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paroxysmal cold hemoglobinuria (PCH)
Etiology:
1) viral infection
-> measles & mumps in children
2) autoimmune disease
3) tertiary syphilis
Epidemiology: rare
Pathology:
-> complement-mediated hemolysis resulting from IgG autoantibody against RBC (P-antigen) activated by the cold
Clinical manifestations:
1) attacks precipitated by cold exposure
2) fever/chills
3) back pain, leg pain, abdominal pain/cramps
4) headache following exposure to the cold
5) malaise
6) nausea/vomiting
7) diarrhea
8) splenomegaly
9) hepatomegaly
10) transient jaundice may be observed
11) pale fingers, toes & tip of nose
12) rapid recovery from acute episode
13) generally asymptomatic between episodes
14) disorder generally extends over many years
Laboratory:
1) IgG autoantibody directed against P-antigen on RBC (Donath-Landsteiner antibody)
2) hemoglobinemia
3) hemoglobinuria
-> hemoglobin in fresh urine without red cells
4) methemoglobin in urine
5) cold hemolysis of blood in vitro may be observed
6) direct antiglobulin test (Coomb's)
a) may show complement, but seldom IgG
b) often negative
7) Donath-Landsteiner test:
a) hemolysis activated by exposure of blood to cold
b) hemolysis takes place only after rewarming
8) peripheral blood smear may show agglutination
Management:
1) treat syphilis if present; responds favorably to treatment
2) chronic autoimmune PCH may respond to immunosuppression
a) prednisone
b) azathioprine
c) cyclophosphamide
3) does NOT respond to splenectomy
General
hemoglobinuria
cold antibody autoimmune hemolytic anemia
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 667-68
- DeGowin & DeGowin's Diagnostic Examination, 6th edition,
RL DeGowin (ed), McGraw Hill, NY 1994, pg 892
- Medical Knowledge Self Assessment Program (MKSAP) 15,
American College of Physicians, Philadelphia 2009