The clinical spectrum of Castleman's disease

Castleman's 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 eval...

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Veröffentlicht in:American journal of hematology 2012-11, Vol.87 (11), p.997-1002
Hauptverfasser: 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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container_end_page 1002
container_issue 11
container_start_page 997
container_title American journal of hematology
container_volume 87
creator 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.
description Castleman's 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 ed. 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%, respectively. Most of the factors identified as risk factors for death on univariate analysis cosegregated with diagnostic criteria for POEMS syndrome, which supported the concept of four 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. Am. J. Hematol. 2012. © 2012 Wiley Periodicals, Inc.
doi_str_mv 10.1002/ajh.23291
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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 ed. 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%, respectively. Most of the factors identified as risk factors for death on univariate analysis cosegregated with diagnostic criteria for POEMS syndrome, which supported the concept of four 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. Am. J. 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J. Hematol</addtitle><description>Castleman's 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 ed. 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%, respectively. Most of the factors identified as risk factors for death on univariate analysis cosegregated with diagnostic criteria for POEMS syndrome, which supported the concept of four 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. Am. J. 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Hematologic involvement in other diseases</subject><subject>POEMS Syndrome - complications</subject><subject>POEMS Syndrome - diagnosis</subject><subject>POEMS Syndrome - mortality</subject><subject>POEMS Syndrome - therapy</subject><subject>Proportional Hazards Models</subject><subject>Retrospective Studies</subject><subject>Treatment Outcome</subject><subject>Young Adult</subject><issn>0361-8609</issn><issn>1096-8652</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kd1rFDEUxYModlt98B-QAREVnPYmmXy9CO1iW6UoQtXHkMnecbPOx5rMqP3vTbvb9QN8SiC_e87NOYQ8onBIAdiRWy0PGWeG3iEzCkaWWgp2l8yAS5rvYPbIfkorAEorDffJHmPK0IqqGXl5ucTCt6EP3rVFWqMf49QVQ1PMXRpb7Fz_LBWLkNAlfEDuNa5N-HB7HpCPp68v5-flxfuzN_Pji9ILpWiJTvJagGN1DVILo32NC6RayIUXWhpda8oa6SsnsNLYUFFBwzyAlI0RHPgBebXRXU91hwuP_Rhda9cxdC5e2cEF-_dLH5b2y_DdcgNQGZkFnm8F4vBtwjTaLiSPbet6HKZkaQ7CVEbpKqNP_kFXwxT7_L0biikhOc_Uiw3l45BSxGa3DAV73YHNHdibDjL7-M_td-Rt6Bl4ugVcyqk30fU-pN-cFDIbX8dwtOF-hBav_u9oj9-e31qXm4mQRvy5m3Dxq5WKK2E_vzuzJ59OleYfhDX8F6phqkk</recordid><startdate>201211</startdate><enddate>201211</enddate><creator>Dispenzieri, Angela</creator><creator>Armitage, James O.</creator><creator>Loe, Matt J.</creator><creator>Geyer, Susan M.</creator><creator>Allred, Jake</creator><creator>Camoriano, John K.</creator><creator>Menke, David M.</creator><creator>Weisenburger, Dennis D.</creator><creator>Ristow, Kay</creator><creator>Dogan, Ahmet</creator><creator>Habermann, Thomas M.</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><general>Wiley-Liss</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>K9.</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>201211</creationdate><title>The clinical spectrum of Castleman's disease</title><author>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.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5771-ea63b50a2bb068598cbede1856dc58698b812f6c4a5e48ef1540f2c0066f95303</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Biological and medical sciences</topic><topic>Biopsy, Needle</topic><topic>Castleman Disease - complications</topic><topic>Castleman Disease - diagnosis</topic><topic>Castleman Disease - mortality</topic><topic>Castleman Disease - therapy</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Data Interpretation, Statistical</topic><topic>Disease-Free Survival</topic><topic>Female</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Hematology</topic><topic>Humans</topic><topic>Kaplan-Meier Estimate</topic><topic>Leukemias. 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J. Hematol</addtitle><date>2012-11</date><risdate>2012</risdate><volume>87</volume><issue>11</issue><spage>997</spage><epage>1002</epage><pages>997-1002</pages><issn>0361-8609</issn><eissn>1096-8652</eissn><coden>AJHEDD</coden><abstract>Castleman's 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 ed. 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%, respectively. Most of the factors identified as risk factors for death on univariate analysis cosegregated with diagnostic criteria for POEMS syndrome, which supported the concept of four 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. Am. J. Hematol. 2012. © 2012 Wiley Periodicals, Inc.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>22791417</pmid><doi>10.1002/ajh.23291</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record>
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subjects Adolescent
Adult
Aged
Biological and medical sciences
Biopsy, Needle
Castleman Disease - complications
Castleman Disease - diagnosis
Castleman Disease - mortality
Castleman Disease - therapy
Child
Child, Preschool
Data Interpretation, Statistical
Disease-Free Survival
Female
Hematologic and hematopoietic diseases
Hematology
Humans
Kaplan-Meier Estimate
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Lymph Nodes - pathology
Male
Medical sciences
Middle Aged
Other diseases. Hematologic involvement in other diseases
POEMS Syndrome - complications
POEMS Syndrome - diagnosis
POEMS Syndrome - mortality
POEMS Syndrome - therapy
Proportional Hazards Models
Retrospective Studies
Treatment Outcome
Young Adult
title The clinical spectrum of Castleman's disease
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