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1. WARM ANTIBODY
IDIOPATHIC
SECONDARY- connective tissue diseases, AIDS, antiphospholipid syndrome, SLE, lymphoma, CLL, etc
DRUG-INDUCED
2. COLD AGGLUTININ SYNDROME
IDIOPATHIC
SECONDARY- mycoplasma, mononucleosis, viral infections, lymphoreticular malignancies
3. PAROXYSMAL COLD HEMOGLOBINURIA
IDIOPATHIC
SECONDARY- syphillis, viral illnesses especially in children |
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WARM ANTIBODY AIHA
Diagnosis- Peripheral blood smear shows microspherocytes. Direct coomb's test is positive. Rare cases of coomb's negative autoimmune hemolytic anemia seen. Most antibodies are directed against the Rh system.
Treatment- Folate, steroids. If steroids do not work, then splenectomy. Note that steroids should be tapered off very slowly. In patients who had splenectomy, recurrent disease could be treated with low dose steroids i.e. 5 mg/day. Otherwise, immunosuppressives should be used. Plasmaphoresis can be used in emergencies. IVIG does not work as well as it does for ITP. Response rate is less than 30% (not a durable response). Danazol & Rituan could also be attempted. A list of patient's medications should be reviewd and potentially offending drugs should be discontinued. COLD AGGLUTININ SYNDROME
Low-grade, compensated anemia.
IgM against the I antigen system.
Usually the thermal amplitude of the antibody is determined.
Corticosteroids, splenectomy, & alkylating agents are generally ineffective.
Some patients may benefit from cyclophosphamide or chlorambucil (if patients have severe anemia).
Plasmaphoresis can be used in emergency situations.
Transfusion possible if the blood product is warmed to room temperature & infused slowly. An in-line warmer may also be used in severe cases.
PAROXYSMAL COLD HEMOGLOBINURIA
Moderate to severe anemia
Diagnosis- Donath-Lansteiner antibody in the patient's plasma. This IgG antibody is directed against the P blood group antigen. It is identified by the BI-THERMAL ASSAY.
Treatment- supportive care. Keep patient warm at all times.In severe cases, empiric steroids?
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