Han Shao-Min, Teng Chieh-Lin, Hwang Guang-Yuh, Chou Guan, Tsai Che-An
Division of Hematology/Oncology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan, R.O.C.
J Chin Med Assoc. 2008 Apr;71(4):210-3. doi: 10.1016/S1726-4901(08)70106-0.
Primary splenic lymphoma (PSL) is a rare disease with ambiguous definition, comprising less than 1% of non-Hodgkin's lymphoma. Even rarer is PSL combined with hemophagocytic lymphohistiocytosis (HLH), which has presentations of fever, cytopenia, hepatosplenomegaly, hyperferritinemia, and phagocytosis of hematopoietic cells in the reticuloendothelial system. We report the case of a 77-year-old man who presented with HLH initially. Refusing diagnostic splenectomy, he received chemotherapy. Spontaneous splenic rupture occurred after chemotherapy. In the following emergency operation, PSL was diagnosed. He received another 5 courses of chemotherapy with the R-CNOP regimen (rituximab, cyclophosphamide, mitoxantrone, vincristine, prednisolone). Now he has no residual or relapsed disease. Diagnostic splenectomy for adult HLH patients without definite etiologies may play an important role.
原发性脾淋巴瘤（PSL）是一种定义不明确的罕见疾病，占非霍奇金淋巴瘤的比例不到1%。PSL合并噬血细胞性淋巴组织细胞增生症（HLH）则更为罕见，其表现为发热、血细胞减少、肝脾肿大、高铁蛋白血症以及网状内皮系统中造血细胞的吞噬现象。我们报告一例77岁男性患者，最初表现为HLH。他拒绝了诊断性脾切除术，接受了化疗。化疗后发生自发性脾破裂。在随后的急诊手术中，确诊为PSL。他接受了另外5个疗程的R-CNOP方案（利妥昔单抗、环磷酰胺、米托蒽醌、长春新碱、泼尼松龙）化疗。目前他没有残留或复发疾病。对于病因不明的成人HLH患者，诊断性脾切除术可能起重要作用。
