Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics, Capital Medical University; Key Laboratory of Major Diseases in Children, Ministry of Education; Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
J Pediatr Hematol Oncol. 2024 Jul 1;46(5):e327-e330. doi: 10.1097/MPH.0000000000002868. Epub 2024 May 13.
The occurrence of hemophagocytic lymphohistiocytosis (HLH) in patients with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) may be due to HAVCR2 gene mutation, leading to T-cell immunoglobulin and mucin domain-containing molecule 3 deficiency, T-cell and macrophage activation, and proinflammatory cytokine production.
We report a patient with SPTCL and HLH for whom ruxolitinib, used as a novel treatment, showed notable therapeutic effects.
Remission of both HAVCR2 mutation-induced high inflammatory characteristics and significant symptoms post-ruxolitinib administration suggested that patients with SPTCL and HLH may not represent typical lymphoma cases. Ruxolitinib, with its relatively low toxic side effects, can provide favorable outcomes.
皮下脂膜炎样 T 细胞淋巴瘤（SPTCL）患者发生噬血细胞性淋巴组织细胞增生症（HLH）可能与 HAVCR2 基因突变有关，导致 T 细胞免疫球蛋白和粘蛋白结构域包含分子 3 缺乏，T 细胞和巨噬细胞激活，并产生促炎细胞因子。
我们报告了一例 SPTCL 合并 HLH 患者，新型治疗药物鲁索替尼治疗取得了显著疗效。
鲁索替尼治疗后，HAVCR2 突变引起的高炎症特征和显著症状均得到缓解，提示 SPTCL 合并 HLH 患者可能并非典型的淋巴瘤病例。鲁索替尼具有相对较低的毒副作用，可提供良好的治疗效果。
