Timár Botond
I. Sz. Patológiai és Kísérleti Rákkutató Intézet, Semmelweis Egyetem, Budapest, Hungary.
Magy Onkol. 2017 Mar 8;61(1):6-11. Epub 2016 Nov 28.
Lymphoplasmacytic lymphoma is a rare low-grade B-cell lymphoma, which is composed of a mixture of small lymphocytes, plasmacytoid cells and plasma cells that typically infiltrate the bone marrow, but lymph nodes and rarely other organs can be involved as well. Waldenström macroglobulinaemia is a lymphoplasmacytic lymphoma with typical bone marrow involvement and is associated with detectable IgM paraproteins. The diagnosis of lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia (LPL/WM) can be challenging, due to similarities to other small B-cell lymphomas with plasmacytic differentiation and/or with IgM paraproteins. The recently discovered MYD88 mutation may help in the diagnosis, as it is present in over 90% of LPL/WMs. This short review covers the pathology of LPL/WM and offers some insight into the new molecular findings that may help in the diagnostic procedure and in the new therapeutic choices.
淋巴浆细胞淋巴瘤是一种罕见的低度B细胞淋巴瘤，由小淋巴细胞、浆细胞样细胞和浆细胞混合组成，通常浸润骨髓，但也可累及淋巴结，很少累及其他器官。华氏巨球蛋白血症是一种伴有典型骨髓受累的淋巴浆细胞淋巴瘤，与可检测到的IgM副蛋白相关。由于与其他具有浆细胞分化和/或IgM副蛋白的小B细胞淋巴瘤相似，淋巴浆细胞淋巴瘤/华氏巨球蛋白血症（LPL/WM）的诊断可能具有挑战性。最近发现的MYD88突变可能有助于诊断，因为超过90%的LPL/WM存在该突变。这篇简短的综述涵盖了LPL/WM的病理学，并对可能有助于诊断程序和新治疗选择的新分子发现提供了一些见解。
