TAFRO syndrome with skin manifestations treated with bortezomib and tocilizumab; a case report

TAFRO syndrome is a new presentation of idiopathic multicentric Castleman disease which is termed as thrombocytopenia, anasarca, myelofibrosis, renal failure and organomegaly (TAFRO). The exact pathophysiology of TAFRO syndrome is unclear and management is mostly based on case reports and expert opinion. In this report, a 37 years old male patient with TAFRO syndrome is discussed. The patient was referred with fever, sweating, anorexia, abdominal distension and generalized edema which has been hospitalized multiple times for such complaints. The patient also developed skin lesions dispersed in red nodules, which was reported as "granuloid hemangioma". Renal biopsy suggested mesangioproliferative glomerulonephritis and bone marrow specimen showed hypercellular active marrow with reticulin fibrosis. The lymph node biopsies were reported as Castleman disease. This report demonstrates that different manifestations of TAFRO syndrome may overlap with other syndromes and can be managed by Bortezomib and Tocilizumab.

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Idiopathic multicentric Castleman disease preceded by cutaneous plasmacytosis successfully treated by tocilizumab

BMJ Case Reports ◽

10.1136/bcr-2020-236283 ◽

2020 ◽

Vol 13 (11) ◽

pp. e236283

Author(s):

Tetsuro Aita ◽

Sugihiro Hamaguchi ◽

Yoko Shimotani ◽

Yohei Nakamoto

Keyword(s):

Interleukin 6 ◽

Case Reports ◽

Human Herpesvirus ◽

Skin Lesions ◽

Castleman Disease ◽

Follicular Hyperplasia ◽

Multicentric Castleman Disease ◽

Interleukin 6 Receptor ◽

History Of ◽

Asymptomatic Phase

A woman aged 45 years with a 1.5-year history of violaceous plaques on the forehead and chest presented with fever, weight loss and aggravation of the plaques. Inflammatory markers and interleukin-6 level were elevated, and superficial lymphadenopathies and splenomegaly were identified by CT scan. Immunohistochemical findings of the lymph node and the skin showed polyclonal plasmacytosis and follicular hyperplasia, leading to the diagnosis of idiopathic multicentric Castleman disease (iMCD) after human herpesvirus-8 infection was excluded. The patient was successfully treated with anti-interleukin-6 receptor antibody, tocilizumab, following relapse after prednisolone therapy.Our literature review found 11 case reports of pathologically confirmed iMCD preceded by cutaneous plasmacytosis. The median duration of asymptomatic phase with only skin lesions was 7.5 years, whereas the phase lasted only for 1.5 years in our case. iMCD can develop shortly after asymptomatic cutaneous plasmacytosis. Tocilizumab can be a treatment of choice for this type of iMCD.

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Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease

American Journal of Hematology ◽

10.1002/ajh.24242 ◽

2016 ◽

Vol 91 (2) ◽

pp. 220-226 ◽

Cited By ~ 111

Author(s):

Noriko Iwaki ◽

David C. Fajgenbaum ◽

Christopher S. Nabel ◽

Yuka Gion ◽

Eisei Kondo ◽

...

Keyword(s):

Castleman Disease ◽

Tafro Syndrome ◽

Multicentric Castleman Disease ◽

Distinct Subtype

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Glycogen synthase kinase 3β/CCR6‐positive bone marrow cells correlate with disease activity in multicentric Castleman disease‐TAFRO

British Journal of Haematology ◽

10.1111/bjh.17993 ◽

2021 ◽

Author(s):

Nobuya Abe ◽

Michihito Kono ◽

Michihiro Kono ◽

Naoki Ohnishi ◽

Tomoya Sato ◽

...

Keyword(s):

Bone Marrow ◽

Disease Activity ◽

Bone Marrow Cells ◽

Glycogen Synthase ◽

Castleman Disease ◽

Glycogen Synthase Kinase ◽

Glycogen Synthase Kinase 3Β ◽

Multicentric Castleman Disease ◽

Marrow Cells

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The potential role of follicular helper T cells in idiopathic multicentric Castleman disease with and without TAFRO syndrome

Pathology - Research and Practice ◽

10.1016/j.prp.2019.152563 ◽

2019 ◽

Vol 215 (10) ◽

pp. 152563

Author(s):

Nozomu Kurose ◽

Xin Guo ◽

Akihiro Shioya ◽

Ken-ichi Mizutani ◽

Motona Kumagai ◽

...

Keyword(s):

T Cells ◽

Potential Role ◽

Castleman Disease ◽

Helper T Cells ◽

Follicular Helper T Cells ◽

Tafro Syndrome ◽

Multicentric Castleman Disease ◽

Follicular Helper

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Acute Kidney Injury Caused by TAFRO Syndrome in a Chinese Patient: Efficacy of Long-Term Corticosteroids Combined with Bortezomib and Cyclophosphamide

Kidney & Blood Pressure Research ◽

10.1159/000505458 ◽

2020 ◽

Vol 45 (4) ◽

pp. 623-630 ◽

Cited By ~ 1

Author(s):

Peng Xia ◽

Lu Zhang ◽

Menglian Zou ◽

Tengyue Zhang ◽

Ran Li ◽

...

Keyword(s):

Renal Failure ◽

Renal Function ◽

Poor Prognosis ◽

Renal Involvement ◽

Castleman Disease ◽

High Dose ◽

Axillary Lymph ◽

Tafro Syndrome ◽

Vascular Type

Introduction: Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized and rare clinical subtype of Castleman disease. Renal involvement in TAFRO syndrome usually presents with mild proteinuria, microscopic hematuria, and acute renal injury requiring temporary renal replacement. There is no standard therapy available and treatment failures are common, leading to a poor prognosis. We report a case of acute renal failure caused by TAFRO syndrome, successfully managed by long-term corticosteroids combined with bortezomib and cyclophosphamide. Case Presentation: The patient was a 52-year-old female who presented with fever, anasarca, oliguria, and abdominal distension at first. She progressed rapidly to anuric renal failure requiring hemodialysis. She also demonstrated thrombocytopenia, anemia, coagulopathy, and a hyperinflammatory status. Her CT scan showed severe polyserositis, splenomegaly, and lymphadenopathy. Her serum vascular epithelial growth factor level was significantly elevated. Axillary lymph node biopsy showed hyaline-vascular type Castleman disease, supporting the diagnosis of TAFRO syndrome. Her renal function recovered after high-dose steroids and supportive treatment. A weekly dosing regimen of bortezomib, cyclophosphamide, and dexamethasone combined with medium dose prednisone in between were deployed. Her blood cell count and renal function remained stable after 6 months. The inflammation was suppressed and the polyserositis resolved completely. Conclusion: TAFRO syndrome is rare and has a poor prognosis due to the lack of standard treatment. Our patient might be the first TAFRO case successfully treated by bortezomib, cyclophosphamide, and corticosteroids.

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