0521779407-10 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:40
Immune Hemolytic Anemia 817➣Cyclophosphamide
2 mg/kg/day PO for patients unfit for surgery or patients
who failed steroids and splenectomy; should not be used for
extended periods due to side effects
➣Other
IVIG, danazol, plasma exchange, anti-B cell monoclonal anti-
bodies
➣CAHA
➣Oral alkylating agents (chlorambucil and cyclophosphamide)
for severe disease secondary to lymphoproliferative disorders;
plasma exchange in critically ill patients unresponsive to tran-
sfusions; steroids for rare IgG cold agglutinins, anti-B cell mon-
oclonal antibodies
➣PCH
➣no specific therapy; transfuse rare P-antigen negative red blood
cells to patients who do not respond to random donor red blood
cells; consider steroids for episodes of severe hemolysis
➣MTHA
➣therapy should follow guidelines outlined above for WAHA
➣DIHA
➣no specific therapy other than withdrawing implicated medi-
cation; consider steroids for severe hemolysisSide Effects & Complications
■doses of prednisone greater than 1 to 2 mg/kg/day provide little
additional benefit and increase the number and severity of side
effects; side effects of chronic steroid administration are reduced
by alternate-day schedules
■splenectomy requires immunization with pneumococcal/me-
ningococcal vaccines to prevent sepsis secondary to encapsulated
bacteria
■azathioprine and cyclophosphamide have been associated with
bone marrow suppression, increased risk of infection and malig-
nancy, and decreased fertility
■in patients with cold-reactive autoantibodies hypothermia must be
avoided during surgery in particular procedures involving extracor-
poreal circuits
follow-up
■regularly assess degree of hemolysis and anemia – CBC, LDH level,
unconjugated bilirubin, DAT, IAT, cold agglutinin titers, cold agglu-
tinin thermal range
■assess potential complications of ongoing therapy