Gorodetskiy Vadim R, Probatova Natalya A, Vasilyev Vladimir I
Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Kashirskoye shosse 34A, Moscow, 115522, Russia.
Department of Pathology, N.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical Sciences, Kashirskoye shosse 24, Moscow, 115478, Russia.
J Med Case Rep. 2017 Apr 20;11(1):116. doi: 10.1186/s13256-017-1266-7.
Primary lymph node plasmacytoma is a rare disease that typically involves lymph nodes of the neck. In only 15% of cases is the disease generalized. Here, we present a case of generalized lymph node plasmacytoma in a patient with Sjögren's syndrome with an unusual course.
A 48-year-old white woman presented to our hospital with enlargement of groups of lymph nodes, liver, and spleen. Her medical history was consistent with a 12-year course of Sjögren's syndrome. Blood and urine immunochemistry showed a massive (72 g/l) M-gradient formed from immunoglobulin Aκ in the serum and monoclonal free κ-type light chains in her urine. A skeletal X-ray revealed no bone destruction. Cytological and histological bone marrow assays showed no signs of plasma cell infiltration. The microarchitecture of her neck and inguinal lymph nodes was destroyed. Only small remnants of B cell follicles were found, while the interfollicular areas were expanded and infiltrated by CD138, MuM1, CD43, and IgAκ-positive plasma cells. After nine cycles of doxorubicin, cyclophosphamide, vincristine, and prednisolone chemotherapy, complete remission was achieved. However, the lymphoma relapsed 3 months later, with histological verification in her femoral lymph node. Despite the absence of subsequent adequate therapy, she gradually achieved complete remission of plasmacytoma with the disappearance of paraproteins.
Currently, primary lymph node plasmacytoma is generally considered a nodal marginal zone lymphoma with an extensive plasmacytic differentiation. In our case, despite the critical histological and immunohistochemical evaluation of three lymph node biopsies from different anatomical areas at different times, no signs of nodal marginal zone lymphoma were found. An 18-year follow-up of our patient with primary lymph node plasmacytoma demonstrated an extremely unusual clinical course. Initially, primary lymph node plasmacytoma was refractory to chemotherapy. However, subsequently, she underwent a complete spontaneous remission of plasmacytoma.
原发性淋巴结浆细胞瘤是一种罕见疾病，通常累及颈部淋巴结。仅15%的病例会出现疾病播散。在此，我们报告1例患有干燥综合征的患者发生播散性淋巴结浆细胞瘤，其病程异常。
一名48岁白人女性因多组淋巴结、肝脏和脾脏肿大就诊于我院。她的病史与12年的干燥综合征病程相符。血液和尿液免疫化学检查显示血清中由免疫球蛋白Aκ形成大量（72 g/l）M峰，尿液中有单克隆游离κ型轻链。骨骼X线检查未发现骨质破坏。细胞学和组织学骨髓检查未显示浆细胞浸润迹象。其颈部和腹股沟淋巴结的微结构被破坏。仅发现少量B细胞滤泡残余，而滤泡间区域扩大，并被CD138、MUM1、CD43和IgAκ阳性浆细胞浸润。经过9个周期的阿霉素、环磷酰胺、长春新碱和泼尼松龙化疗后，实现了完全缓解。然而，3个月后淋巴瘤复发，经组织学证实位于其股淋巴结。尽管随后未进行充分治疗，但她的副蛋白消失，浆细胞瘤逐渐实现完全缓解。
目前，原发性淋巴结浆细胞瘤一般被认为是具有广泛浆细胞分化的淋巴结边缘区淋巴瘤。在我们的病例中，尽管对来自不同解剖区域的3次淋巴结活检在不同时间进行了严格的组织学和免疫组化评估，但未发现淋巴结边缘区淋巴瘤的迹象。对我们这位原发性淋巴结浆细胞瘤患者进行的18年随访显示了极其异常的临床病程。起初，原发性淋巴结浆细胞瘤对化疗耐药。然而，随后她的浆细胞瘤实现了完全自发缓解。
