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华氏巨球蛋白血症:一例报告。

Waldenstrom's Macroglobulinemia: A case report.

作者信息

Kolikkat Nejima, Moideen Shamsudeen, Khader Aysha, Mohammed T P, Uvais N A

机构信息

Department of Pathology, Iqraa International Hospital and Research Centre, Calicut, Kerala, India.

Department of Internal Medicine, Iqraa International Hospital and Research Centre, Calicut, Kerala, India.

出版信息

J Family Med Prim Care. 2020 Mar 26;9(3):1768-1771. doi: 10.4103/jfmpc.jfmpc_972_19. eCollection 2020 Mar.

DOI:10.4103/jfmpc.jfmpc_972_19
PMID:32509689
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7266205/
Abstract

Waldenstrom's macroglobulinemia (WM) is a rare and slowly progressive disorder, a variant of lymphoplasmacytic lymphoma, which needs therapy only when patient becomes symptomatic. WM presents usually with constitutional symptoms, organomegaly, cytopenias, and hyperviscosity syndrome. This neoplasm is composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that typically involve the bone marrow, and it is associated with an immunoglobulin M (IgM) gammopathy. Here we report the case a 60-year-old male with WM who initially presented with anemia and fatigue. The patient had no lymphadenopathy or any organomegaly. The diagnosis of WM was made after morphological and immunohistochemical examination of bone marrow of the patient along with an elevated serum IgM level. The patient responded well to plasmapheresis and chemotherapy. This case is unusual because the patient lacked the common clinical features of WM. A thorough clinical and hematological work up including serum electrophoresis, bone marrow study, and immunohistochemistry helps in distinguishing WM from other lymphomas and plasma cell dyscrasias.

摘要

华氏巨球蛋白血症(WM)是一种罕见且进展缓慢的疾病,是淋巴浆细胞淋巴瘤的一种变体,仅在患者出现症状时才需要治疗。WM通常表现为全身症状、器官肿大、血细胞减少和高黏滞综合征。这种肿瘤由小淋巴细胞、浆细胞样淋巴细胞和浆细胞组成,通常累及骨髓,并与免疫球蛋白M(IgM)丙种球蛋白病相关。在此,我们报告一例60岁患有WM的男性患者,最初表现为贫血和疲劳。该患者无淋巴结病或任何器官肿大。对患者骨髓进行形态学和免疫组化检查并结合血清IgM水平升高后,确诊为WM。该患者对血浆置换和化疗反应良好。此病例不寻常,因为患者缺乏WM的常见临床特征。包括血清电泳、骨髓检查和免疫组化在内的全面临床和血液学检查有助于将WM与其他淋巴瘤和浆细胞发育异常相鉴别。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4592/7266205/cc19acb85b8a/JFMPC-9-1768-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4592/7266205/ef1106ac5009/JFMPC-9-1768-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4592/7266205/017f03ee922f/JFMPC-9-1768-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4592/7266205/cc19acb85b8a/JFMPC-9-1768-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4592/7266205/ef1106ac5009/JFMPC-9-1768-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4592/7266205/017f03ee922f/JFMPC-9-1768-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4592/7266205/cc19acb85b8a/JFMPC-9-1768-g003.jpg

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本文引用的文献

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Waldenström Macroglobulinemia and Cerebral Venous Thrombosis: From Diagnosis to Complication.华氏巨球蛋白血症与脑静脉血栓形成:从诊断到并发症
Case Rep Med. 2019 Jul 14;2019:9581605. doi: 10.1155/2019/9581605. eCollection 2019.
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Central Nervous System Involvement by Waldenstrom Macroglobulinemia: A Case Report of the Bing-Neel Syndrome.华氏巨球蛋白血症累及中枢神经系统:宾-尼尔综合征1例报告
Case Rep Hematol. 2019 Mar 14;2019:4075960. doi: 10.1155/2019/4075960. eCollection 2019.
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Waldenström macroglobulinemia: 2017 update on diagnosis, risk stratification, and management.
华氏巨球蛋白血症:2017 年诊断、风险分层和治疗更新。
Am J Hematol. 2017 Feb;92(2):209-217. doi: 10.1002/ajh.24557.
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Waldenström macroglobulinemia in hepatitis C: case report and review of the current literature.丙型肝炎合并华氏巨球蛋白血症:病例报告及当前文献综述
Case Rep Oncol Med. 2014;2014:165670. doi: 10.1155/2014/165670. Epub 2014 Aug 27.
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Treatment recommendations for patients with Waldenström macroglobulinemia (WM) and related disorders: IWWM-7 consensus.华氏巨球蛋白血症(WM)及相关疾病患者的治疗建议:IWWM - 7共识
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