Myeloproliferative neoplasm related nephropathy

Primary Myelofibrosis associated Nephropathy is a recently described entity. Primary Myelofibrosis (PMF) is a Myeloproliferative Neoplasm (MPN) where the marrow space shows fibrosis with megakaryocyte dysplasia. We present a case of a 32 year old patient who was detected to have proteinuria, renal dysfunction and anemia ,on a routine health check up. Primary Myelofibrosis was confirmed by positive Jak 2 expression. Kidney biopsy showed focal segmental glomerulosclerosis along with thickened glomerular basement membrane. The histomorphological features of renal and bonemarrow biopsies are analysed and a detailed review of the literature is done.
 

Keywords: Myeloproliferative neoplasm, Focal segmental glomeruloscerosis, Primary myelofibrosis