The pathophysiology and current treatments for the subcutaneous panniculitis-like T cell lymphoma: An updated review

被引:6
|
作者
Lin, En-Cheng [1 ]
Liao, Jia-Bin [3 ]
Fang, Yu-Han [1 ]
Hong, Chien-Hui [1 ,2 ]
机构
[1] Kaohsiung Vet Gen Hosp, Dept Dermatol, Kaohsiung, Taiwan
[2] Natl Yang Ming Chiao Tung Univ, Dept Dermatol, Sch Med, Taipei 11221, Taiwan
[3] Kaohsiung Vet Gen Hosp, Dept Pathol & Lab Med, Kaohsiung, Taiwan
关键词
cutaneous lymphoma; hemophagocytic syndrome; subcutaneous panniculitis-like T cell lymphoma; EPSTEIN-BARR-VIRUS; HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; CUTANEOUS LYMPHOMA; PROGNOSTIC-FACTORS; SEZARY-SYNDROME; CYCLOSPORINE-A; CLASSIFICATION; PATHOGENESIS; MECHANISMS; MUTATIONS;
D O I
10.1111/ajco.13787
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cutaneous T cell lymphoma, which is indolent in nature but could claim life if not correctly diagnosed and promptly treated. SPTCL is usually presented clinically as painless subcutaneous and erythematous nodules over the trunk or extremities. Active clinical vigilance for these subcutaneous nodules or panniculitis-like lesions is warranted. A biopsy must be performed in order to make a correct diagnosis. Positron emission tomography scan is utilized for disease staging and treatment follow-up. Due to the rarity of this lymphoma, a standard treatment protocol is not established yet. However, most cases of SPTCL could be treated well under immunosuppressive or polychemotherapeutic drugs except in cases with hemophagocytic syndrome. Hematopoietic stem cell transplantation may be used in refractory or relapse cases. In this review, we presented a case of SPTCL with long-term complete remission. Meanwhile, since most clinical evidences and experiences of SPTCL are based mostly on case reports or small case series, and the understanding of the SPTCL pathophysiology is limited, we reviewed and updated the pathophysiology and treatments of SPTCL.
引用
收藏
页码:27 / 34
页数:8
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