淋巴浆细胞淋巴瘤/华氏巨球蛋白血症1例及文献分析

A case report of lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia and its related literature analysis

淋巴浆细胞淋巴瘤/华氏巨球蛋白血症(Lymphoplasmacytic Lymphoma/Waldenstrom Macroglobulinemia,LPL/WM)是一种罕见的惰性成熟B细胞淋巴瘤,目前对于该疾病的研究现状主要集中于病理表现、治疗方案的研究。该病治疗上以利妥昔单抗联合化疗和自体造血干细胞移植为主。新兴的靶向治疗布鲁顿酪氨酸激酶(Bruton Tyrosine Kinase,BTK)抑制剂疗效显著,但对该疾病的临床表现、鉴别诊断的研究报道较少。因其临床表现具有非特异性,该病常需与其他血液系统疾病鉴别,随着分子机制研究不断进展,髓系分化因子88(Myeloid Differentiation Primary Response 88,MYD88)L265P以及CXCR4 WHIM突变点逐渐被发现,更利于该疾病的诊断明确和与其他血液系统疾病的鉴别诊断。文章通过对1例免疫球蛋白(Immunoglobulin,Ig)M WM的鉴别诊断与治疗进行阐述并结合近年来相关文献进行分析,旨在探讨国内外目前对于WM的临床症状、鉴别诊断及临床治疗等方面的认识,为临床上提前发现和明确诊断该疾病提供经验。

Lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia is a rare indolent mature B-cell lymphoma. The research status of this disease mainly focuses on the study on pathological manifestations and treatment plans. The treatment methods of the disease are mainly rituximab plus chemotherapy, and autologous hematopoietic stem cell transplantation. Bruton tyrosine kinase inhibitor is an emerging targeted therapy with remarkable curative effect, but there are few reports on the clinical manifestations and differential diagnosis of the disease. Because of its non-specific clinical manifestations, the disease often needs to be differentiated from other hematological diseases. With the continuous progress of molecular mechanism research, MYD88 L265P and CXCR4 WHIM mutation points have been gradually discovered, which is more conducive to the diagnosis of the disease and the comparison with other hematological diseases. In this paper, the differential diagnosis and treatment of one case of Ig M WM was described, and the relevant literature in recent years was analyzed, in order to explore the current understanding on the clinical symptoms, differential diagnosis and clinical treatment of WM at home and abroad, and provide more reference for early detection and definitive diagnosis of the disease in clinic.