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3. Autoimmune Hemolysis
Combine patients serum with cells from a panel of RBCs with known antigens
Add Coombs reagent to this mixture If anti-RBC antigens are in serum, agglutination occurs
Treatment
Prednisolone 1mg/kgc/d 1-2 weeks, Azathioprine, cyclophosphamide Splenectomy-selected cases Rituximab (anti CD20 ) Ig(iv) 0.4g/kg/d x 5d Folic acid 5mg/d Treat underlying disease (if secondary)
AUTOIMMUNE HEMOLYSIS Cold Type Most commonly IgM mediated Antibodies bind best at 30 or lower Fix entire complement cascade Leads to formation of membrane attack complex, which leads to RBC lysis in vasculature Typically only complement found on cells (Direct Cooms test) 90% associated with other illnesses
Cold Hemagglutinin Disease Chronic: Often seen in elderly patients Tends to be associated with Lymphoma, CLL, Waldenstroms macroglobulinemia Increased Erythrocyte Sedimentation Rate (ESR) Raynauds syndrome, acrocyanosis and hemoglobinuria seen during cold weather Severity depend on antibody titer, thermal amplitude (2330), environmental temperature
Cold Hemagglutinin Disease Acute/Transient Often secondary to lymphoproliferative disease (lymphoma) or Mycoplasma pneumonia infections (anti-I specificity) Also seen in patients with Infectious Mononucleosis with anti-i specificity. Transient: When infectious process is complete auto antibodies fade.
Direct Antiglobulin Test (DAT) Positive with cold auto antibodies Only Complement (C3 components) is present because these are IgM antibodies
Treatment
Keep warm Corticosteroids-little value Chlorambucil or cyclophosphamide Plasma exchange Splenectomy occasional useful If blood transfusion requaired-use in line blood warmer
Selection Of Blood For Transfusion MUST KNOW if there is an underlying unexpected clinically significant allo antibody. Transfusion of patients with WAIHA is usually NOT recommended. Why? Transfused cells are usually destroyed as rapidly as the patients own cells. When transfusion is unavoidable, usually must transfuse with Least Incompatible Blood. Patient is generally transfused with small volumes, to maintain O2 carrying capacity, until hemolysis diminishes or other therapy can effect a more lasting benefit.
Cold Hemagglutinin Disease Paroxysmal Cold Hemoglobinuria Auto-Antibody with anti-P specificity (P Blood Group) Antibody is a Biphasic IgG class antibody that binds complement at cold temperatures and lyses red cells at warm temperatures Donath-Landstiener test is confirmatory for PCH