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

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Japanese variant of multicentric castleman's disease associated with serositis and thrombocytopenia--a report of two cases: is TAFRO syndrome (Castleman- Kojima disease) a distinct clinicopathological entity?

Multicentric Castleman's disease (MCD) is a polyclonal lymphoproliferative disorder that manifests as marked hyper-γ-globulinemia, severe inflammation, anemia, and thrombocytosis. Recently, Takai et al. reported a new disease concept, TAFRO syndrome, named from thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Furthermore, Kojima et al. reported Japanese MCD cases with ef...

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

Multicentric Castleman disease (MCD) describes a heterogeneous group of disorders involving systemic inflammation, characteristic lymph node histopathology, and multi-organ dysfunction because of pathologic hypercytokinemia. Whereas Human Herpes Virus-8 (HHV-8) drives the hypercytokinemia in a cohort of immunocompromised patients, the etiology of HHV-8-negative MCD is idiopathic (iMCD). Recentl...

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Castleman-Kojima disease in a South Asian adolescent.

Angiofollicular lymph node hyperplasia, with the eponym Castleman disease (CD) is a histomorphologic entity uniting a group of diseases with related and occasionally overlapping pathogenesis. Histologic variants of CD include the hyalinevascular, plasmacytic, and mixed types and each of these may be clinically unicentric or multicentric. Recent reports describe a variant of idiopathic multicent...

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Chronic Myelomonocytic Leukemia following Multicentric Castleman Disease

Multicentric Castleman disease (MCD) is a rare nonmalignant lymphoproliferative disorder presenting systemic symptoms such as fever, night sweats, fatigue, anemia, effusions, and multifocal lymphadenopathy. The etiology of MCD has not been clarified to date. The coexistence of MCD with chronic myelomonocytic leukemia (CMML) has been rarely reported. Although the pathogenesis remains unclear, th...

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Siltuximab for Multicentric Castleman Disease-Letter.

We read with great interest the article by Deisseroth and colleagues (1) focusing on the recent global approval of the monoclonal IL6 antibody siltuximab for the treatment of patients with HHV-8-, HIV-negative multicentric Castleman disease (MCD). Up to now, treatment options for this rare lymphoproliferative disorder are very limited. Lately, the phase III, double-blind, randomized trial of va...

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ژورنال

عنوان ژورنال: Blood

سال: 2015

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood-2015-07-662122