Myelodysplastic syndromes with nephrotic syndrome

Abstract

It is sometimes reported that the immunological abnormalities in myelodysplastic syndromes (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 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 laboratory data and the findings at the physical examination. The diagnoses of nephrotic syndrome and glomerular disease were established when 24-hr urinary 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 patients (4%) had glomerular disease, and three (2.4%) had nephrotic syndrome. Of the five patients with glomerular disease, two had refractory anemia (RA), and three had chronic myelomonocytic leukemia (CMMOL). Three of the total 11 patients with CMMOL were diagnosed as having nephrotic syndrome. Among the CMMOL patients, those with nephrotic syndrome showed higher absolute monocyte numbers than did those without nephrotic syndrome (8830 +/- 4677/microl vs. 3061 +/- 2887/microl, P = 0.03). One CMMOL patient was treated with VP-16 and hydroxyurea. As the white blood cell count in this patient decreased, the 24-hr urine protein excretion and the serum tumor necrosis factor alpha level decreased. The relationship between nephrotic syndrome and CMMOL was not clear. High monocyte count and the serum cytokines in MDS patients may play a partial role in the evolution of glomerulonephritis, and CMMOL may be closely related to nephrotic syndrome.

A Forgotten Cause of Kidney Injury in Chronic Myelomonocytic Leukemia

“…Tubule cells presumably undergo toxic injury leading to atrophy, interstitial fibrosis, and progressive kidney disease. This mechanism of kidney injury has been reported only rarely in the modern literature 8–12…”

“…The mechanism by which lysozyme induces kidney failure is incompletely understood. Filtered lysozyme appears to be a direct tubular toxin,7,8 and animal studies suggest that endocytic reabsorption of such low-molecular-weight cationic proteins as lysozyme by the proximal tubules exacerbates superimposed ischemic tubular damage 1…”

“…The high level of tubular lysozyme may induce significant kaliuresis with severe hypokalemia. 15,16 Alternatively, lysozyme may induce direct tubular injury and kidney failure 7,8. It is worth mentioning that kidney injury from other cationic macromolecules has been reported in the literature.…”

Pathogenesis, diagnosis and management of paraneoplastic glomerulonephritis

“…This pseudonephrotic syndrome should be excluded before a diagnosis of paraneoplastic glomerulonephritis is made. Chemotherapy effectively ameliorated paraneoplastic glomerulonephritis in three patients,71,77 and a 2008 case report demonstrated mycophenolate treatment to be effective for CMML-associated membranous nephropathy 79…”

“…The prevalence of paraneoplastic glomerulonephritis in patients with myelodysplastic syndrome and chronic myelomonocytic leukemia (CMML), a myelodysplastic/myeloproliferative disorder , is 2% and 27%, respectively, as reported in a small series of 114 patients with myelodysplastic syndrome and 11 patients with CMML 71. Myelodysplastic syndrome is associated with autoimmunity and approximately 10% of patients with myelodysplastic syndrome have clinical autoimmune disorders 72.…”