A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?

• Published in: Orphanet journal of rare diseases Vol. 17; no. 1; p. 432
• Main Authors: Duan, Yanlong, Gao, Huixia, Zhou, Chunju, Jin, Ling, Yang, Jing, Huang, Shuang, Zhang, Meng, Zhang, Yonghong, Wang, Tianyou
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 12.12.2022; BioMed Central; BMC
• Subjects: Adolescent; Autografts; Biopsy; Bone marrow; Cancer; Care and treatment; Case reports; Chemotherapy; Child; Children; Clinicopathological characteristics; Complications and side effects; Corticosteroids; Cyclosporins; Cytotoxicity; Disease; Drug Therapy, Combination; Drug withdrawal; Drugs; Hematology; Hematopoietic stem cells; Hemophagocytic syndrome; Hospitals; Humans; Hybridization; Immunomodulation; Immunomodulator agents; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Immunotherapy; Lymphocytes T; Lymphohistiocytosis, Hemophagocytic - drug therapy; Lymphoma; Lymphoma, T-Cell - drug therapy; Medical prognosis; Medical research; Panniculitis - drug therapy; Panniculitis - pathology; Pathology; Patient outcomes; Patients; Rare diseases; Remission; Remission (Medicine); Retrospective Studies; Skin diseases; Stem cell transplantation; Subcutaneous panniculitis T-cell lymphoma; T cell lymphoma; China; Beijing China; Subcutaneous panniculitis T-cell lymphoma; Chemotherapy; Clinicopathological characteristics; Immunomodulator agents; Children
• ISSN: 1750-1172; 1750-1172
• DOI: 10.1186/s13023-022-02575-4

Subcutaneous panniculitis T-cell lymphoma (SPTCL) is a rare, cytotoxic T-cell lymphoma with which some patients have accompanying hemophagocytic syndrome (HPS). There is currently no standard treatment regimen. In the past, the most commonly used treatment was multidrug chemotherapy. In contrast, numerous case reports or small series suggest that immunosuppressive drugs could also be effective for some patients. Since this NHL subtype is extremely rare in children and adolescents, to improve the understanding of this disease and standardize its rational treatment, we retrospectively summarized the treatment regimens of 18 pathologically diagnosed children with SPTCL to compare the clinical efficacy of multidrug chemotherapy and immunomodulatory therapy. The median age of onset was 11.1 years. Painless subcutaneous nodules or skin patchy lesions were found in all patients, most commonly involving the lower extremities and/or trunk. Before January 1, 2019, the treatment was mainly chemotherapy, and 10 patients were initially treated with chemotherapy, among whom was one patient who progressed during initial treatment, was voluntarily discharged and was subsequently lost to follow-up, one patient who died of disease progression, and the remaining 8 patients who all achieved sustained remission, with a complete remission (CR) rate of 80% (8/10). Corticosteroids combined with cyclosporine A or ruxolitinib were the most common initial immunosuppressive agents at our center after January 1, 2019 and had a CR rate of 71.4% (5/7). In addition, 1 patient achieved partial remission (PR) during follow-up, and one had autologous hematopoietic stem cell transplantation (AHSCT) after 4 months of drug withdrawal. There were 7 patients (38.9%, one case in chemotherapy group and six cases in immunotherapy group) with HPS and 4/5 screened patients (80%) with positive HAVCR2 gene mutations. The median follow-up was 17 months. The prognosis of SPTCL is relatively good. Previous multi-drug and long-term chemotherapy treatment has clear efficacy, and recent immunomodulatory therapy as pre-chemotherapy therapy can also benefit patients.