Glomerular involvement in myelodysplastic syndromes

Abstract

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented with hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient hematological improvement occurred. Relapse subsequently occurred that manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease, but hematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, 2 years after the onset of the MDS. Patient 2, who had refractory anemia with clonal monosomy 19, presented with bowel disease, hepatosplenomegaly, anemia and non-organ-specific autoantibodies. Prednisone led to both clinical and hematological remission. The hematologic disease relapsed 12 months later, when nephrotic-range proteinuria, hematuria and mild azotemia were also found. Corticosteroid treatment led to long-lasting renal and hematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis, and AL amyloidosis were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS and (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.

Antiretroviral activity of 5-azacytidine during treatment of a HTLV-1 positive myelodysplastic syndrome with autoimmune manifestations

“…MDSs have been linked to several types of glomerular diseases, such as glomerulosclerosis, membranous nephropathy, IgA nephropathy, and ANCA associated nephropathy [21-25]. Unfortunately, in our case, due to lack of histopathologic examination, only speculations can be made about the type of the patient's renal disease.…”

Glomerular diseases seen with cancer and chemotherapy: a narrative review

“…Myelodysplastic syndrome has been rarely associated with MN, MCD, FSGS, and mesangial proliferative GN. 47…”

Presumptive colchicine toxicity in a 17‐year‐old Arab gelding being treated for chronic active hepatitis

“…Although both aplastic anaemia and pancytopenia in horses are rare conditions many cases are characterised as idiopathic because a predisposing cause cannot be found (Archer and Miller 1965;Berggren 1981;Ward et al 1982;Lavoie et al 1987;Milne et al 1995). Infectious, toxic, irradiative and neoplastic causes of pancytopenia are well documented in man (Bridsky and Jones 2005) and other species (Shimoda et al 2000;Weiss 2003), and the condition can be seen in man in association with severe hepatic (Young 1999) and renal disease (Bogdanovic et al 2001). To the author's knowledge there are no documented cases of pancytopenia in horses with chronic liver disease, although anaemia associated with haemolysis has been reported (Tennant et al 1972).…”