%0 Case Reports
%T Ruxolitinib Monotherapy for a Child With HAVCR2 Gene Mutation Associated Subcutaneous Panniculitis-like T-cell Lymphoma: A Case Report.
%A Zhang G
%A Zhou C
%A Wei A
%A Zhang R
%A Zhao Y
%A Ma H
%A Lian H
%A Wang D
%A Wang T
%J J Pediatr Hematol Oncol
%V 46
%N 5
%D 2024 Jul 1
%M 38748615
%F 1.17
%R 10.1097/MPH.0000000000002868
%X 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.
METHODS: 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.