Abstract: TH-PO0194
Fib and Flow: Fibrillary Deposits in the Wake of Myeloproliferative Disease
Session Information
- Onconephrology: Anticancer Therapies, PTLD, Paraneoplastic Diseases, and More
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1700 Onconephrology
Authors
- Yousuf, Ali K, University of Alabama at Birmingham Health System, Birmingham, Alabama, United States
- Konda, Raghunandan, University of Alabama at Birmingham Health System, Birmingham, Alabama, United States
- Fatima, Huma, University of Alabama at Birmingham Health System, Birmingham, Alabama, United States
- Vachhani, Pankit, University of Alabama at Birmingham Health System, Birmingham, Alabama, United States
- Rizk, Dana V., University of Alabama at Birmingham Health System, Birmingham, Alabama, United States
- Rajasekaran, Arun, Renal Medicine Associates, Albuquerque, New Mexico, United States
- Gandhi, Mamatha, University of Alabama at Birmingham Health System, Birmingham, Alabama, United States
Introduction
Primary Myelofibrosis (PMF) is a chronic myeloproliferative neoplasm that is characterized by clonal proliferation of hematopoietic stem cells, bone marrow fibrosis, and extramedullary hematopoiesis. Myeloproliferative neoplasm (MPN)-related glomerulopathy is a rare diagnosis with only a few cases that have been reported in literature. We present a case of biopsy proven MPN-associated glomerulopathy with fibrillary deposits.
Case Description
A 54-year-old male with uncontrolled hypertension originally presented with weight loss and splenomegaly. Bone marrow biopsy revealed PMF. The patient was enrolled in a clinical trial for ruxolitinib and selinexor. He developed AKI [peak SCr 2.3 mg/dL from baseline 1.7 - 1.8 mg/dL] prompting selinexor discontinuation. Subsequently, he developed nephrotic range proteinuria with UACR 5.9 g/g, UPCR 6.9 g/g, and serum albumin 4.2 g/dL. Comprehensive glomerulonephritis (GN) workup including anti-PLA2R and anti-THSD7A were negative. Kidney biopsy - LM demonstrated severe arterial hyaline arteriosclerosis, focal and segmental glomerulosclerosis, and interstitial infiltration by atypical megakaryocytes. Frozen and pronase IF showed nonspecific staining patterns for immunoreactants. EM demonstrated global deposition of randomly oriented straight fibrils [7-13 nm] classified as fibrillary-like deposits with global foot process effacement [90%]. Congo red, DNAJB9 stains, and mass spectrometry were negative. A diagnosis of MPN-related glomerulopathy with fibrillary deposits was made. Tacrolimus and intravenous rituximab [2 doses of 1g two weeks apart] were administered due to nonresponse to conservative management.
Discussion
MPN-related glomerulopathy is a rare form of kidney involvement seen in patients with PMF. Histopathologic findings often include mesangial sclerosis, segmental glomerulosclerosis, and glomerular or interstitial infiltration by megakaryocytes. Concomitant fibrillary deposits have not been described in literature to date. Treatment is largely supportive, with disease-directed therapy potentially offering some benefit. Increased awareness among clinicians and nephrologists is needed to better understand the disease course natural and optimize management of this rare condition.