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Abstract：
Lymphoplasmacytic lymphoma (LPL) is a malignant proliferation of small lymphocytes, lymphoplasmocytoid cells and plasmocytes affecting the bone marrow, lymph nodes and spleen. Its incidence is 1/100,000 and represents 8% of all lymphomas. A total of ~5% of patients with LPL may secrete non-IgM of IgG, IgA, kappa or lambda type or be non-secretory. In the present study, a case of a 62-year-old female patient who was diagnosed with non-IgM LPL with kappa light chain monoclonal paraprotein production and normal serum immunoglobulin levels was reported. The MYD88 L265P mutation was detected by molecular genetic analysis using a sample of the bone marrow. The patient underwent initial treatment with a combination of Bendamustine-Rituximab, and later on, Ibrutinib (a Bruton kinase inhibitor) was added to the treatment protocol. The authors\' aim was to describe a case of a rare type of LPL studied and cured at the University Hospital \'St. Ivan Rilski\', as well as to show the methods used for its diagnosis and their applicability. The difficulty in diagnosing such rare cases of LPL which are associated with marked plasmacytic differentiation and IgA paraprotein secretion resembling plasma cell neoplasia was addressed. From the other side, the characteristic features in favor of LPL diagnosis are the immunophenotype profile of plasmocytes, as well as the presence of MYD88 L265P mutation. Finally, the methods of management and treatment of this type of lymphoma were reported, highlighting the favorable effect of the treatment with Bruton TK inhibitor (Ibrutinib).
摘要：
淋巴浆细胞性淋巴瘤（LPL）是一种恶性增生的小淋巴细胞，淋巴细胞样细胞和影响骨髓的浆细胞，淋巴结和脾脏。其发病率为1/100,000,占所有淋巴瘤的8%。总共约5%的LPL患者可能分泌IgG的非IgM，IgA,κ或λ型或非分泌型。在本研究中,据报道,1例62岁女性患者被诊断为非IgM型LPL,其κ轻链单克隆副蛋白产生和血清免疫球蛋白水平正常.使用骨髓样品通过分子遗传分析检测MYD88L265P突变。患者接受了苯达莫司汀-利妥昔单抗联合治疗，后来，将依鲁替尼(布鲁顿激酶抑制剂)添加到治疗方案中。作者的目的是描述在大学医院“圣伊万·里尔斯基”研究和治愈的罕见类型的LPL病例，以及显示用于其诊断的方法及其适用性。解决了诊断此类罕见的LPL病例的困难，这些病例与明显的浆细胞分化和类似浆细胞瘤的IgA副蛋白分泌有关。从另一边，有利于LPL诊断的特征是浆细胞的免疫表型，以及MYD88L265P突变的存在。最后,报告了这种类型淋巴瘤的管理和治疗方法，强调布鲁顿TK抑制剂（依鲁替尼）治疗的有利效果。
