乳腺NK/T细胞淋巴瘤合并噬血细胞综合征一例并文献复习

Breast NK/T cell lymphoma combined with hemophagocytic syndrome： a case report and review of the literatures

目的 探讨乳腺NK/T细胞淋巴瘤合并噬血细胞综合征患者的临床特征.方法 报道1例35岁女性乳腺NK/T细胞淋巴瘤合并噬血细胞综合征的患者,分析其诊治经过并复习相关文献.结果 该患者以乳腺包块为首发症状,后出现发热,按乳腺炎抗感染治疗2个月余,无效,逐渐出现白细胞减低进而全血细胞减低,出现高三酰甘油血症、低纤维蛋白原血症,骨髓中可见吞噬红细胞现象,铁蛋白、乳酸脱氢酶异常升高,白细胞内EB病毒拷贝数异常升高,肝功能异常等.经腋下淋巴结及乳腺包块活组织检查最终诊断为NK/T细胞淋巴瘤.给予激素及依托泊苷化疗,肝功能基本恢复正常,凝血功能好转,铁蛋白、各种酶学指标降低,患者最终死于肺部感染.结论 淋巴瘤合并噬血细胞综合征临床表现缺乏特异性,常易误诊、漏诊,且病情进展凶险,预后较差.

Objective To explore the clinical features of breast NK/T cell lymphoma with hemophagocytic syndrome （HPS）.Methods Clinical data of a 35-year old female patient with breast NK/T cell lymphoma combined with HPS was analyzed and the literatures were reviewed.Results The patient with breast mass as starting symptom,following by fever.Anti-infection according to mastitis was invalid.Gradually appear leukocytopenia,pancytopenia,high triglyceride,low fibrinogen concentration.Phagocytes addicted to red blood cells in bone marrow were observed.Ferritin and lactic dehydrogenase were abnomally elebated.EB virus copy numbers abnormally elevated in leukocyte and the liver function grew worse.Breast NK/T cell lymphoma was diagnosed finally through the axillary lymph nodes and breast biopsy.Glucocorticoid and etoposide chemotherapy restored liver function.Blood coagulation function,ferritin and all kinds of enzymology indexes were reduced.But the patient finally died of the lung infection.Conclusions Clinical manifestation of lymphoma combined with HPS is complicated,lacking specificity,often easy to misdiagnosis and missed diagnosis.The progression is in disaster and has poor prognosis.