Successful treatment of TAFRO syndrome, a variant type of multicentric Castleman disease with thrombotic microangiopathy, with anti-IL-6 receptor antibody and steroids

TAFRO syndrome is a distinct idiopathic multicentric Castleman disease characterized by the association of thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We report the first case occurring in a Caucasian pregnant woman. At 34 weeks of gestation, our patient presented with all clinical and biological symptoms compatible with a TAFRO syndrome. Tough quick cesarean section was performed as symptoms got worse with onset of multiorgan failure requiring mechanical ventilation for acute respiratory distress, continuous renal replacement, and vasopressors. Nine days after ICU admission, steroid boluses were started and allowed spectacular clinical and biological improvement. As systemic inflammatory manifestations are important, TAFRO syndrome can be mistaken with severe autoimmune diseases, systemic infections, hematological malignancies, or hemophagocytic lymphohistiocytosis.

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

Immunopathologia Persa ◽

10.15171/ipp.2020.01 ◽

2019 ◽

Vol 6 (1) ◽

pp. e01-e01

Author(s):

Seyedeh Tahereh Mohaddes ◽

Zahra Rezaei Borojerdi ◽

Maryam Miri ◽

Mohammad Moeini Nodeh ◽

Alireza Rezaei ◽

...

Keyword(s):

Bone Marrow ◽

Renal Failure ◽

Case Reports ◽

Abdominal Distension ◽

Skin Lesions ◽

Castleman Disease ◽

Tafro Syndrome ◽

Multicentric Castleman Disease ◽

Bone Marrow Specimen ◽

Mesangioproliferative Glomerulonephritis

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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TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy

Case Reports in Oncology ◽

10.1159/000518079 ◽

2021 ◽

pp. 1359-1365

Author(s):

Corinne Williams ◽

Alexis Phillips ◽

Vikram Aggarwal ◽

Liron Barnea Slonim ◽

David C. Fajgenbaum ◽

...

Keyword(s):

Monoclonal Antibody ◽

Delayed Diagnosis ◽

Antibody Therapy ◽

Castleman Disease ◽

Definitive Diagnosis ◽

High Morbidity ◽

Monoclonal Antibody Therapy ◽

Tafro Syndrome ◽

Multicentric Castleman Disease ◽

Effective Treatment Modality

TAFRO syndrome is defined by the presence of thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis/renal dysfunction (R), and organomegaly (O) and can be seen with idiopathic multicentric Castleman disease (iMCD) or as an isolated process without iMCD. Although the diagnosis of iMCD in patients with TAFRO can be challenging to make, iMCD should remain high on the differential diagnosis. Similar to iMCD, the pathophysiology of TAFRO is not well understood but is thought to be related to hypercytokinemia, with interleukin (IL)-6 playing a pivotal role. Anti-IL-6 monoclonal antibody therapy is an effective treatment modality for iMCD, but to date, there is no clear guidance on treatment of TAFRO in the absence of definitive diagnosis of iMCD, leading to suboptimal management and high morbidity. We report a case of TAFRO syndrome and demonstrate benefit with the empiric use of anti-IL-6 antibody therapy in the context of delayed diagnosis of iMCD.

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