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.
中科院分区:
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.
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.
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