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Published on: December 7, 2014
Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib
Arun Rajasekaran1, Thuy-Trang Ngo2, Maen Abdelrahim3
1Department of Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. arun.nephron@gmail.com.
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.
Insights
Primary myelofibrosis (PMF) can cause early kidney damage, specifically MPN-related glomerulopathy. Prompt diagnosis and treatment improved kidney function in a patient with early-stage PMF.
Area of Science:
- Nephrology
- Hematology
- Oncology
Background:
- Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) causing bone marrow fibrosis and extramedullary hematopoiesis.
- Renal involvement in PMF is uncommon, typically appearing as MPN-related glomerulopathy in advanced disease stages.
Observation:
- This report details the first case of early renal glomerular involvement in PMF/MPN.
- A 60-year-old male with PMF and stage 4 CKD presented with edema, AKI, and nephrotic-range proteinuria.
Findings:
- Kidney biopsy revealed early MPN-related glomerulopathy, characteristic of the condition.
- Early diagnosis and management stabilized kidney function to baseline levels.
Implications:
- Further research is needed to identify risk factors and standardize treatment for MPN-related glomerulopathy.
- Investigating whether aggressive PMF treatment impacts kidney disease progression is crucial.
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