Yangmin Zhu1, Qingyan Gao1, Jing Hu1, Xu Liu1, Dongrui Guan1, Fengkui Zhang2. 1. Department of Therapeutic Center of Anemia, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College (CAMS & PUMC), Tianjin, China. 2. Department of Therapeutic Center of Anemia, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College (CAMS & PUMC), Tianjin, China. Electronic address: zhangfengkuixys@163.com.
Abstract
AIM: Large granular lymphocyte leukemia (LGLL) is a rare lymphoproliferative disorder associated with failure of hematopoiesis and autoimmune diseases. This study describes the clinical features and treatment responses of 108 patients with T-cell large granular lymphocyte leukemia (T-LGLL). METHODS: Clinical data were collected from T-LGLL patients treated at an anemia treatment center within the hematology and blood diseases unit of a single hospital from January 2009 to April 2019. RESULTS: The majority of patients (78 %) were symptomatic at the time of presentation. Splenomegaly was observed in 41 % of cases, while hepatomegaly and lymphadenopathy were rare (6 % and 7 %, respectively). Cyclosporine (CsA) monotherapy was used as first-line therapy for 16 patients, with an overall response rate (ORR) of 56 %. CsA in combination with steroids was administered in 83 patients, with an ORR of 48 %. Among patients experiencing relapse or resistance to first-line therapy, 10 received antithymocyte globulin (ATG) therapy, with an ORR of 50 %; an additional 9 patients received a modified regimen of high-dose cyclophosphamide (CTX) therapy, yielding an ORR of 78 %. CONCLUSIONS: This study provides new information regarding the clinical features and therapeutic strategies for T-LGLL, which can be used to improve clinical decision making for T-LGLL patients. The data presented here indicate the CsA is an effective option for the treatment of T-LGLL, while modified regimens of high-dose CTX or ATG are safe and effective choices for patients with CsA refractory disease.
AIM: Large granular lymphocyte leukemia (LGLL) is a rare lymphoproliferative disorder associated with failure of hematopoiesis and autoimmune diseases. This study describes the clinical features and treatment responses of 108 patients with T-cell large granular lymphocyte leukemia (T-LGLL). METHODS: Clinical data were collected from T-LGLL patients treated at an anemia treatment center within the hematology and blood diseases unit of a single hospital from January 2009 to April 2019. RESULTS: The majority of patients (78 %) were symptomatic at the time of presentation. Splenomegaly was observed in 41 % of cases, while hepatomegaly and lymphadenopathy were rare (6 % and 7 %, respectively). Cyclosporine (CsA) monotherapy was used as first-line therapy for 16 patients, with an overall response rate (ORR) of 56 %. CsA in combination with steroids was administered in 83 patients, with an ORR of 48 %. Among patients experiencing relapse or resistance to first-line therapy, 10 received antithymocyte globulin (ATG) therapy, with an ORR of 50 %; an additional 9 patients received a modified regimen of high-dose cyclophosphamide (CTX) therapy, yielding an ORR of 78 %. CONCLUSIONS: This study provides new information regarding the clinical features and therapeutic strategies for T-LGLL, which can be used to improve clinical decision making for T-LGLL patients. The data presented here indicate the CsA is an effective option for the treatment of T-LGLL, while modified regimens of high-dose CTX or ATG are safe and effective choices for patients with CsA refractory disease.
Authors: Noemí Muñoz-García; María Jara-Acevedo; Carolina Caldas; Paloma Bárcena; Antonio López; Noemí Puig; Miguel Alcoceba; Paula Fernández; Neus Villamor; Juan A Flores-Montero; Karoll Gómez; María Angelina Lemes; Jose Carlos Hernández; Iván Álvarez-Twose; Jose Luis Guerra; Marcos González; Alberto Orfao; Julia Almeida Journal: Cancers (Basel) Date: 2020-11-25 Impact factor: 6.639
Authors: Youssef Salama; Fang Zhao; Jennifer L Oliveira; Ji Yuan; Dragan Jevremovic; Ronald S Go; Wei Ding; Sameer A Parikh; Mithun V Shah; Paul J Hampel; Aref Al-Kali; William G Morice; Min Shi Journal: Blood Cancer J Date: 2022-02-22 Impact factor: 9.812