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Kidney Week

Abstract: TH-PO571

Idiopathic Multicentric Castleman's Disease (iMCD)-TAFRO Syndrome with Atypical Renal Biopsy Findings

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Chen, Ciara Lynn, Eisenhower Army Medical Center, Fort Gordon, Georgia, United States
  • Strenge, Karen, Eisenhower Army Medical Center, Fort Gordon, Georgia, United States
  • Tobin, Trevor Wesley, Eisenhower Army Medical Center, Fort Gordon, Georgia, United States
Introduction

Idiopathic Multicentric Castleman’s Disease (iMCD) is a rare lymphoproliferative disorder characterized by enlarged lymph nodes in HHV-8 negative patients. It is associated with fevers, weight loss, hepatosplenomegaly, cell line dyscrasias, and organ dysfunction. One subtype of iMCD is TAFRO syndrome, characterized by the presence of thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. We report a patient who presented with a seronegative proliferative glomerulonephritis and was subsequently diagnosed with iMCD-TAFRO syndrome.

Case Description

A 47-year-old male with no past medical history presented to the hospital with non-specific lab abnormalities. Vital signs showed new hypertension, tachycardia, and intermittent fevers. Physical exam noted diffuse anasarca. Laboratory investigation revealed elevated inflammatory markers, new anemia, and acute renal injury. He did not have nephrotic syndrome per urine protein quantification. Urine microscopy revealed microscopic hematuria with hyaline and granular casts. Glomerulonephritis serologic evaluation was negative. Rapidly progressive renal failure necessitated initiation of renal replacement therapy and pulse dose steroids. Renal biopsy revealed a proliferative glomerulonephritis without crescents or segmental necrosis. Immunofluorescence demonstrated trace focal mesangial granular deposits of IgM and C3. Electron microscopy showed moderate foot process effacement without subendothelial or mesangial deposits. Other evaluations included a PET/CT scan, bone marrow biopsy, and cervical lymph node biopsy. Pending lymph node biopsy results, IV cyclophosphamide was started for presumed seronegative ANCA vasculitis. Results from the cervical lymph node biopsy were consistent with iMCD. The patient was transitioned to siltuximab with a prednisone taper.

Discussion

iMCD-TAFRO syndrome can present with proliferative glomerulonephritis. While this patient presented with common clinical findings of iMCD, renal biopsy results were atypical relative to prior reports of a MPGN or TMA-like histopathology. iMCD-TAFRO syndrome may not always conform to typically seen patterns. Diagnosis requires a high index of suspicion and should be considered for glomerulonephritis with non-specific biopsy findings in the context of a systemic inflammatory disease.