Polycythemia vera in a patient with the human immunodeficiency virus: a case report

It has been suggested that HIV plays a role in the generation of myeloproliferative disorders, including polycythemia vera (PV). Seven cases of polycythemia in HIV patients have been described in the literature, but only 3 of these met criteria for determining a primary origin (vera) of polycythemia. We report a case of PV in a patient infected with HIV. A 45 year old non-smoking homosexual male presented with 15.7 g/dl hemoglobin in 1991, and was diagnosed with HIV. After 7 years, he presented with plethora, splenomegaly, an erythrocyte mass of 71 ml/kg, and an oxygen saturation of 93.9% (the latter three constituting the major criteria for the diagnosis of PV). Erythrocytes 7.35 x 10(6)/ml hemoglobin, 21.4 g/dl, hematocrit 63%, leukocytes 12,400, erythropoietin < 5 nmoll/ml. These values are all compatible with a diagnosis of PV. The CD4 count was 321 cells/mm(3) and HIV viral load was undetectable. The patient was initially treated with zidovudine. He was then treated with didanosine, lamivudine, and saquinavir, but all of them failed to slow the increase in erythrocyte levels. After a diagnosis of PV, he was treated with hydroxyurea and phlebotomy, which normalized the hemogram. CD4 count rose to 474 cells/mm(3) and HIV viral load remained at undetectable levels. The patient remains in stable condition with combination treatment after 1 year. We suggest that this is a case of HIV infection which may have led to the emergence of polycythemia vera. Treatment of the HIV did not prevent the appearance of the myeloproliferative disorder.