Myelodysplastic syndromes with nephrotic syndrome

Division of Blood Transfusion Service, Gunma University School of Medicine, Gunma, JapanIt is sometimes reported that the immunological abnormalities in myelodysplastic syn-dromes (MDS) induce autoimmune disease (i.e., acute systemic vasculitic syndrome,chronic cutaneous vasculitis, polyneuropathy, relapsing polychondritis, and steroid-responsive pulmonary disorders). We investigated the clinical features of patients withMDS accompanied by nephrotic syndrome. We enrolled 125 patients with MDS who wereadmitted between January 1979 and May 1996 in this study. The renal function wasassessed based on the laboratory data and the findings at the physical examination. Thediagnoses of nephrotic syndrome and glomerular disease were established when 24-hrurinary excretion was more than 3.5 g and serum total protein was less than 6.0 g/dl, andwhen the 24-hr protein excretion was more than 1.5 g. Five patients (4%) had glomerulardisease, and three (2.4%) had nephrotic syndrome. Of the five patients with glomerulardisease, two had refractory anemia (RA), and three had chronic myelomonocytic leuke-mia (CMMOL). Three of the total 11 patients with CMMOL were diagnosed as havingnephrotic syndrome. Among the CMMOL patients, those with nephrotic syndromeshowed higher absolute monocyte numbers than did those without nephrotic syndrome(8830 ± 4677/µl vs. 3061 ± 2887/µl, P= 0.03). One CMMOL patient was treated with VP-16and hydroxyurea. As the white blood cell count in this patient decreased, the 24-hr urineprotein excretion and the serum tumor necrosis factor alpha level decreased. The rela-tionship between nephrotic syndrome and CMMOL was not clear. High monocyte countand the serum cytokines in MDS patients may play a partial role in the evolution ofglomerulonephritis, and CMMOL may be closely related to nephrotic syndrome. Am. J.Hematol. 60:200–204, 1999.