Basophilic Chronic Granulocytic Leukaemia with Hyperhistaminaemia

Abstract

Two patients with typical Philadelphia chromosome positive chronic granulocytic leukemia (CGL) developed an accelerated phase of the disease characterized by an increased white blood cell count and marked basophilia in the bone marrow and peripheral blood. Histamine levels were extremely high in both patients. Hyperhistaminemia was manifested as wheezing, urticaria, diarrhea and pruritus in 1 patient and as peptic ulcer disease and peripheral edema in both patients. In 1 case, gastric acid studies revealed a very high basal to stimulated ratio. Treatment with the investigational agent metiamide, an H2 receptor histamine antagonist, resulted in marked improvement in symptoms and reduction in gastric acid output. Extreme basophilia in CGL may be associated with hyperhistaminemia, and manifestations of both the H1 and H2 type may occur.