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Autoimmune Hemolytic Anemias

AB
Autoimmune Hemolytic Anemia (AIHA)Acquired disorder characterized by premature erythrocyte destruction due to abnormal production of antibodies against the patient's own RBC antigens
Compensated AnemiaRate of RBC production equals the rate of RBC destruction
Uncompensated AnemiaRate of RBC destruction exceeds the rate of RBC production
Uncompensated anemia laboratory test resultsIncreased reticulocyte count;increased bilirubin and LDH; decreased Haptoglobin
Types of AIHA1. WARM- 70% - autoantibody optimally reactive 30-37C. 2. COLD - 18% - reactive 4-30 C. 3. DRUG INDUCED - 12%.
Characteristics of benign cold autoantibodies1) Reactive RT or below 2) Titer less than 64. 3) Common specificity anti-I,-IH. 3) DAT positive due to C3 usually 1+ or less. 4) Not clinically significant
Characteristics of Pathologic cold autoantibodies1) Reactive 30-37C. 2) Titer >1000. 3) Common specificity = anti-I. 4) DAT positive due to C3 2-4+. 5) Clinically signifiant 6) Secondary to viral infections i.e. M. pneumonia
Treatments for WAIHA1) Corticosteroids 2) IVIG 3) Splenectomy - when Steroid therapy fails 4) Immunosuppressive Drugs - last resort
WAIHA1) Caused by IgG autoantibody reactive at 37C. 2) RBC destruction is extravascular via the reticuloendothelial system. 3) DAT positive due to IgG or IgG and C3
Cold Hemagglutin Disease (CHD)1) Occurs predominantly in older individuals. 2) Anti-I is the most common specificify. Anti-i is less common and rarely anti-Pr. 3) DAT positive due to anti-C3. 4) Treatment: stay in warm climate and transfusion support if needed
Paroxysmal Cold Hemoglobinuria (PCH)1) Least common 1% of all AIHA 2) Seen most commonly in children following viral infection 3) Biphasic hemolysin - IgG antibody attaches to RBC's at lower temps., binds complement, RBC's lyse as blood circulates to warmer core body temperatures. 3) Eluate negative 4) Antibody specificity is anti-P. 5) Donath Landsteiner Test is diagnostic
Drug Induced Immune Hemolytic Anemia (DIIHA)Rare - 1/1,000,000
Drug induced antibody production1) Antibody against the membrane bound drug (Drug-adsorption (Hapten) Mechanism) 2) Antibody against an RBC membrane component 3) Antibody against a drug/RBC component (Immune Complex Mechanism)
Drug Induced Causes of DAT1) Antibody production. 2) Non-immunologic protein adsorption (NIPA) onto the RBC membrane
Serologic Classification of Drug - Induced Antibodies1) Drug dependant - drug must be present in the test system. 2) Drug Independant - autoantibody induced by drug but invitro reactivity does not require presence of the drug
Drug dependant antibody characteristics1) Antibodies reactive only with drug-treated RBC's i.e. penicillin and some cephalosporins. 2) Antibodies reactive with untreated RBC's suspended in a solution of the drug i.e. quinine and ceftriaxone
Drug independant antibody characteristics1) Drug action alters T-cell function that results in failure to recognize "self" antigen as self but rather as foreign. 2) Drug examples: methyldopa, fludarabine 3) Serologically cannot distinguish drug independant autoantibodies from idiopathic warm autoantibodies


Bloodworks Northwest
Seattle, WA

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