Insufficient evidence exists to use histopathologic subtype to guide treatment of idiopathic multicentric Castleman disease

Fajgenbaum, David C; Wu, David; Goodman, Aaron M.; Wong, Raymond; Chadburn, Amy; Nasta, Sunita; Srkalović, Gordan; Mukherjee, Sudipto; Leitch, Heather A.; Jayanthan, Raj; Ferrero, Simone; Sato, Yasuharu; Schey, Steve; Dispenzieri, Angela; Oksenhendler, Éric; Zinzani, Pier Luigi; Lechowicz, Mary Jo; Hoffmann, Christian; Pemmaraju, Naveen; Bagg, Adam; Fosså, Alexander; Lim, Megan S.; Rhee, F. Van

doi:10.1002/ajh.25992

Cited by 21 publications

(20 citation statements)

References 32 publications

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“…18,19 We previously reported that patients with both iMCD and TAFRO had uniform clinical features and pathological findings with hyper-vascular proliferation in lymph nodes as well as myelofibrosis and megakaryocyte hyperplasia in bone marrow. 13,20 Thereafter, iMCD patients with TAFRO (iMCD-TAFRO) symptoms have been considered to have an aggressive clinical subtype of iMCD. 19 The iMCD patients who do not meet the criteria for TAFRO often have elevated platelet counts, hypergammaglobulinemia, and a less aggressive course; these cases are described as iMCD not otherwise specified (iMCD-NOS).…”

Section: Introductionmentioning

confidence: 99%

Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease

et al. 2021

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Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly (TAFRO) syndrome is a heterogeneous entity manifesting with a constellation of symptoms described above that can occur in the context of idiopathic multicentric Castleman disease (iMCD) as well as infectious diseases, malignancies, and rheumatologic disorders. So, iMCD-TAFRO is an aggressive subtype of iMCD with TAFRO syndrome and often hyper-vascularized lymph nodes. Since we proposed diagnostic Yoshito Nishimura and Noriko Iwaki contributed equally to this study.

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Section: Introductionmentioning

confidence: 99%

Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease

et al. 2021

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“…These features are often used to classify patients into the hyaline vascular/hypervascular, mixed, or plasmacytic histopathological subtypes; limited data exist to indicate clinical relevance of these subtypes. 7 Recently, patients have been divided into clinical subtypes with prognostic implications, including patients with thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin myelofibrosis/ renal failure, and organomegaly, referred to as the TAFRO subtype of iMCD (iMCD-TAFRO). [8][9][10][11] Other patients, referred to here as iMCD not otherwise specified (iMCD-NOS) are less well characterized but often demonstrate thrombocytosis, hypergammaglobulinemia, and more mild symptoms.…”

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confidence: 99%

Bone marrow findings of idiopathic Multicentric Castleman disease: A histopathologic analysis and systematic literature review

Belyaeva

Rubenstein

Pierson

et al. 2022

Hematological Oncology

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Idiopathic multicentric Castleman disease (iMCD) is a polyclonal lymphoproliferative disorder characterized by constitutional symptoms, generalized lymphadenopathy, cytopenias, and multi-organ dysfunction due to excessive cytokines, notably Interleukin-6. Idiopathic multicentric Castleman disease is often subclassified into iMCD-TAFRO, which is associated with thrombocytopenia (T), anasarca (A), fever/elevated C-reactive protein (F), renal dysfunction (R), and organomegaly (O), and iMCD not otherwise specified (iMCD-NOS), which is typically associated with thrombocytosis and hypergammaglobulinemia. The diagnosis of iMCD is challenging as consensus clinico-pathological diagnostic criteria were only recently established and include several non-specific lymph node histopathological features. Identification of further clinico-pathological features commonly found in iMCD could contribute to more accurate and timely diagnoses. We set out to characterize bone marrow (BM) histopathological features in iMCD, assess differences between iMCD-TAFRO and iMCD-NOS, and determine if these findings are specific to iMCD. Examination of BM specimens from 24 iMCD patients revealed a high proportion with hypercellularity, megakaryocytic atypia, reticulin fibrosis, and plasmacytosis across patients with both iMCD-NOS and iMCD-TAFRO with significantly more megakaryocytic hyperplasia (p = 0.001) in the iMCD-TAFRO cases. These findings were also consistent with BM findings from 185 published cases of iMCD-NOS and iMCD-TAFRO. However, these findings are relatively nonspecific as they can be seen in various other infectious, malignant, and autoimmune diseases. K E Y W O R D S bone marrow, castleman disease, iMCD, megakaryocytic hyperplasia, plasmacytosis, reticulin fibrosis 1 | INTRODUCTION Multicentric Castleman disease (MCD) describes a heterogeneous group of polyclonal lymphoproliferative disorders characterized by intense episodic systemic inflammatory symptoms, generalized lymphadenopathy, cytopenias, and multi-organ dysfunction. 1 Clinical symptoms and disease pathogenesis are driven by excessive cytokines, often including Interleukin-6 (IL-6). 2,3 There are currently three recognized subtypes. Uncontrolled infection with human herpesvirus-8 (HHV8) causes the hypercytokinemia in

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“…Moreover, it is necessary to rule out POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome, IgG4-related disease, hepatic cirrhosis, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome before the diagnosis of TAFRO syndrome [ 2 ], which we have conducted and confirmed through the examination results during hospitalization. In addition, autopsy examination of the retroperitoneal lymphadenopathy did not show Castleman's disease-like features such as hyaline vascular, hypervascular, and/or plasmacytic pathology [ 8 ], as described in the diagnostic criteria for TAFRO syndrome [ 2 ]. We speculate that the biopsy results may have been masked by the administration of steroids and immunosuppressive drugs, which interfered with the expression of the typical pathological characteristic of TAFRO syndrome.…”

Section: Discussionmentioning

confidence: 99%

TAFRO syndrome with a fatal clinical course following BNT162b2 mRNA (Pfizer-BioNTech) COVID-19 vaccination: A case report

Yamada

Sada

Kashihara

et al. 2022

Journal of Infection and Chemotherapy

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Insufficient evidence exists to use histopathologic subtype to guide treatment of idiopathic multicentric Castleman disease

Abstract: Frits van Rhee 22 | the Castleman Disease Collaborative Network Scientific Advisory Board diagnostic criteria international working group and treatment guidelines international working group

Cited by 21 publications

References 32 publications

Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease

Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease

Bone marrow findings of idiopathic Multicentric Castleman disease: A histopathologic analysis and systematic literature review

TAFRO syndrome with a fatal clinical course following BNT162b2 mRNA (Pfizer-BioNTech) COVID-19 vaccination: A case report

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