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诊断难题:华氏巨球蛋白血症/浆细胞白血病

A Diagnostic Dilemma: Waldenström's Macroglobulinemia/Plasma Cell Leukemia.

作者信息

Sethi Bhawna, Butola K S, Kumar Yogesh

机构信息

Department of Pathology, VCSGGMS & RI Srinagar, Pauri Garhwal, Uttarakhand, India.

出版信息

Case Rep Pathol. 2012;2012:271407. doi: 10.1155/2012/271407. Epub 2012 Sep 9.

Abstract

Waldenström's macroglobulinemia is a B-cell neoplasm characterized by infiltration of the bone marrow by a lymphoplasmacytic infiltrate and an IgM monoclonal gammopathy. It is an uncommon disease with overall incidence of approximately 3 per million persons per year, accounting for approximately 1% to 2% of all hematologic cancers. It has only one-sixth the estimated prevalence of plasma cell myeloma. Disease symptoms can be due to infiltration of bone marrow and other tissue sites by malignant lymphoplasmacytic cells or due to the effects of elevated serum IgM levels. However, patients may present with constitutional symptoms only or may be asymptomatic. In our case, patient presented with chief complaints of fatigability and dyspnoea and was misdiagnosed as plasma cell leukemia on peripheral blood film and bone marrow morphology, but turned out to be a case of Waldenström's macroglobulinemia on cytoflorometry. The patient was referred for chemotherapy but expired on 10th day of admission. The suspected cause of death was cardiorespiratory failure.

摘要

华氏巨球蛋白血症是一种B细胞肿瘤,其特征是骨髓被淋巴浆细胞浸润以及出现IgM单克隆丙种球蛋白病。它是一种罕见疾病,总体发病率约为每年每百万人中有3例,约占所有血液系统癌症的1%至2%。其估计患病率仅为浆细胞骨髓瘤的六分之一。疾病症状可能是由于恶性淋巴浆细胞浸润骨髓和其他组织部位,或者是由于血清IgM水平升高的影响。然而,患者可能仅表现为全身症状或可能无症状。在我们的病例中,患者以疲劳和呼吸困难为主诉就诊,在外周血涂片和骨髓形态学检查中被误诊为浆细胞白血病,但在细胞荧光分析中被证实为华氏巨球蛋白血症。该患者被转诊接受化疗,但在入院第10天死亡。推测的死亡原因是心肺衰竭。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eb3d/3443994/7ef0d63b6d82/CRIM.PATHOLOGY2012-271407.001.jpg

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