Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib.

Abstract

BACKGROUND:

Primary myelofibrosis (PMF) is a type of myeloproliferative neoplasm (MPN) characterized by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, leading to the deposition of fibrous tissue, and by a propensity toward extramedullary hematopoiesis. Renal involvement in PMF is rare, but kidney tissue samples from these patients reveal MPN-related glomerulopathy, a recently discovered condition, in the late stages of the disease.

CASE PRESENTATION:

We present the first case described in the medical literature of a patient with early renal glomerular involvement in PMF/MPN. A 60-year-old man with stage 4 chronic kidney disease and a recent diagnosis of PMF (within 4 weeks of presentation at our renal division) presented with generalized body swelling, acute kidney injury, and massive nephrotic-range proteinuria. Kidney biopsy was performed to determine the etiology of the patient's renal dysfunction and revealed early renal glomerular involvement that was histologically characteristic of MPN-related glomerulopathy. Early diagnosis and prompt medical management returned the patient's kidney functionality to the levels seen on initial presentation at our hospital.

CONCLUSION:

Large studies with long follow-up durations are necessary to identify and categorize the risk factors for the development of MPN-related glomerulopathy, to standardize therapeutic regimens, and to determine whether aggressive management of the myelofibrosis slows the progression of kidney disease.

• Authors: Arun Rajasekaran, Thuy-Trang Ngo, Maen Abdelrahim, William Glass, Amber Podoll, Srdan Verstovsek, Ala Abudayyeh
• Journal: BMC nephrology (BMC Nephrol) Vol. 16 Pg. 121 (Aug 01 2015) ISSN: 1471-2369 [Electronic] England
• PMID: 26232031 (Publication Type: Case Reports, Journal Article)

Chemical References

• Nitriles
• Protein Kinase Inhibitors
• Pyrazoles
• Pyrimidines
• ruxolitinib

Topics

• Acute Kidney Injury (drug therapy, etiology, pathology)
• Glomerulonephritis, Membranoproliferative (drug therapy, etiology, pathology)
• Humans
• Kidney (pathology)
• Male
• Middle Aged
• Nitriles
• Primary Myelofibrosis (complications, drug therapy, pathology)
• Protein Kinase Inhibitors (therapeutic use)
• Proteinuria (drug therapy, etiology)
• Pyrazoles (therapeutic use)
• Pyrimidines
• Renal Insufficiency, Chronic (complications, drug therapy, pathology)
• Treatment Outcome