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hemoglobin H disease

Clinical manifestations: 1) onset of symptoms may be in childhood or as an adult (in milder cases) 2) hemolytic anemia 3) marked splenomegaly Laboratory: 1) complete blood count a) hemoglobin 6-10 g/dL b) MCV 60-70 fL (microcytosis) 2) peripheral smear a) target cells b) Heinz bodies 3) reticulocytes are increased 4) hemoglobin electrophoresis: Hgb A (70-95%), Hgb H (5-30%)

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 585-86
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 650-51