title: Myeloproliferative neoplasms cause glomerulopathy.
abstract: Myeloproliferative neoplasms are clonal hematopoietic stem cell disorders that can produce an undefined glomerulopathy. To better characterize the glomerular disease associated with myeloproliferative neoplasms, we evaluated features of 11 patients with myeloproliferative neoplasm-related glomerulopathy that included 8 patients with primary myelofibrosis, and 1 each with chronic myelogenous leukemia, polycythemia vera, and essential thrombocythemia. Indications for biopsy were nephrotic-range proteinuria (nephrotic syndrome in four) and chronic renal insufficiency. The mean time from diagnosis of the neoplasms to biopsy was 7.2 years. Histologically, mesangial sclerosis and hypercellularity were seen in all 11 cases, segmental sclerosis in 8, features of chronic thrombotic microangiopathy in 9, and intracapillary hematopoietic cells in 4. On follow-up, seven patients had persistent renal dysfunction and four progressed to end-stage renal disease (ESRD). Thus, glomerulopathy appears to be a late complication of myeloproliferative neoplasms, particularly primary myelofibrosis, with guarded prognosis. Greater awareness of this entity and larger studies are needed to define possible therapies.
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.
authors.forename: Samar M
authors.initials: SM
authors.lastname: Said
authors.forename: Nelson
authors.initials: N
authors.lastname: Leung
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sethi
authors.forename: Lynn D
authors.initials: LD
authors.lastname: Cornell
authors.forename: Mary E
authors.initials: ME
authors.lastname: Fidler
authors.forename: Joseph P
authors.initials: JP
authors.lastname: Grande
authors.forename: Sandra
authors.initials: S
authors.lastname: Herrmann
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
authors.forename: Vivette D
authors.initials: VD
authors.lastname: D'Agati
authors.forename: Samih H
authors.initials: SH
authors.lastname: Nasr
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology / Q000473:pathology; D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000150:complications; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications*; D011087:Polycythemia Vera / Q000150:complications; D055728:Primary Myelofibrosis / Q000150:complications; D011379:Prognosis; D013920:Thrombocythemia, Essential / Q000150:complications; D013997:Time Factors