills

(rigors) can e controlled y thesedative e##ect o# 2enadryl oremerol ($-0 mg givenintramuscularly or intravenously

9ote' emerol may cause acuteres!iratory arrest. 6n o!iateantagonist (9arcan) should eimmediately availale.

!epsis Due to%acterial

$ontaminationof Donor %lood

Treatment o# se!tic shoc" includes'terminating the sus!ected

trans#usion immediately, cardio-vascular and res!iratory su!!ort,

lood culture o# the !atient, andadministration o# road s!ectrumantiiotics including anti-

!seudomonas coverage i# the lood com!onent involved is ed2lood /ells&



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Anemia

•Abnormally low number of RBC or Hb levels

•Reduced oxygen carrying capacity

Causes

•Blood loss

•Increased rate of red cell destruction

–Hemolytic anemia

•Deficient or impaired red cell production


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Anemia

Risk factors

•Poor diet

•Intestinal disorders

•Menstruation•Pregnancy

•Chronic conditions

•Family history


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Anemia

•“NOT A DISEASE” but a symptom

–Dependent on severity, speed of development, age,health status and compensatory mechanisms

–Associated with impaired O2transport, alteration inRBC structure or with chronic illness

–Not expressed until 50% of RBC mass is lost


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Anemia

Signs and symptoms•The main symptom of most types of anemia isfatigue–Weakness–Pale skin–Tachycardia–Shortness of breath–Chest pain

–Dizziness–Cognitive problems–Numbness or coldness in your extremities–Headache


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Anemia

Iron Deficiency Anemia•Most common form of anemia–Affects about one in five women

–Half of pregnant women and 3 percent of men in theUnited States.

•The cause is a shortage of the element iron–Nutritional imbalance–Slow, chronic bleeding disorders

–Inability to recycle plasma iron


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Anemia

Vitamin Deficiency Anemias

•Folate and vitamin B-12 deficiency

•Intestinal disorder that affects the absorption of

nutrients•Fall into a group of anemias called megaloblasticanemias, in which the bone marrow produceslarge, abnormal red blood cells.


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Anemia

Anemia of Chronic Disease

•Interfere with the production of red blood cells,resulting in chronic anemia

•Kidney failure also can be a cause of anemia–The kidneys produce a hormone callederythropoietin, which stimulates your bone marrowto produce red blood cells.•A shortage of erythropoietin, which can result from kidneyfailure or be a side effect of chemotherapy, can result in ashortage of red blood cells.


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Anemia

Aplastic Anemia

•Life-threatening anemia caused by a decrease inthe bone marrow's ability to produce all three

types of blood cells — red blood cells, white blood cells and platelets

•Cause of aplastic anemia is unknown–autoimmune disease

–Chemotherapy–Radiation therapy

–Environmental toxins


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Anemia

Anemias associated with bone marrow disease•Leukemia and myelodysplasia, can causeanemia by affecting blood production in the

bone marrow•Effects vary from a mild alteration in bloodproduction to a complete, life-threateningshutdown of the blood-making process–Myelodysplasia is a pre-leukemic condition

that can cause anemia.•Other cancers of the blood or bone marrow, suchas multiple myeloma, myeloproliferative disordersor lymphoma, can cause anemia.


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Anemia

Hemolytic Anemias

•Red blood cells are destroyed faster than bonemarrow can replace them.

•Autoimmune disorders can produce antibodiesto red blood cells, destroying them prematurely–Hemolytic anemias may cause yellowing of the skin(jaundice) and an enlarged spleen.


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Anemia

Hereditary Spherocytosis

•Mutations in the ankyrin molecule with asecondary deficiency of spectrin along the cell

membrane–Reduced red cell stability•Does not affect oxygen carrying capacity

•Splenic sequestration


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Anemia

Sickle cell anemia•Defective form of hemoglobin that forces red blood cells to assume an abnormal crescent(sickle) shape.–Mutation for the gene coding for the β-globulin chain•Valine is substituted for glutamic acid HbS

•Red cells die prematurely, resulting in a chronicshortage of red blood cells.–Block blood flow through small blood vessels in the body, producing other, often painful, symptoms.


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Anemia

α- Thalassemia

•Common in Asians

•Deletion of glubulin chain loci

•4 possible degrees of α thalassemia:

–Silent carrier, loss of a single α globulin gene

–α thalassemia trait, loss of a pair of globulin gene

–HbH disease, only a single gene is present–Hydrops fetalis, deletion of all α globulin


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Polycythemia Vera

•An acquired disorder of the bone marrow thatcauses the overproduction of all three blood celllines

–white blood cells, red blood cells, and platelets•It is a rare disease that occurs more frequently inmen than women, and rarely in patients under40 years old.

•causes is unknown


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Polycythemia Vera

•Usually develops slowly, and most patients areasymtomatic

–abnormal bone marrow cells proliferate

uncontrollably leading to acute myelogenousleukemia

•Patients have an increased tendency to form blood clots that can result in strokes or heart

attacks–Some patients may experience abnormal bleeding because their platelets are abnormal


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Polycythemia Vera

Symptoms •Headache

•Dizziness

•Pruritus•Fullness in the left upper abdomen

•Erythema (face)

•Shortness of breath•Orthopnea

•Symptoms of phlebitis


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White Blood Cells

•Collectively known as White Blood Cells (WBC)

•Formed elements of the blood with organelles

and a nucleus but lack hemoglobin

•Protect the body against microorganisms andremove dead cells and debris from the body


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White Blood Cells


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White Blood Cells

Per µl blood Per µl of blood

Total WBC count 5,000 – 10,000

Neutrophils 50 - 70% 2,000 – 7,000

Lymphocytes 20 - 40% 1,000 – 4,000Monocytes 1 – 6% 50 – 600

Eosinophils 1 – 5% 50 – 500

Basophils 0 – 2% 0 - 100


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WBC Disorders

•Leukopenia

–Decreased peripheral white cell count due to decreasenumbers of any specific types of leukocytes

•Leukocytosis–Non–neoplastic elevation of WBC count


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WBC Disorders

Neutropenia

•Reduction in the number of granulocytes(


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WBC Disorders

Neutropenia

•Decreased or defective granulopoiesis

–Aplastic anemia

–Anti-neoplastic agents–Other drugs: chloramphenicol, sulfonamides,chlorpromazine

•Accelerated removal or destruction

–Aggressive and chronic infections


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WBC Disorders

Manifestation of Neutropenia

•Infections

Signs and Symptoms

•Malaise, chills, fever

•Ulcerative necrotizing lesions of the mouth, skin

vagina and GI tract


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WBC Disorders

Reactive Leukocytosis

•Increase number of WBC

•Common reaction due to a variety of

inflammatory states caused by microbial or non-microbial stimuli

•Usually non-specific


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WBC Disorders

Causes of Leukocytosis

Polymorphonuclear leukocytosis Acute bacterial infections

Eosinophilic leukocytosis Allergic disorders

Monocytosis Chronic infections

Lymphocytosis Chronic immunologic disease


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Neoplastic Proliferation of White Cells

1. Leukemia – neoiplasms of the hematopoieticstem cells

2. Malignant lymphomas – cohesive tumor

lesions; neoplastic lymphocytes3. Plasma cell dyscrasias – arising from the bones; localized disseminated proliferation ofantibody forming cells

4. Histocytoses – proliferative lesions ofhistiocytes


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Leukemia

•Malignant neoplasm of the hematopietic stemcells

•BM replaced by unregulated, proliferating,immature neoplastic cells blood leukemia enter spleen, lymph nodes

•Most common cancer in the paediatric age•Leading cause of death in children between 3and 14 years old


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Classification of Leukemia

A.According to cell type and state of cellmaturity

• Lymphocytic – immature lymphocytes and theirprogenators

• Myelocytic – pluripotent myeloid stem cells andinterferes with maturation of all granulocytes, RBCand platelets

B. Acute or Chronic• Acute – immature cells (blast)

• Chronic – well differentiated leukocytes


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Acute Leukemia (Cell Kinetic Studies)

•Block in the differentiation of leukemic cells withprolonged genration time clonal expansion ofthe transformed stem cells + failure of

maturation accumulation of leukemic blast

suppress normal hematopoietic stem cells


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Acute Leukemia

Features

•Sudden onset (3 months)

•Depressed marrow function

•Bone pain and tenderness

•Generalized lymphadenophaty

•Splenomegaly, hepatomegaly

•CNS: headache, vomiting


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Acute Lymphocytic Leukemia (ALL)

• Most common leukemia in children (80%)

• Treatable and potentially curable

• Classified according to lymphocytes and state

of maturation1. Early B cell

2. Pre-B cell

3. Mature B cell

4. Early T cell

5. Mature T cell


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Acute Myleocytic Leukemia (AML)

•Acute Non-lymphocytic Leukemia (ANLL)

•Most common in adults; >50% 60years old

•70% of adults will enter remission with

induction chemo–25-35% of those in remission will have a 5 yearsurvival rate

•BM transplant


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Acute Myleocytic Leukemia (AML)

Treatment

• Selective radiation

• Chemotherapy

1. Induction

2. Intensification

3. Maintenance and consolidation

• Bone marrow transplant


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Chronic Leukemia

•Insidious onset

•Incidental findings during routine exam


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Chronic Lymphocytic Leukemia

•Proliferation and accumulation of maturelymphocytes which are immunologicallyincompetent

–B cell line (US)–T cell line (Asia)

•Hairy cell leukemia


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Chronic Myelocytic Leukemia

•15% of all leukemias

•Chromosomal abnormality (Ph1)

•Mostly B cell disease

–Leukocytosis–Splenomegaly

–Hepatomegaly

–Lympadenopathy

•Bone marrow transplant 5 year survival for50-75% of patients


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Chronic Myelocytic Leukemia

Two distinct phases

•Chronic

–Last about 3-4 years

–Near end accelerated phase: fever, night sweats,malaise

•Acute

–2-4 months

–Poor prognosis, palliative management


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Malignant Lymphomas

•Primary solid tumors of the lymphoid system

•Cancers involving lymphocytes duringmaturation or storage in the bone marrow

•Third most common malignacy in children


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Malignant Lymphomas

Hodgkin’s Lymphoma

•Disorders primarily involving the lymphoidtissues

•Anatomical spread•Morphological presence ofReed-Sternberg cells

•60-90% cure rate


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Malignant Lymphomas

Manifestations of Hodgkin’s

•A symptoms

–Painless progressive enlargement of a single or group

of nodes (neck)–May spread continuously through out the lymphaticsystem

•B symptoms

–Fever, night sweat, weight loss

–Fatigue, anemia


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Malignant Lymphomas

Treatment for Hodgkin’s

•Radiation

•Chemotherapy


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Malignant Lymphomas

Non-Hodgkin’s Lymphoma

•Involves lymphoid tissue and may spread tovarious tissues

•Mostly B cell (80%)•Cause may be viral or genetic

–EBV

–Immunosuppresed patients•AIDS•After organ transplant


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Malignant Lymphomas

Treatment

•Early stage radiation

•Late stage chemo and radiation

•BM transplant

Documents

Transfusion Disorders