Affiliation:
1. Aster Medcity, Kochi, Kerala, India
2. A
Abstract
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.
Publisher
IP Innovative Publication Pvt Ltd
Reference6 articles.
1. Swerdlow S H, Campo E, Harris L, Jaffe E S, Pilleri S A., Stein H, Primary myelofibrosis.WHO Classification of tumours of Hematopoietic and Lymphoid tissues. Revised 4th Edn..44-50
2. Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib
3. Nephrotic syndrome in primary myelofibrosis with renal extramedullary hematopoiesis and glomerulopathy in the JAK inhibitor era
4. Said S M, Leung N, Sethi S, Cornell LD, Fidler ME, Grande JP, Myeloproliferative neoplasms cause glomerulopathy.Kidney int 2011;80(7):753-9
5. Focal segmental glomerulosclerosis and mesangial sclerosis associated with myeloproliferative disorders