儿童皮下脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征一例附文献复习

患儿,女,8岁。皮肤结节、斑块8个月,右大腿肿胀1个月,伴发热1周。肝脏肋下约1~2 cm可触及、脾脏肋下约2~3 cm可触及,质软,无触痛。实验室检查:血细胞降低,肝酶、血脂、乳酸脱氢酶、C反应蛋白升高,血沉加快,凝血功能异常,铁蛋白升高。免疫组化:CD3、CD8、颗粒酶B、βF1阳性,Ki-67:约50%(+)。骨髓活检:骨髓增生活跃。皮损病理检查:皮下脂肪见异型细胞。诊断为脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征。我们对相关文献进行了复习。

Subcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome in chil-dren:a case report and literature review

A female 8-year-old patient presented with skin nodules and plaques for 8 months. She got up-per thigh swelling one month ago and fever for one week. Physical examination revealed hepatomegaly and splenomegaly without tenderness. Laboratory examination revealed significant decreased level of blood cells, and increased level of alanine aminotransferase, glutamic-oxalacetic transaminase, c-reactive protein and high ESR impaired blood coagulation elevated ferritin. Immunohistochemical staining showed positivity of CD3, CD8, Granzyme B,βF1 and Ki-67. Bone marrow biopsy showed proliferous and active. Skin lesion biopsy showed heterocyst in subcutaneous fat. Subcutaneous panniculitis-like T-cell lymphoma complicated by he-mophagocytic syndrome was made. Relevant literature was reviewed.