Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature

To review and summarize the clinical features, treatment strategies, and prognosis of subcutaneous panniculitis-like T-cell lymphoma complicated with hemophagocytic lymphohistiocytosis (SPTCL–HLH). We searched the Web of Science, Embase, Cochrane Library, and PubMed databases. The keywords were subc...

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Published in:Clinical and experimental medicine 2023-12, Vol.23 (8), p.4575-4583
Main Authors: Ou, Wenxin, Zhao, Yunze, Wei, Ang, Ma, Honghao, Zhang, Liping, Lian, Hongyun, Zhang, Qing, Wang, Dong, Li, Zhigang, Wang, Tianyou, Zhang, Rui
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones
Anthracycline
Anthracyclines
Arthritis
Autoantibodies
Autoimmune diseases
Chemotherapy
Clinical Practice
Corticosteroids
Disease
Hematology
Hemophagocytic syndrome
Histiocytosis
Humans
Immunoregulation
Immunosuppressive agents
Internal Medicine
Lymphatic diseases
Lymphocytes T
Lymphocytosis
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - drug therapy
Lymphohistiocytosis, Hemophagocytic - genetics
Lymphoma
Medical prognosis
Medicine
Medicine & Public Health
Mutation
Oncology
Panniculitis - complications
Panniculitis - diagnosis
Panniculitis - drug therapy
Patients
Point mutation
Prognosis
Remission
Remission (Medicine)
Stem cell transplantation
Steroids
Systematic review
T-cell lymphoma
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Summary:To review and summarize the clinical features, treatment strategies, and prognosis of subcutaneous panniculitis-like T-cell lymphoma complicated with hemophagocytic lymphohistiocytosis (SPTCL–HLH). We searched the Web of Science, Embase, Cochrane Library, and PubMed databases. The keywords were subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis or hemophagocytic syndrome. The patients were divided into a mutated group and a wild-type group based on the existence of HAVCR2 gene mutation. A total of 45 reports, including 63 patients with SPTCL–HLH, were included in the systematic review. Twelve patients detected gene mutations, including 11 with the HAVCR2 gene mutation and 1 with the STXBP2 gene mutation. Thirty-one patients were tested for autoantibodies. Compared with the wild-type group, patients in the mutated group were younger ( p  = 0.017), and the autoantibody-positive rate was higher ( p  = 0.006). The main treatment target of 17 patients was to control HLH, yielding an ORR of 88.2%. Two cases relapsed, and both were treated with corticosteroid monotherapy. The corticosteroid monotherapy experienced a higher recurrence rate than the corticosteroids plus other immunoregulatory agents therapy (66.7 vs. 0.0%, p  = 0.029). Eighteen patients received initial anthracycline-based chemotherapy, and 50.0% reached remission. The ORR of initial chemotherapy aiming at controlling HLH was higher than those of anthracycline-based chemotherapy ( p  = 0.015). The ORR was higher in patients initially controlled for HLH versus chemotherapy without HLH control first (90.5 vs. 61.5%, p  = 0.024). Interestingly, one patient with juvenile idiopathic arthritis developed SPTCL–HLH during tocilizumab therapy, discontinuing tocilizumab led to a remission of the disease spontaneously. Sixteen patients received stem cell transplantation (SCT). Fifteen patients, including 5 with relapsed/refractory SPTCL–HLH, responded well and survived after receiving SCT. One case who received a sibling-identical SCT relapsed. Further analysis revealed a homozygous HAVCR2 mutation with the donor. The 2-year overall survival (OS) was 91.0% ± 4.4%. There was a significant difference in the OS among patients of different age groups, and patients aged 40–60 had the lowest 2-year OS (66.7% ± 19.2%). Patients with HAVCR2 gene mutations are younger and more likely to be misdiagnosed with autoimmune diseases. Initial treatment of corticosteroids plus immunoregulato
ISSN:1591-9528
1591-8890
1591-9528
DOI:10.1007/s10238-023-01210-1