Gao Xiaoning, Li Jie, Lin Ji, Liu Daihong, Yu Li, Wang Quanshun
Department of Hematology, Chinese PLA General Hospital, Medical School of Chinese PLA, Beijing, China (mainland).
Department of Pathology, Chinese PLA General Hospital, Medical School of Chinese PLA, Beijing, China (mainland).
Am J Case Rep. 2017 Jun 1;18:611-616. doi: 10.12659/ajcr.903679.
BACKGROUND Splenic marginal zone lymphomas (SMZLs) are generally uncommon, indolent lymphomas that typically affect older adults, but the development of the transformation to high-grade lymphoma may occur in a small proportion of patients and represents a rare event with blastic cell infiltration in the lymph nodes and bone marrow. CASE REPORT Here, we present a young adult patient who was diagnosed with a SMZL and developed a high-grade transformation to diffuse large B cell lymphoma (DLBCL) with central nervous system involvement. The patient was a 31-year-old woman whose hematologic medical history began with severe anemia and thrombocytopenia associated with atypical lymphoid infiltrate in the bone marrow and massive splenomegaly. A splenectomy was performed and revealed the SMZL. She was first treated with the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) and subsequently with the R-FMD regimen (rituximab, fludarabine, mitoxantrone, and dexamethasone) because the peripheral blood leukocytes were remarkably increased postoperatively. Six months after the splenectomy, she complained of headaches. A magnetic resonance imaging scan of her brain revealed intracerebral tumorous lesions from which a biopsy was taken. On morphological and immunohistochemical examination, the tumor fulfilled the criteria for a DLBCL. Treatment with pulse-dose intravenous methylprednisolone followed by high-dose methotrexate was promptly initiated, but the patient's condition continued to deteriorate and she died of the disease 13 months after the splenectomy. CONCLUSIONS Although there is a general tendency for SMZL to display low aggressiveness, central nervous system involvement associated with a histological transformation to high-grade lymphoma, as presented here, can occur in advanced stage of the disease.
背景 脾边缘区淋巴瘤（SMZL）通常并不常见，是一种惰性淋巴瘤，典型地影响老年人，但一小部分患者可能会发生向高级别淋巴瘤的转化，这是一种罕见事件，表现为淋巴结和骨髓中有母细胞浸润。病例报告 在此，我们报告一名年轻成年患者，其被诊断为SMZL，并发生了向伴有中枢神经系统受累的弥漫性大B细胞淋巴瘤（DLBCL）的高级别转化。该患者为一名31岁女性，其血液病史始于严重贫血和血小板减少，伴有骨髓中非典型淋巴细胞浸润和巨大脾肿大。进行了脾切除术，病理显示为SMZL。她首先接受CHOP方案（环磷酰胺、阿霉素、长春新碱和泼尼松）治疗，随后因术后外周血白细胞显著增加而接受R-FMD方案（利妥昔单抗、氟达拉滨、米托蒽醌和地塞米松）治疗。脾切除术后6个月，她主诉头痛。脑部磁共振成像扫描显示脑内有肿瘤性病变，并进行了活检。经形态学和免疫组化检查，肿瘤符合DLBCL的标准。立即开始用脉冲剂量静脉注射甲泼尼龙随后大剂量甲氨蝶呤进行治疗，但患者病情持续恶化，脾切除术后13个月死于该病。结论 尽管SMZL总体上倾向于表现出低侵袭性，但如此处所示，与向高级别淋巴瘤的组织学转化相关的中枢神经系统受累可发生在疾病晚期。
