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

2016 ◽  
Vol 91 (2) ◽  
pp. 220-226 ◽  
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

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

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

scholarly journals TAFRO syndrome or Castleman-Kojima syndrome: a variant of multicentric Castleman disease

Blood ◽  
2015 ◽  
Vol 126 (18) ◽  
pp. 2163-2163 ◽  
Author(s):  
Jennifer M. Hawkins ◽  
Vinodh Pillai
Keyword(s):  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease

The clinicopathological comparison among nodal cases of idiopathic multicentric Castleman disease with and without TAFRO syndrome

2018 ◽  
Vol 77 ◽  
pp. 130-138 ◽  
Author(s):  
Nozomu Kurose ◽  
Chizuru Futatsuya ◽  
Ken-ichi Mizutani ◽  
Motona Kumagai ◽  
Akihiro Shioya ◽  
...  
Keyword(s):  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease

scholarly journals Anasarca, Fever, Thrombocytopenia, Organomegaly, and Multiorgan Failure in a 24-Year-Old Pregnant Woman

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Guillaume Morel ◽  
Joy Mootien ◽  
Philippe Guiot ◽  
Khaldoun Kuteifan
Keyword(s):  
Mechanical Ventilation ◽  
Pregnant Woman ◽  
Cesarean Section ◽  
Hematological Malignancies ◽  
Multiorgan Failure ◽  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease ◽  
First Case ◽  
Systemic Infections

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.

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

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.

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

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.

scholarly journals Kidney biopsy findings in two patients with TAFRO syndrome: case presentations and review of the literature

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Qianyun Zhou ◽  
Yuanyuan Zhang ◽  
Guangping Zhou ◽  
Jihong Zhu
Keyword(s):  
Lymph Node ◽  
Renal Insufficiency ◽  
Epigastric Pain ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease ◽  
Renal Biopsies ◽  
Case Presentations

Abstract Background TAFRO syndrome is a clinical subtype of idiopathic multicentric Castleman disease (iMCD) that is characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis (or renal dysfunction), and organomegaly. TAFRO syndrome has only recently been described, and many clinicians are unaware of this disease, leading to delays in diagnosis and treatment. We present two patients with TAFRO syndrome in whom renal biopsies were performed. Case presentation Both patients had subacute onset and exhibited renal insufficiency, edema, anemia, thrombocytopenia, polyserositis and lymphadenopathy over the disease course. However, there were many differences in their clinical manifestations. Case 1 was a 30-year-old woman admitted due to intermittent vaginal bleeding for 3 weeks. Laboratory tests on admission showed severe renal insufficiency (creatinine: 624 μmol/L), severe anemia (Hb: 41 g/L), and moderate thrombocytopenia (61 × 109/L). Case 2 was a 42-year-old man. Acute epigastric pain was his initial complaint, and computed tomography (CT) revealed retroperitoneal exudation around the pancreas. He was diagnosed with acute pancreatitis, and after treatment with a proton pump inhibitor (PPI) and somatostatin, his abdominal pain still recurred. During treatment, renal failure gradually increased, with oliguria, fever, anemia, thrombocytopenia, edema and massive ascites. Lymph node histologies were consistent with the hyaline-vascular (HV) type and mixed type, respectively, and renal histopathologies were consistent with thrombotic microangiopathy (TMA)-like renal lesions and membranoproliferative glomerulonephritis (MPGN), respectively. Their general conditions improved after glucocorticoid therapy, but their renal functions did not recover completely. On the basis of glucocorticoids, second-line treatments with tocilizumab and rituximab, respectively, were applied. Conclusions The diagnosis of TAFRO syndrome is based mainly on clinical manifestations and lymph node biopsies. A reliable early diagnosis and appropriate rapid treatment are essential to improve patient outcomes. Clinicians should deepen their understanding of this disease and similar conditions. Once the disease is suspected, lymph node biopsies should be performed as soon as possible. In addition, renal biopsies should be actively performed in patients with renal involvement.

scholarly journals Multicentric Castleman disease with TAFRO syndrome and Sjögren's

2019 ◽  
Vol 7 (12) ◽  
pp. 2388-2392 ◽  
Author(s):  
Zi Ying Li ◽  
Shirley Kim ◽  
SiYi Huang ◽  
Raza Mian
Keyword(s):  
Castleman Disease ◽  
Tafro Syndrome ◽  
Multicentric Castleman Disease
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