边缘区淋巴瘤合并淀粉样变性1例及文献复习

Marginal zone lymphoma associated amyloidosis:A case report and literature review

边缘区淋巴瘤是血液系统中比较常见的惰性恶性肿瘤,而合并淀粉样变性罕见。中南大学湘雅三医院于2018年1月收治1例边缘区淋巴瘤合并心肌淀粉样变性的患者,其以气促半年为主要表现,检查结果提示多浆膜腔积液、IgM κ型M蛋白阳性、心肌淀粉样变性,骨穿考虑成熟小淋巴增殖性疾病,最后骨髓活体组织检查诊断为边缘区淋巴瘤累及骨髓。本疾病的治疗方案包括利妥珠单抗单药治疗,联合放射、化学药物治疗和造血干细胞移植等,由于临床表现缺乏特异性,容易漏诊、误诊,须依靠组织病理及免疫组织化学确诊,预后不良。

The marginal zone lymphoma is a common indolent malignant tumor in the blood system,but the patients with myocardial amyloidosis is rare.In January 2018,a marginal zone lymphoma associated with myocardial amyloidosis was accepted by the Third Xiangya Hospital,Central South University,main complaining of shortness of breath for more than half a year.The patient manifested with multiple serous effusion,positive for IgM κ-type M protein,and myocardial amyloidosis.Bone marrow puncture suggested as mature small lymphocyte proliferative disease,and the bone marrow biopsy eventually diagnosed as marginal zone lymphoma involving the bone marrow.The treatment of this disease includes rituzumab,radiochemotherapy and hematopoietic stem cell transplantation.Due to the lack of sepcificity in sympotoms,it is necessary to rely on histopathological biopsy and immunohistochemistry to confirm the diagnosis.The disease is easily missed or misdiagnosed and the prognosis is poor.