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KMID : 0366220140490040253
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Korean Journal of Hematology
2014 Volume.49 No. 4 p.253 ~ p.258
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Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric CastlemanEs disease: a single medical center experience
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Seo Se-Young
Yoo Chang-Hoon
Yoon Dok-Hyun
Kim Shin
Park Jung-Sun
Park Chan-Sik
Huh Joo-Ryung
Suh Cheol-Won
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Abstract
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Background: Multicentric Castleman’s disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD.
Methods: Between 1990 and 2013, 32 patients with multicentric CD were identified from the data-base of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown hu-man immunodeficiency virus infection status, 28 human immunodeficiency virus-neg-ative patients with multicentric CD were included in this analysis.
Results: Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, as-cites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly asso-ciated with patient survival.
Conclusion: Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors.
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KEYWORD
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lticentric Castleman’s disease, Giant lymph node hyperplasia, Angiofollicular lymphoid hyperplasia, Prognosis, HIV
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