MARKEDLY HIGH PLASMA ERYTHROPOIETIN AND GRANULOCYTE-COLONY-STIMULATING FACTOR LEVELS IN PATIENTS WITH PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA

In patients with paroxysmal nocturnal hemoglobinuria (PNH), we measure d plasma concentrations of endogenous hematopoiesis-regulatory cytokin es to characterize bone marrow (BM) hypoplasia which is a major cause of death. Contrary to 10 healthy individuals, all 14 patients with PNH showed increases of erythropoietin (Epo) and granulocyte-colony stimu lating factor (G-CSF). There were no signs of infection, renal dysfunc tion or hypoxia. The lower the hemoglobin level and granulocyte count, the higher the plasma Epo and G-CSF levels. In contrast, marked diffe rences were not found in the levels of interleukin-3 (IL-3), tumor nec rosis factor-alpha (TNF-alpha), stem cell factor (SCF), granulocyte/ma crophage-colony stimulating factor (GM-CSF), or interferon-gamma (IF-g amma). The cytokine profiles of PNH patients were quite similar to tho se of patients with aplastic anemia (AA) and myelodysplastic syndrome (MDS). The cytokine profiles may support a pathological relationship b etween PNH and these stem cell disorders. (C) 1997 Elsevier Science Ir eland Ltd.