Myelodysplastic syndromes with nephrotic syndrome

It is sometimes reported that the immunological abnormalities in myelodyspl astic syndromes (MDS) induce autoimmune disease (i.e,, acute systemic vascu litic syndrome, chronic cutaneous vasculitis, polyneuropathy, relapsing pol ychondritis, and steroid-responsive pulmonary disorders). We investigated t he clinical features of patients with MDS accompanied by nephrotic syndrome . We enrolled 125 patients with MDS who were admitted between January 1979 and May 1996 in this study. The renal function was assessed based on the la boratory data and the findings at the physical examination. The diagnoses o f nephrotic syndrome and glomerular disease were established when 24-hr uri nary excretion was more than 3.5 g and serum total protein was less than 6. 0 g/dl, and when the 24-hr protein excretion was more than 1.5 g. Five pati ents (4%) had glomerular disease, and three (2.4%) had nephrotic syndrome. Of the five patients with glomerular disease, two had refractory anemia CPA ), and three had chronic myelomonocytic leukemia (CMMOL). Three of the tota l 11 patients with CMMOL were diagnosed as having nephrotic syndrome. Among the CMMOL patients, those with nephrotic syndrome showed higher absolute m onocyte numbers than did those without: nephrotic syndrome (8830 +/- 4677/m u l vs. 3061 +/- 2887/mu l, P = 0.03). One CMMOL patient was treated with V P-16 and hydroxyurea. As the white blood cell count in this patient decreas ed, the 24-hr urine protein excretion and the serum tumor necrosis factor a lpha level decreased. The relationship between nephrotic syndrome and CMMOL was not clear. High monocyte count. and the serum cytokines in MDS patient s may play a partial role In the evolution of glomerulonephritis, and CMMOL may be closely related to nephrotic syndrome. Am. J. Hematol. 60:200-204, 1999. (C) 1999 Wiley-Liss, Inc.