Ruxolitinib Monotherapy for a Child With HAVCR2 Gene Mutation Associated Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Case Report

Gege Zhang; Chunju Zhou; Ang Wei; Rui Zhang; Yunze Zhao; Honghao Ma; Hongyun Lian; Dong Wang; Tianyou Wang

doi:10.1097/MPH.0000000000002868

Ruxolitinib Monotherapy for a Child With HAVCR2 Gene Mutation Associated Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Case Report

J Pediatr Hematol Oncol. 2024 May 13. doi: 10.1097/MPH.0000000000002868. Online ahead of print.

Authors

Gege Zhang¹, Chunju Zhou², Ang Wei¹, Rui Zhang¹, Yunze Zhao¹, Honghao Ma¹, Hongyun Lian¹, Dong Wang¹, Tianyou Wang¹

Affiliations

¹ 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.
² Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

PMID: 38748615
DOI: 10.1097/MPH.0000000000002868

Abstract

Background: 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.

Observation: We report a patient with SPTCL and HLH for whom ruxolitinib, used as a novel treatment, showed notable therapeutic effects.

Conclusions: 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.