Polycythemia Vera in Childhood: A Case With Chromosomal Abnormality, Immunoglobulin Deficiency, and Chronic Consumption Coagulopathy

We have studied a patient with polycythemia vera accompanied by thrombocythemia, massive splenomegaly, and a severe bleeding tendency. Diminished plasma levels of factors I, V, and VIII in association with increased serum fibrinopeptides suggested consumption coagulopathy. Cytogenetic analysis of bone marrow and peripheral blood cells demonstrated partial deletion of an E-group chromosome. He also had mild IgA deficiency. Treatment with melphalan maintained his peripheral blood cell counts at normal values, reduced spleen size, and resulted in return toward normal of diminished clotting factors. He no longer experienced abnormal bleeding.