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Abstract: TH-PO551

Prominent Swelling of Glomerular Endocapillary Cells in Two Cases of TAFRO Syndrome

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

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Shimada, Keiki, The Jikei University School of Medicine, Minato-ku, Japan
  • Sasaki, Takaya, The Jikei University School of Medicine, Minato-ku, Japan
  • Koike, Kentaro, The Jikei University School of Medicine, Minato-ku, Japan
  • Honda, Yu, The Jikei University School of Medicine, Minato-ku, Japan
  • Ishikawa, Masahiro, The Jikei University School of Medicine, Minato-ku, Japan
  • Tsuboi, Nobuo, The Jikei University School of Medicine, Minato-ku, Japan
  • Yokoo, Takashi, The Jikei University School of Medicine, Minato-ku, Japan
Introduction


TAFRO syndrome, which is characterized by thrombocytopenia (T), anasarca (A), fevers (F), reticulin myelofibrosis/renal disorder (R), and organomegaly (O), is sometimes a fatal systemic inflammatory disease. The histopathological findings remain unclear because it is often difficult to perform renal biopsy due to severe thrombocytopenia. We herein report the histopathological findings of two cases of TAFRO syndrome in which renal biopsies were performed.

Case Description


Case 1: A 51-year-old male was admitted due to fever, fatigue, high CRP value, thrombocytopenia, and resistance to antibiotic drug treatment of two weeks’ duration. He showed anasarca and multiple lymph node enlargement. He was diagnosed with TAFRO syndrome. Even though intensive care, including administration of corticosteroids, tocilizumab, and hemodialysis were continued, he died on the 24th day of hospitalization due to multiple organ dysfunction. Renal biopsy revealed marked swelling of glomerular endocapillary cells.
Case 2: A 34-year-old man was referred due to fatigue, pretibial pitting edema, urinary protein, and urinary occult blood of one-month duration. He was admitted to the hospital for renal dysfunction and heightened inflammatory response. Hepatosplenomegaly, ascites, and thrombocytopenia was observed. He was diagnosed with TAFRO syndrome due to elevated VEGF and IL-6 levels. Prednisolone was administered and the heightened inflammatory response and proteinuria were ameliorated. Renal biopsy revealed endocapillary hypercellularity, endothelial cell enlargement, mesangiolysis, and duplication of basement membrane. These findings were compatible with thrombotic microangiopathy.

Discussion


The findings suggest that endothelial dysfunction is consistent with the characteristics of TAFRO syndrome, which was observed in this case as a membranous proliferative glomerulonephritis (MPGN)-like glomerular lesion. Renal impairment in TAFRO syndrome may be caused by secondary MPGN due to endothelial cell disorder.