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03-06-2022 | IgA Vasculitis | Case Report

Rapidly progressive glomerulonephritis in a patient with angioimmunoblastic T-cell lymphoma: a rare autopsy case showing IgA vasculitis and cylinder-like deposits

Authors: Hirofumi Watanabe, Fumiyoshi Fujishima, Kyoko Inokura, Rui Makino, Kensuke Daikoku, Rui Sasaki, Ryo Ichinohasama, Hiroshi Sato, Kensuke Joh, Hironobu Sasano

Published in: Medical Molecular Morphology | Issue 3/2022

Abstract

Angioimmunoblastic T-cell lymphoma (AITL), a hematological malignancy, originates from follicular helper T cells. The primary site of AITL is the lymph nodes, but extranodal presentation is frequent in patients with advanced stages. Here, we report a rare case of a patient with AITL presenting with rapidly progressive glomerulonephritis (RPGN). The patient underwent computed tomography, which showed systemic lymph node swelling. RPGN was noted at the time of admission. Livedo was observed in the lower limbs with purpura on the foot. The patient was diagnosed with AITL based on lymph node biopsy. Skin biopsy revealed vasculitis with immunoglobulin A (IgA) deposits. Renal biopsy revealed endocapillary proliferative glomerulonephritis with massive subendothelial deposits and intraluminal thrombi. Immunofluorescence showed IgA, IgG, and complement component 3c-predominant granular staining pattern in the capillary and mesangial areas. Electron micrographs demonstrated dense cylindrical-like deposits in the subendothelial space. Chemotherapy drugs were administered, but the patient’s respiratory distress increased until death. Upon autopsy, membranoproliferative glomerulonephritis and extensive necrotizing cellular crescent formation were observed in the glomeruli. Taken together, this case is a rare combination of AITL and RPGN showing both cylinder-like deposits suggestive of cryoglobulinemic glomerulonephritis (CN) and IgA vasculitis.

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Title: Rapidly progressive glomerulonephritis in a patient with angioimmunoblastic T-cell lymphoma: a rare autopsy case showing IgA vasculitis and cylinder-like deposits
Authors: Hirofumi Watanabe
Fumiyoshi Fujishima
Kyoko Inokura
Rui Makino
Kensuke Daikoku
Rui Sasaki
Ryo Ichinohasama
Hiroshi Sato
Kensuke Joh
Hironobu Sasano
Publication date: 03-06-2022
Publisher: Springer Nature Singapore
Keywords: IgA Vasculitis
Lymphoma
IgA Vasculitis
Autopsy
Glomerulonephritis
Published in: Medical Molecular Morphology / Issue 3/2022
Print ISSN: 1860-1480
Electronic ISSN: 1860-1499
DOI: https://doi.org/10.1007/s00795-022-00325-w

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Rapidly progressive glomerulonephritis in a patient with angioimmunoblastic T-cell lymphoma: a rare autopsy case showing IgA vasculitis and cylinder-like deposits

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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