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Kidney Week

Abstract: TH-PO552

A Case of Multicentric Castleman Disease – TAFRO Variant, Presenting with Renal TMA

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Jenssen, Fredrik, Medical University of South Carolina, Charleston, South Carolina, United States
  • Budisavljevic, Milos N., Medical University of South Carolina, Charleston, South Carolina, United States
  • Elliott, Andrew B., Medical University of South Carolina, Charleston, South Carolina, United States
  • Asfar, Waleed, Medical University of South Carolina, Charleston, South Carolina, United States
Introduction

Castleman’s disease is a rare systemic lymphoproliferative disorder in which immune cells become activated to produce excess cytokines, particularly IL-6. A subset of cases are related to HIV or HHV-8. There is a wide heterogeneity of symptoms in the disease, ranging from asymptomatic to multisystem organ dysfunction. TAFRO syndrome is a unique clinicopathologic variant of multicentric Castleman’s disease first described in 2010 in Japan by Takai et al. It includes thrombocytopenia, anasarca, microcytic anemia, fever, reticulin fibrosis, renal dysfunction, and organomegaly. To our knowledge, this syndrome has yet to be reported in the U.S.

Case Description

20 y.o. male with previous normal kidney function, presented with AKI, lymphadenopathy, anasarca, and diarrhea after 2 month history of myalgia, malaise, back pain, diarrhea, vomiting, low grade fevers, and night sweats. He endorsed large amounts of NSAIDs use for myalgias. He presented with serum creatinine of 2.4 mg/dL which continued to rise despite conservative therapy with isotonic volume expansion and peaked at 4.7 mg/dL. The urinalysis was bland and urine protein-to-creatinine ratio was 0.1 g/g. Extensive workup including serologies for hepatitis and autoimmune disease were negative. Noncontrast CT was obtained which revealed prominent axillary, retroperitoneal, mesenteric, and inguinal lymphadenopathy. A renal biopsy revealed thrombotic microangiopathy (TMA) with mostly arterial changes, and evidence of mesangiolysis. Further workup for hemolytic-uremic syndrome and thrombotic thrombocytopenic purpura was negative. An excisional left axillary lymph node biopsy revealed classic findings of Castleman's disease. PCR for CMV, EBV, HIV, HHV-6, and HHV-8 were all negative. He was started on oral steroids, as well as IL-6 inhibitor (siltuximab) and developed hyperkalemia which was refractory to medical management, and required correction via conventional hemodialysis. He was continued on prednisone and received situximab infusion every 3 weeks with full recovery of renal function.

Discussion

Specific targeting of pathogenic mechanism of Castlaman’s associated TMA with siltuximab in our case resulted in complete recovery of dialysis dependent renal TMA. To our knowledge, this is the first case of extremely favorable outcome of Castlaman’s disease associated TMA treated with IL-6 inhibitor.