Internal Medicine

0521779407-10 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:40

818 Immune Hemolytic Anemia Immunodeficiency Disorders

■surveillance for neoplastic process as cause of chronic hemolysis or

as a side-effect of therapy

complications and prognosis

Complications

■severe anemia, cardiac failure, renal failure, DIC (thrombosis and

bleeding) neurologic abnormalities, and death

Prognosis

■WAHA

■25% of steroid-treated patients will experience complete remission;

remaining patients will have either partial or no response at all;

response rate also depends on treatment of underlying disorder.

■splenectomy produces partial or complete remission in 50% of cases

■CAHA

■post-infectious CAHA may be very severe and life threatening requir-

ing aggressive supportive care in hospital. However, the majority of

cases are self-limited with full recovery within one month

■chronic idiopathic CAHA is characterized by prolonged survival with

spontaneous remissions and exacerbations

■in CAHA associated with malignancy the hemolysis often parallels

the activity of the neoplasm

■PCH

■post-infectious PCH is self-limited with full recovery within one

month; primary PCH has a natural history that extends over many

years punctuated by episodes of severe hemolysis

■MTHA

■steroids usually effective; disorder often persistent with intermittent

episodes of hemolysis

■DIHA

■positive DAT and hemolysis may persist for weeks to months follow-

ing discontinuation of drug

Immunodeficiency Disorders, Congenital..................

ANNE-MARIE IRANI, MD

history & physical

History

■Otitis media, sinusitis, pneumonia, meningitis: B-cell deficiency; X-

linked or autosomal recessive