Rajić Ljubica, Bilić Ernest, Femenić Ranka, Mestrović Daniel, Ilić Ivana, Lasan-Trcić Ruzica, Dubravcić Klara, Husar Karmela, Kardum-Skelin Ika, Tesović Goran, Culig Zdravka, Konja Josip
Department of Haematology and Oncology, Pediatric Clinic, Zagreb University Hospital Center, Zagreb, Croatia.
Coll Antropol. 2010 Jun;34(2):679-82.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell lymphoma of CD3+CD8+ phenotype characterized by deep-seated skin nodules or plaques mimicking panniculitis, a result of neoplastic lymphocytes infiltrating the subcutaneous fatty tissue. We present a case of a 19-month year old boy with SPTCL diagnosed and successfully treated in our institution. Disease first presented with symptoms of high fever and painful erythematous nodule located below the umbilicus. Later on the infiltrates appeared on the face, legs, arms and the back of the body. As the most decisive in obtaining the diagnosis, skin biopsy showed atypical, small to medium-sized lymphatic cells infiltrating the deeper dermal layers as well as the subcutaneous adipous tissue surrounding the adipocytes. Immunohystochemical analysis showed neoplastic lymphocytes positive for CD2, CD3, CD5, CD7, CD8, Tia-1, granzyme B and perforine, and negative for CD20, CD34, TDT and CD56. No infiltration of blood vessels or epidermis was evident. Specific T-cell lymphomas protocol (EURO-LB 02) was then initiated which resulted with rapid regression of all general and local symptoms. The treatment was completed according to schedule and the child is now, 24 months after the initiation of the treatment, in complete remission.
皮下脂膜炎样T细胞淋巴瘤(SPTCL)是一种罕见的CD3 + CD8 +表型的T细胞淋巴瘤,其特征为深层皮肤结节或斑块,类似脂膜炎,是肿瘤性淋巴细胞浸润皮下脂肪组织的结果。我们报告一例19个月大的男孩,在我们机构被诊断为SPTCL并成功治疗。疾病最初表现为高热和位于脐下的疼痛性红斑结节。后来,面部、腿部、手臂和身体背部出现浸润。作为确诊的最关键因素,皮肤活检显示非典型的中小淋巴细胞浸润深层真皮层以及围绕脂肪细胞的皮下脂肪组织。免疫组织化学分析显示肿瘤性淋巴细胞CD2、CD3、CD5、CD7、CD8、Tia-1、颗粒酶B和穿孔素呈阳性,而CD20、CD34、末端脱氧核苷酸转移酶(TDT)和CD56呈阴性。未见血管或表皮浸润。随后启动了特定的T细胞淋巴瘤方案(EURO-LB 02),所有全身和局部症状迅速消退。治疗按计划完成,在治疗开始24个月后,患儿目前处于完全缓解状态。