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原发性肺浆细胞瘤伴罕见IgG λ单克隆丙种球蛋白病。

Primary pulmonary plasmacytoma presenting with rare IgG lambda monoclonal gammopathy.

作者信息

Rahim Yasmin, Tareen Farrukh Zia, Ahmed Rashida, Khan Javaid Ahmed

机构信息

Department of Pulmonology and Critical Care, Aga Khan University, Karachi, Pakistan.

Department of Hematology and Oncology, Aga Khan University, Karachi, Pakistan.

出版信息

BMJ Case Rep. 2019 Mar 6;12(3):e227514. doi: 10.1136/bcr-2018-227514.

Abstract

Extramedullaryplasmacytoma (EMP) represents a peculiar and typically progressive malignancy that can originate outside the bone marrow. Primary pulmonary plasmacytoma (PPP) is a rare subset of EMP, confined to the lung. A 55-year-old man, diabetic, non-smoker presented to our clinic with a right chest wall swelling. A routine chest radiograph showed a well-circumscribed opacity in the right upper lung zone. A CT of the chest revealed a large right upper lobe mass with extensive local infiltration. Biopsy and immunohistochemical evaluation led to a diagnosis of PPP. Screening for multiple myeloma was negative. Serum immunofixation showed an IgG lambda monoclonal gammopathy, found in a minority of PPP patients. In view of disease extent, treatment with chemotherapy and radiotherapy was initiated. The patient is currently in out patient follow-up and has shown a favourable response to the treatment with a considerable decrease in serum IgG levels.

摘要

髓外浆细胞瘤(EMP)是一种特殊的、通常呈进行性发展的恶性肿瘤,可起源于骨髓外。原发性肺浆细胞瘤(PPP)是EMP的一种罕见亚型,局限于肺部。一名55岁男性,患有糖尿病,不吸烟,因右胸壁肿胀前来我院就诊。常规胸部X线片显示右上肺野有一个边界清晰的不透光区。胸部CT显示右肺上叶有一个大肿块,伴有广泛的局部浸润。活检和免疫组化评估确诊为PPP。多发性骨髓瘤筛查为阴性。血清免疫固定电泳显示IgG λ单克隆丙种球蛋白病,少数PPP患者中可见。鉴于疾病范围,开始进行化疗和放疗。该患者目前正在门诊随访,对治疗反应良好,血清IgG水平显著下降。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1871/6424279/3b6b077610c1/bcr-2018-227514f01.jpg

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