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免疫抑制治疗效果良好的儿童皮下脂膜炎样T细胞淋巴瘤:两例报告

Pediatric subcutaneous panniculitis-like T-cell lymphoma with favorable result by immunosuppressive therapy: a report of two cases.

作者信息

Nagai Kozo, Nakano Naoko, Iwai Tsuyako, Iwai Asayuki, Tauchi Hisamichi, Ohshima Kouichi, Ishii Eiichi

机构信息

Department of Hematology and Oncology, Shikoku Medical Center for Children and Adults , Kagawa , Japan.

出版信息

Pediatr Hematol Oncol. 2014 Sep;31(6):528-33. doi: 10.3109/08880018.2014.896062. Epub 2014 Mar 31.

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare type of skin lymphoma. Histopathology mimicking a lobular panniculitis makes it difficult to distinguish SPTL from benign autoimmune disease. We present cases of a 10-year-old female and an 11-year-old male with SPTL showing recurrent panniculitis and systemic manifestations. Initially, antibiotics and steroids were administered to treat infectious disease and benign panniculitis. However, they experienced recurrent fever and erythema nodosum. Additional immunohistochemistry and T-cell receptor (TCR) gene rearrangement analyses were performed, enabling the establishment of an SPTL diagnosis. The affected patients were given immunosuppressive therapy with favorable results.

摘要

皮下脂膜炎样T细胞淋巴瘤(SPTL)是一种罕见的皮肤淋巴瘤。其组织病理学表现类似小叶性脂膜炎,使得SPTL难以与良性自身免疫性疾病相区分。我们报告了1例10岁女性和1例11岁男性SPTL病例,均表现为复发性脂膜炎和全身症状。最初,给予抗生素和类固醇治疗感染性疾病和良性脂膜炎。然而,他们仍反复发热和出现结节性红斑。进一步进行了免疫组织化学和T细胞受体(TCR)基因重排分析,从而确诊为SPTL。对受累患者给予免疫抑制治疗,效果良好。

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