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.
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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)
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Chemical References |
- Nitriles
- Protein Kinase Inhibitors
- Pyrazoles
- Pyrimidines
- ruxolitinib
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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
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