Department of Hematology, Kyushu Railway Memorial Hospital, Kitakyushu, Japan.
Department of Gastroenterology, Kyushu Railway Memorial Hospital, Kitakyushu, Japan.
J Clin Exp Hematop. 2022;62(4):273-278. doi: 10.3960/jslrt.22037.
Lymphoplasmacytic lymphoma (LPL) usually involves bone marrow (BM) and sometimes lymph nodes and spleen. LPL presenting as a hepatic mass lesion is extremely rare, with only one case reported in the English literature. A 70-year-old Japanese female presented to us with a right hypochondriac mass with tenderness. Computed tomography (CT) revealed a 14 cm-sized bulky hepatic mass. Laboratory findings showed a normal white blood cell count of 4.1×10/L with 4% plasmacytoid lymphocytes; normocytic anemia, Hb 9.4 g/dL; high soluble IL-2 receptor level, 2,290 U/mL; and elevated IgG, 10,306 mg/dL. Furthermore, IgG-κ monoclonal protein was detected. F-fluorodeoxyglucose-positron emission tomography/CT revealed abnormal uptake in the liver mass; left supraclavicular, parasternal, abdominal, and left inguinal lymph nodes; and bilateral lung bases. Magnetic resonance imaging showed no bone lesions. BM aspiration and liver biopsy showed predominant infiltration of small lymphocytes admixed with plasmacytoid lymphocytes and plasma cells. In the liver specimen, lymphoepithelial lesions were not observed. The small lymphocytes were positive for CD20, CD79a, and bcl-2, and negative for CD5, CD10, cyclin D1, and IRTA1; plasma cells in BM were positive for CD19, CD45, IgG, and κ-chain, and negative for CD20, and CD56. MYD88 L265P mutation, reported in approximately 40% of non-IgM LPL cases, was not detected in the liver specimen and BM cells. The frequency is lower than that of typical IgM LPL. These findings led us to a diagnosis of LPL with IgG-κ paraproteinemia. The patient underwent four courses of R-CHOP and two courses of Bendamustine-R. Partial remission was achieved.
淋巴浆细胞淋巴瘤（Lymphoplasmacytic lymphoma，LPL）通常累及骨髓（Bone marrow，BM），有时也累及淋巴结和脾脏。表现为肝肿块病变的 LPL 极为罕见，仅在英文文献中有一例报道。一位 70 岁的日本女性因右季肋部肿块伴触痛就诊。计算机断层扫描（Computed tomography，CT）显示 14cm 大小的肝肿块。实验室检查发现白细胞计数正常为 4.1×10/L，其中 4%为浆细胞样淋巴细胞；正细胞性贫血，Hb 9.4g/dL；可溶性白细胞介素 2 受体水平升高，2290U/mL；免疫球蛋白 G（Immunoglobulin G，IgG）升高，10306mg/dL。此外，还检测到 IgG-κ 单克隆蛋白。氟-18 氟代脱氧葡萄糖正电子发射断层扫描/CT 显示肝脏肿块；左锁骨上、胸骨旁、腹部和左腹股沟淋巴结；以及双侧肺基底异常摄取。磁共振成像（Magnetic resonance imaging，MRI）显示无骨病变。BM 抽吸和肝活检显示小淋巴细胞与浆细胞样淋巴细胞和浆细胞混合浸润。肝标本中未观察到淋巴上皮病变。小淋巴细胞 CD20、CD79a 和 bcl-2 阳性，CD5、CD10、cyclin D1 和 IRTA1 阴性；BM 中的浆细胞 CD19、CD45、IgG 和 κ 链阳性，CD20 和 CD56 阴性。在大约 40%的非 IgM LPL 病例中报道的 MYD88 L265P 突变在肝标本和 BM 细胞中未检测到。其频率低于典型的 IgM LPL。这些发现提示我们诊断为 IgG-κ 副蛋白血症 LPL。患者接受了 4 个周期的 R-CHOP 和 2 个周期的苯达莫司汀-R 治疗。达到部分缓解。
