Focal segmental glomerulosclerosis and mesangial sclerosis associated withmyeloproliferative disorders

The myeloproliferative disorders (MPDs) are clonal disorders of the hematop oietic stem cell and classified as polycythemia vera (PV), essential thromb ocythemia (ET), or agnogenic myeloid metaplasia (AMM), depending on the mai n hematopoietic lineage involved. Primary renal parenchymal lesions are not commonly reported in these cases. We conducted a retrospective analysis of 138 consecutive patients with MPD to determine the frequency of renal pare nchymal complications. Five patients (3.6%) (two PV, two ET, one AMM) were found to have focal segmental glomerulosclerosis (FSGS) and diffuse mesangi al sclerosis, presenting as proteinuria in all the cases and progressing to chronic renal failure in two cases, A possible common risk factor was a hi gh platelet count, because abnormal platelet activation in MPD has been sho wn to contribute to the development of glomerulosclerosis. The pathophysiol ogic basis of our observations and the implications in management of MPD pa tients remain to be studied, (C) 1999 by the National Kidney Foundation, In c.