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

被引：0

作者：

Zhang, Gege ^{[1
,2
,3
,4
]}

Zhou, Chunju ^{[5
]}

Wei, Ang ^{[1
,2
,3
,4
]}

Zhang, Rui ^{[1
,2
,3
,4
]}

Zhao, Yunze ^{[1
,2
,3
,4
]}

Ma, Honghao ^{[1
,2
,3
,4
]}

Lian, Hongyun ^{[1
,2
,3
,4
]}

Wang, Dong ^{[1
,2
,3
,4
]}

Wang, Tianyou ^{[1
,2
,3
,4
]}

机构：

[1] Capital Med Univ, Hematol Ctr, Beijing Key Lab Pediat Hematol Oncol, Beijing Childrens Hosp, Beijing, Peoples R China

[2] Capital Med Univ, Natl Key Discipline Pediat, Beijing, Peoples R China

[3] Capital Med Univ, Minist Educ, Key Lab Major Dis Children, Beijing, Peoples R China

[4] Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, Beijing, Peoples R China

[5] Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, Dept Pathol, Beijing, Peoples R China

来源：

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY | 2024年 / 46卷 / 05期

关键词：

HAVCR2; mutation; hemophagocytic lymphohistiocytosis; ruxolitinib; subcutaneous panniculitis-like T-cell lymphoma;

D O I：

10.1097/MPH.0000000000002868

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页码：e327 / e330

页数：4

共 12 条

[1] Pediatric hemophagocytic lymphohistiocytosis [J].

Canna, Scott W. ;

Marsh, Rebecca A. .

BLOOD, 2020, 135 (16) :1332-1343

[2]

Chaweephisal P, 2021, J PEDIAT HEMATOL ONC, V43, pE80, DOI 10.1097/MPH.0000000000001954

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

Duan, Yanlong ;

Gao, Huixia ;

Zhou, Chunju ;

Jin, Ling ;

Yang, Jing ;

Huang, Shuang ;

Zhang, Meng ;

Zhang, Yonghong ;

Wang, Tianyou .

ORPHANET JOURNAL OF RARE DISEASES, 2022, 17 (01)

[4] Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome [J].

Gayden, Tenzin ;

Sepulveda, Fernando E. ;

Dong-Anh Khuong-Quang ;

Pratt, Jonathan ;

Valera, Elvis T. ;

Garrigue, Alexandrine ;

Kelso, Susan ;

Sicheri, Frank ;

Mikael, Leonie G. ;

Hamel, Nancy ;

Bajic, Andrea ;

Dali, Rola ;

Deshmukh, Shriya ;

Dervovic, Dzana ;

Schramek, Daniel ;

Guerin, Frederic ;

Taipale, Mikko ;

Nikbakht, Hamid ;

Majewski, Jacek ;

Moshous, Despina ;

Charlebois, Janie ;

Abish, Sharon ;

Bole-Feysot, Christine ;

Nitschke, Patrick ;

Bader-Meunier, Brigitte ;

Mitchell, David ;

Thieblemont, Catherine ;

Battistella, Maxime ;

Gravel, Simon ;

Van-Hung Nguyen ;

Conyers, Rachel ;

Diana, Jean-Sebastien ;

McCormack, Chris ;

Prince, H. Miles ;

Besnard, Marianne ;

Blanche, Stephane ;

Ekert, Paul G. ;

Fraitag, Sylvie ;

Foulkes, William D. ;

Fischer, Alain ;

Neven, Benedicte ;

Michonneau, David ;

de Saint Basile, Genevieve ;

Jabado, Nada .

NATURE GENETICS, 2018, 50 (12) :1650-+

[5] Use of the JAK Inhibitor Ruxolitinib in the Treatment of Hemophagocytic Lymphohistiocytosis [J].

Keenan, Camille ;

Nichols, Kim E. ;

Albeituni, Sabrin .

FRONTIERS IN IMMUNOLOGY, 2021, 12

[6] Efficacy of ruxolitinib in subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis [J].

Levy, Romain ;

Fusaro, Mathieu ;

Guerin, Frederic ;

Chetouani, Ahmed ;

Moshous, Despina ;

Fischer, Alain ;

de Saint Basile, Genevieve ;

Sepulveda, Fernando E. ;

Neven, Benedicte .

BLOOD ADVANCES, 2020, 4 (07) :1383-1387

[7] Recognition and Regulation of T Cells by NK Cells [J].

Pallmer, Katharina ;

Oxenius, Annette .

FRONTIERS IN IMMUNOLOGY, 2016, 7

[8] A novel immunoregulatory role for NK-cell cytotoxicity in protection from HLH-like immunopathology in mice [J].

Sepulveda, Fernando E. ;

Maschalidi, Sophia ;

Vosshenrich, Christian A. J. ;

Garrigue, Alexandrine ;

Kurowska, Mathieu ;

Menasche, Gael ;

Fischer, Alain ;

Di Santo, James P. ;

de Saint Basile, Genevieve .

BLOOD, 2015, 125 (09) :1427-1434

[9]

Sin Jonathan H, 2019, Hematol Oncol Stem Cell Ther, V12, P166, DOI 10.1016/j.hemonc.2017.07.002

[10] Negative regulation of Nod-like receptor protein 3 inflammasome activation by T cell Ig mucin-3 protects against peritonitis [J].

Wang, Wei ;

Shi, Qingzhu ;

Dou, Shuaijie ;

Li, Ge ;

Shi, Xinhui ;

Jiang, Xingwei ;

Wang, Zhiding ;

Yu, Dandan ;

Chen, Guojiang ;

Wang, Renxi ;

Xiao, He ;

Hou, Chunmei ;

Feng, Jiannan ;

Shen, Beifen ;

Ma, Yuanfang ;

Han, Gencheng .

IMMUNOLOGY, 2018, 153 (01) :71-83

← 1 2 →