Department of Pediatrics, Division of Hematology and Oncology.
Department of Medicine, Division of Hematology.
J Pediatr Hematol Oncol. 2021 Jan;43(1):e80-e84. doi: 10.1097/MPH.0000000000001954.
We identified 3 adolescents with alpha-beta subtype subcutaneous panniculitis-like T-cell lymphoma.
Three patients presented with prolonged fever, abnormal skin lesions, and cytopenia described in the context. All had the same disease entity, which showed the prolonged duration of B systemic symptoms till diagnosis, difficulty to distinguish from autoimmune diseases, presence of hemophagocytic lymphohistiocytosis syndrome, good response, and remained on long-term remission with nonchemotherapy treatment, which included oral corticosteroid and cyclosporin.
Although diagnosis can only be "highly suspected" with pathologic review, some cases may need multiple serial skin biopsy to clarify diagnosis because of the discrete distribution of specific histology. T-cell receptor gene rearrangement, which demonstrates a monoclonal pattern of alpha and beta chain gene, is the essential requirement for specific diagnosis. The role of molecular analysis by identification of germline hepatitis A virus cellular receptor 2 (HAVCR2) gene mutation can be much valuable in classifying susceptible patients.
我们鉴定了 3 例 alpha-beta 亚型皮下脂膜炎样 T 细胞淋巴瘤的青少年患者。
3 名患者均表现为长期发热、异常皮肤病变和血细胞减少。所有患者均患有同一疾病实体，其 B 系统症状持续时间长至诊断，难以与自身免疫性疾病相区别，存在噬血细胞性淋巴组织细胞增生症，对非化疗治疗反应良好，长期缓解，治疗包括口服皮质类固醇和环孢素。
尽管通过病理检查只能“高度怀疑”诊断，但由于特定组织学的分布离散，某些病例可能需要多次连续皮肤活检以明确诊断。T 细胞受体基因重排，表现为 alpha 和 beta 链基因的单克隆模式，是明确诊断的必要条件。通过鉴定种系甲型肝炎病毒细胞受体 2（HAVCR2）基因突变进行分子分析的作用在分类易感患者方面具有很大价值。
