A Peculiar Familial Hemolytic Anemia in the Tropics. Its Differential Diagnosis from Thalassemia (Mediterranean Anemia)

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Abstract
This anemia is characterized by marked impairment of the general condition, severe anemia and marked jaundice. Hemolytic attacks are frequent; erythroblastosis is marked; the anemia is microcytic, sometimes normocytic and constantly hypochromic, with no spherocytosis, and normal red cell fragility. The disease most frequently starts in childhood; splenectomy neither improves the anemia nor the jaundice; on the contrary, the anemia becomes more marked, the hemolytic attacks continue as before. The anemia is converted to a macrocytic and markedly erythroblastic one. The difference between this type of hemolytic anemia and thalassemia major is discussed. These 2 hemolytic diseases differ in the heredity pattern. Symptomatology, possible etiology, differential diagnosis, prognosis and therapy of this particular type of hemolytic anemia are discussed and an illustrative case report is added.

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