Castleman-Kojima disease (TAFRO syndrome): a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia …

H Kawabata, K Takai, M Kojima… - Journal of clinical and …, 2013 - jstage.jst.go.jp
H Kawabata, K Takai, M Kojima, N Nakamura, S Aoki, S Nakamura, T Kinoshita, Y Masaki
Journal of clinical and experimental hematopathology, 2013jstage.jst.go.jp
Since the first description by Castleman in 1956, 1 the conceptual view and diagnostic
criteria of Castleman's disease (CD) have been continuously developed. In the 1980s, three
disorders as eponyms of CD were reviewed by Frizzera: localized CD of hyaline-vascular
(HV) type, localized CD of plasma cell (PC) type, and “multicentric-type” CD (MCD). 2-4
MCD refers to an idiopathic clinicopathologic entity characterized by a predominantly
lymphadenopathic appearance consistently involving peripheral lymph nodes. This disorder …
Since the first description by Castleman in 1956, 1 the conceptual view and diagnostic criteria of Castleman’s disease (CD) have been continuously developed. In the 1980s, three disorders as eponyms of CD were reviewed by Frizzera: localized CD of hyaline-vascular (HV) type, localized CD of plasma cell (PC) type, and “multicentric-type” CD (MCD). 2-4 MCD refers to an idiopathic clinicopathologic entity characterized by a predominantly lymphadenopathic appearance consistently involving peripheral lymph nodes. This disorder is regarded as an enigmatic disease because the nodal histologic change of MCD is non-specific and can be found in the context of several clinical situations. 5, 6 Therefore, it is important that all of the known causes of this morphology, which is now expanding, be ruled out before a diagnosis of MCD is made.
MCD was originally described to have a morphology of the PC type of CD by Frizzera et al. 2-4 However, histological findings of their series were characterized by HV-type lymphoid follicles with vascular proliferation and plasmacytosis in the interfollicular area. These histological findings should have been evaluated as the mixed type of CD described by Flendrig. 7 In the early 1980s, Mori et al. demonstrated a new clinicopathologic entity, namely, idiopathic plasmacytic lymphadenopathy (IPL), with polyclonal hyperimmunoglobulinemia showing histological findings that resemble the PC type of CD, but preserving a normal germinal center appearance in the affected nodes. 8 Later, Frizzera concluded that IPL is identical to MCD reported in Western countries, 2, 5 but unfortunately he paid little attention to the significance of the morphologic difference in the germinal centers of MCD, that is, either IPL type or not. Recently, Suda et al. 9 and Kojima et al. 10-13 showed that no Japanese patients with idiopathic MCD are associated with human herpes virus 8 (HHV8), in contrast to those in Western countries. Notably, Kojima et al. further revealed that Japanese patients are delineated into two
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