Extramedullary Waldenstrom macroglobulinemia

被引：61

作者：

Banwait, Ranjit ^{[1
,2
]}

Aljawai, Yosra ^{[1
,2
]}

Cappuccio, Joseph ^{[1
,2
]}

McDiarmid, Serena ^{[1
,2
]}

Morgan, Elizabeth A. ^{[2
,3
]}

Leblebjian, Houry ^{[1
,2
]}

Roccaro, Aldo M. ^{[1
,2
]}

Laubach, Jacob ^{[1
,2
]}

Castillo, Jorge J. ^{[1
,2
]}

Paba-Prada, Claudia ^{[1
,2
]}

Treon, Steven ^{[1
,2
]}

Redd, Robert ^{[2
,4
]}

Weller, Edie ^{[2
,4
]}

Ghobrial, Irene M. ^{[1
,2
]}

机构：

[1] Dana Farber Canc Inst, Boston, MA 02115 USA

[2] Harvard Univ, Sch Med, Boston, MA USA

[3] Brigham & Womens Hosp, Dept Pathol, Boston, MA 02115 USA

[4] Dana Farber Canc Inst, Dept Biostat & Computat, Boston, MA 02115 USA

来源：

AMERICAN JOURNAL OF HEMATOLOGY | 2015年 / 90卷 / 02期

关键词：

CONSENSUS PANEL RECOMMENDATIONS; 2ND INTERNATIONAL WORKSHOP; MULTIPLE-MYELOMA; MYD88; L265P; CXCR4; MUTATIONS;

D O I：

10.1002/ajh.23880

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Disease assessment in Waldenstrom Macroglobulinemia (WM) is dependent on the percent involvement of B-cell neoplasm in the bone marrow and IgM paraprotein in the serum. A subset of patients also demonstrates extramedullary involvement, which is infrequently examined. The role of extramedullary involvement in the diagnosis and prognosis of WM is poorly understood. The purpose of this study is to report the characteristics of WM patients with extramedullary disease (EMD). Nine hundred and eight-five patients with WM were evaluated at one academic center and the presence of EMD was assessed in these patients. Forty-three (4.4%) patients were identified to have EMD. Nine (21%) patients presented with involvement at WM diagnosis, while 34 (79%) developed EMD post-therapy for WM. Most frequent EMD sites involved were pulmonary (30%), soft tissue (21%), cerebrospinal fluid (23%), renal (8%), and bone (9%). The median overall survival at 10 years was 79% (95% CI: 57-90%). This is the first study to describe the clinical characteristics, response and overall survival in patients with extramedullary WM. Further studies to define the molecular characteristics of this entity and mechanisms of its development are warranted. Am. J. Hematol. 90:100-104, 2015. (c) 2014 Wiley Periodicals, Inc.

引用

页码：100 / 104

页数：5

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