The clinical spectrum of Castleman's disease.

The clinical spectrum of Castleman's disease.
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DOI:
10.1002/ajh.23291
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发表时间:
2012-11
影响因子:
12.8
通讯作者:
Habermann, Thomas M.
Habermann, Thomas M.
中科院分区:
医学1区
文献类型:
--
作者:
Dispenzieri, Angela;Armitage, James O.;Loe, Matt J.;Geyer, Susan M.;Allred, Jake;Camoriano, John K.;Menke, David M.;Weisenburger, Dennis D.;Ristow, Kay;Dogan, Ahmet;Habermann, Thomas M.

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Castleman病(CD)是一种罕见且鲜为人知的淋巴增生性疾病。症状和病程范围很广,但没有大型研究描述这种疾病的自然病史。从梅奥诊所和内布拉斯加州大学评估的113例CD患者的记录中提取基本的临床和实验室数据。评估这些变量对确诊后总生存率(OS)的影响。60例患者有多中心病变。在多中心CD患者中,32%的患者有足够的标准来诊断POEMS综合征。对于所有患者,2年、5年和10年的OS分别为92%、76%、59%。大多数死亡危险因素与POEMS综合征的诊断标准共分离,支持以下4类CD的概念:1)单中心CD(91%);2)多中心CD伴发骨硬化型POEMS综合征(90%);3)多中心CD不伴POEMS综合征(65%);4)多中心CD伴POEMS综合征无骨硬化性病变(27%)。我们已经证明,CD代表一种疾病谱,可以通过简单的预后因素来区分,这为进一步研究提供了一个框架。
Castleman Disease (CD) is a rare, poorly understood lymphoproliferative disease. The spectrum of symptoms and course of disease are broad, but there is no large study describing the natural history of this disease. Basic clinic and laboratory data from the records of 113 patients with CD evaluated at the Mayo Clinic and University of Nebraska were abstracted. The impact of these variables on overall survival (OS) from time of diagnosis was evaluated. Sixty patients had multicentric disease. Of the patients with multicentric CD, 32% had criteria sufficient for a diagnosis of POEMS syndrome. For all patients, 2, 5, and 10-year OS was 92%, 76%, 59%. Most of the factors identified as risk factors for death on univariate analysis co-segregated with diagnostic criteria for POEMS syndrome, which supported the concept of 4 categories of CD, which are (along with their 5-year OS): 1) unicentric CD (91%); 2) multicentric CD associated with the osteosclerotic variant of POEMS syndrome (90%); 3); multicentric CD without POEMS syndrome (65%); and 4) multicentric CD with POEMS syndrome without osteosclerotic lesions (27%). We have demonstrated that CD represents a spectrum of disease that can be differentiated by simple prognostic factors that provide a framework for further study.
DOI: 10.1097/00000478-200301000-00010
发表时间: 2003-01-01
影响因子: 5.6
作者:
Amin, HM;Medeiros, LJ;Jones, D
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发表时间: 2011-07-01
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期刊: BLOOD
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发表时间: 1968-01-01
影响因子: 39.2
作者:
MALLORY, A;SPINK, WW
通讯作者: SPINK, WW