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.

journal_name

Kidney Int

journal_title

Kidney international

authors

Said SM,Leung N,Sethi S,Cornell LD,Fidler ME,Grande JP,Herrmann S,Tefferi A,D'Agati VD,Nasr SH

doi

10.1038/ki.2011.147

subject

Has Abstract

pub_date

2011-10-01 00:00:00

pages

753-9

issue

7

eissn

0085-2538

issn

1523-1755

pii

S0085-2538(15)55110-7

journal_volume

80

pub_type

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