Morales-Polanco M R, Drijansky-Morgenstern R, Murillo-Meza E, Gómez-Morales E
Hematology, ABC Medical Center, Mexico City, Mexico.
Case Rep Gastroenterol. 2008 Mar 13;2(1):109-15. doi: 10.1159/000120757.
Diffuse large B-cell lymphoma is the most common form of lymphoma. It usually begins in the lymph nodes; up to 40% may have an extranodal presentation. According to a definition of primary extranodal lymphoma with presentation only in extranodal sites, there are reports of large B-cell lymphomas limited to liver or spleen as separate entities, and to date there have been only three documented cases of primary hepatosplenic presentation. This paper reports a fourth case. Due to a review of the literature and the clinical course of the case reported, we conclude that primary hepatosplenic large B-cell lymphoma has been found predominantly in females older than 60 years. The patients reported had <2 months of evolution prior to diagnosis, prominent B symptoms, splenomegaly in three and hepatomegaly in two, none with lymph node involvement. All had thrombocytopenia and abnormal liver function tests; three had anemia and elevated serum lactic dehydrogenase levels, two with hemophagocytosis in bone marrow. Because of the previously mentioned data, it can be stated that primary hepatosplenic lymphoma is an uncommon and aggressive form of disease that requires immediate recognition and treatment.
弥漫性大B细胞淋巴瘤是淋巴瘤最常见的形式。它通常始于淋巴结；高达40%的病例可能有结外表现。根据仅在结外部位出现的原发性结外淋巴瘤的定义，有报道称局限于肝脏或脾脏的大B细胞淋巴瘤为独立的实体，迄今为止仅有三例原发性肝脾表现的记录病例。本文报告第四例。通过对文献的回顾以及所报告病例的临床病程，我们得出结论，原发性肝脾大B细胞淋巴瘤主要见于60岁以上的女性。所报告的患者在诊断前病程小于2个月，有明显的B症状，三例有脾肿大，两例有肝肿大，均无淋巴结受累。所有患者均有血小板减少和肝功能检查异常；三例有贫血和血清乳酸脱氢酶水平升高，两例骨髓有噬血细胞现象。基于上述数据，可以说原发性肝脾淋巴瘤是一种罕见且侵袭性的疾病形式，需要立即识别和治疗。
