Atypical hyaline vascular-type castleman's disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy.

Noriko Iwaki, Yasuharu Sato, Katsuyoshi Takata, Eisei Kondo, Kyotaro Ohno, Mai Takeuchi, Yorihisa Orita, Shinji Nakao, Tadashi Yoshino

Research output: Contribution to journalArticle

33 Citations (Scopus)

Abstract

Recently, atypical Castleman's disease (CD) was reported in Japan. This disease is considered as TAFRO syndrome or non-idiopathic plasmacytic lymphadenopathy (IPL), a constellation of clinical symptoms, namely, thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly without hyper-γ-globulinemia. Histopathologically, this disease is similar to hyaline vascular (HV)-type CD. Here, we present a 43-year-old Japanese woman meeting the clinical criteria of TAFRO syndrome who was successfully treated with combined corticosteroid therapy. She showed a rapidly progressive course of thrombocytopenia, systemic lymphadenopathy, fever, anasarca, and increase in acute inflammatory proteins without hyper-γ-globulinemia. Lymph node biopsy was performed and revealed HV-type CD without human herpes virus 8 infection, which was clinicopathologically compatible with non-IPL. The association of these atypical features with well-known multicentric Castleman's disease (MCD), namely, HV-type histology with systemic lymphadenopathy, marked thrombocytopenia even with a high level of interleukin-6, and increased acute inflammatory proteins without hyper-γ-globulinemia, suggests that TAFRO syndrome as presented in our case is a novel entity, which may have been diagnosed as MCD in the past. To define this novel entity more clearly and to demonstrate its etiology, further nationwide surveys of this syndrome and MCD are needed.

Original languageEnglish
Pages (from-to)87-93
Number of pages7
JournalJournal of clinical and experimental hematopathology : JCEH
Volume53
Issue number1
Publication statusPublished - 2013
Externally publishedYes

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Giant Lymph Node Hyperplasia
Hyalin
Vascular Diseases
Thrombocytopenia
Edema
Fever
Reticulin
Virus Diseases
Blood Vessels
Interleukin-6
Histology
Japan
Adrenal Cortex Hormones
Proteins
Fibrosis
Lymph Nodes
Biopsy
Lymphadenopathy
Multi-centric Castleman's Disease

ASJC Scopus subject areas

  • Medicine(all)

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Atypical hyaline vascular-type castleman's disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy. / Iwaki, Noriko; Sato, Yasuharu; Takata, Katsuyoshi; Kondo, Eisei; Ohno, Kyotaro; Takeuchi, Mai; Orita, Yorihisa; Nakao, Shinji; Yoshino, Tadashi.

In: Journal of clinical and experimental hematopathology : JCEH, Vol. 53, No. 1, 2013, p. 87-93.

Research output: Contribution to journalArticle

Iwaki, Noriko ; Sato, Yasuharu ; Takata, Katsuyoshi ; Kondo, Eisei ; Ohno, Kyotaro ; Takeuchi, Mai ; Orita, Yorihisa ; Nakao, Shinji ; Yoshino, Tadashi. / Atypical hyaline vascular-type castleman's disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy. In: Journal of clinical and experimental hematopathology : JCEH. 2013 ; Vol. 53, No. 1. pp. 87-93.
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