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Abstract: TH-PO560

Gross Hematuria and Proteinuria in Myelofibrosis: A Curious Case of Myelofibrosis-Related Glomerulopathy

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Edwin, Robin, Atrium Health Wake Forest Baptist, Winston-Salem, North Carolina, United States
  • Woldemichael, Jobira A., Atrium Health Wake Forest Baptist, Winston-Salem, North Carolina, United States
  • Dao, Kevin, Atrium Health Wake Forest Baptist, Winston-Salem, North Carolina, United States
  • Mirza, Mohid, Atrium Health Wake Forest Baptist, Winston-Salem, North Carolina, United States
  • Fletcher, Alison J., Atrium Health Wake Forest Baptist, Winston-Salem, North Carolina, United States
  • Mikhailov, Alexei V., Atrium Health Wake Forest Baptist, Winston-Salem, North Carolina, United States
Introduction

Renal involvement in myeloproliferative disease is rare and presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to renal extramedullary hematopoiesis (EMH) and glomerulopathy. Renal EMH presents as infiltration of the interstitial, functional damage of parenchyma and vessels and sclerosing mass-like lesions causing hydronephrosis and renal insufficiency.

Case Description

69 year old male patient with JAK2 mutated Polycythemia Vera progression to Myelofibrosis; admitted for respiratory failure with lobar pneumonia. Patient was on Ruxolitinib for 7 years. Baseline creatinine was 1.43mg/dL. Patient had an AKI with creatinine of 4 mg/dL attributed to contrast use and hypotension. During the hospital course, the patient developed an acute onset colicky flank pain associated with frank hematuria. CT imaging and renal duplex ultrasound were normal except for hepatosplenomegaly. Additional testing showed proteinuria (3.7g/g). Numerous dysmorphic RBCs and muddy brown casts present on microscopy. Serology for proteinuria were non-revealing. Renal biopsy showed diffuse moderate acute tubular injury, glomerulopathy with mesangial expansion, thickened glomerular capillary walls and megakaryocytes consistent with myeloproliferative neoplasm related glomerulopathy. The patient did not require dialysis. His hematuria and creatinine improved to 1.6 mg/dL. Patient remains on Ruxolitinib and received splenic radiation for symptomatic splenomegaly.

Discussion

Patient with myeloproliferative disease presenting with AKI, proteinuria, hematuria and acute abdominal colicky pain should raise suspicion of myeloproliferative related glomerulopathy.

Mesangial expansion and hypercellularity on light microscopy on Periodic Acid-Schiff stain.