H. Nakakuma et al., MARKEDLY HIGH PLASMA ERYTHROPOIETIN AND GRANULOCYTE-COLONY-STIMULATING FACTOR LEVELS IN PATIENTS WITH PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA, International journal of hematology, 66(4), 1997, pp. 451-457
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.