Acquired hemoglobin H disease.

Abstract

Abstract Although hemoglobin H disease is usually inherited, similar syndromes may be acquired. In a 73-year-old woman of British descent with a myeloproliferative syndrome characterized by striking megakaryocytic hyperplasia and thrombocytosis, we also found hemolytic anemia with hypochromia, microcytosis, marked poikilocytosis and 20 per cent hemoglobin H (beta4). Incubation with brilliant cresyl blue produced inclusions in 60 per cent of the red cells. Hematologic studies before the onset of this illness gave normal results. In vitro studies of globinchain synthesis revealed a marked decrease of alpha-chain production similar to that found in hereditary hemoglobin H disease. The alpha/beta ratio of radioactivities was 0.13, demonstrating a severe imbalance of globin synthesis. Studies of the patient's two children gave normal results. Hematologic and globin synthesis in this acquired disorder is similar to those of its hereditary counterpart.