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Abstract: PO1917

Delayed Thrombotic Microangiopathy Post Bone Marrow Transplant, an Atypical Presentation: A Case Report

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Vujjini, Vikas, Penn Medicine, Philadelphia, Pennsylvania, United States
  • Deep, Aman, Penn Medicine, Philadelphia, Pennsylvania, United States
  • Palmer, Matthew, Penn Medicine, Philadelphia, Pennsylvania, United States
  • Geara, Abdallah Sassine, Penn Medicine, Philadelphia, Pennsylvania, United States
Introduction

Thrombotic Microangiopathy (TMA) is a potentially lethal complication of Bone Marrow Transplantation (BMT). We report a case of delayed TMA post-BMT which was successfully treated with Rituximab.

Case Description

A 53-year-old male known to have hepatosplenic gamma-delta T-cell lymphoma (HSTCL) was referred for evaluation of worsening creatinine. He was diagnosed with HSTLC 6 years ago that was refractory to multiple therapies, and ultimately, two years later, he received Double Unit Cord Blood (dUCB) transplantation with good response and minimal residual disease. Despite receiving tacrolimus and mycophenolate mofetil (MMF) for prophylaxis of Graft versus Host Disease (GVHD), he developed mild popular rash consistent with Grade I GHVD skin and recurrent pneumonitis concerning for lung GHVD which responded to steroids. The tacrolimus and MMF were discontinued and steroids were gradually tapered off. Eight months post-transplant, serum creatinine (sCr) started to gradually increase from a baseline of 1.0 mg/dL. We were consulted eighteen months post-dUCB when sCr reached 1.8 mg/dL. Additional evaluation showed mild proteinuria (UPCR 0.77 g/g of creatinine), no active urine sediment, low haptoglobin (< 30 mg/dL) and worsening thrombocytopenia (105 THO/µL). A renal biopsy showed glomeruli with variable capillary wall thickening and double contours, moderate fibrosis of 40-50%, negative immunofluorescence for complement and immunoglobulin, and the electron microscopy showed subendothelial expansion and endothelial swelling. These findings were compatible with chronic TMA lesion concerning for renal GVHD. The patient was treated with weekly Rituximab 375 mg/m2 for a total of 4 doses with stabilization of sCr and normalization for hemolysis labs, including normalization of platelet count.

Discussion

TMA is a well described complication post-BMT with multifactorial etiology (medication, GVHD, radiation, etc.) with early onset within the first 30-45 days after transplantation, and the mortality rate is approximately 30-80%. Our patient had the unusual delayed presentation post-BMT (after 18 months). Although historically not the first line, several case reports have been published showing use of Rituximab, an anti-CD20 monoclonal antibody, with positive response.