BACKGROUND: Hepatosplenic γ δ T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma (PTCL) with aggressive clinical behavior. To date, no standard therapy for HSTCL has been established. This study analyzed the clinical features, treatment, and prognosis for patients with HSTCL to determine the best therapeutic approach. METHODS: We reviewed the clinical characteristics, treatments, and responses to treatment of patients in our center between January 2001 and June 2021, and also reviewed related literature. RESULTS: Median patient age was 38 years (range 16-60 years) and the patients included eight males and six females. HSTCL in these patients typically presented with B symptoms (71.4%), splenomegaly (100%), and hepatomegaly (50.0%), but lymphadenopathy was extremely rare. In these patients, routine laboratory testing showed elevated lactate dehydrogenase (71.4%), liver dysfunction (42.9%), and decreased fibrinogen (35.7%). In the induction phase, five of the 14 patients received chemotherapy regimens containing anthracycline (CHOP, or CHOP plus bortezomib or Chidamide), and six were treated with non-CHOP chemotherapy. Seven patients responded to induction treatment, four of whom received allogeneic hematopoietic cell transplantation and then achieved a complete response in the consolidation phase. survival time of patients who received alloHCT range from 10 to 27 months. CONCLUSION: Hepatosplenic γ δ T-cell lacks a standard therapy and is often refractory to conventional chemotherapy regimens. Intensive induction chemotherapy followed by hematopoietic cell transplantation may improve the prognosis of HSTCL.
BACKGROUND: Hepatosplenic γ δ T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma (PTCL) with aggressive clinical behavior. To date, no standard therapy for HSTCL has been established. This study analyzed the clinical features, treatment, and prognosis for patients with HSTCL to determine the best therapeutic approach. METHODS: We reviewed the clinical characteristics, treatments, and responses to treatment of patients in our center between January 2001 and June 2021, and also reviewed related literature. RESULTS: Median patient age was 38 years (range 16-60 years) and the patients included eight males and six females. HSTCL in these patients typically presented with B symptoms (71.4%), splenomegaly (100%), and hepatomegaly (50.0%), but lymphadenopathy was extremely rare. In these patients, routine laboratory testing showed elevated lactate dehydrogenase (71.4%), liver dysfunction (42.9%), and decreased fibrinogen (35.7%). In the induction phase, five of the 14 patients received chemotherapy regimens containing anthracycline (CHOP, or CHOP plus bortezomib or Chidamide), and six were treated with non-CHOP chemotherapy. Seven patients responded to induction treatment, four of whom received allogeneic hematopoietic cell transplantation and then achieved a complete response in the consolidation phase. survival time of patients who received alloHCT range from 10 to 27 months. CONCLUSION: Hepatosplenic γ δ T-cell lacks a standard therapy and is often refractory to conventional chemotherapy regimens. Intensive induction chemotherapy followed by hematopoietic cell transplantation may improve the prognosis of HSTCL.
Authors: Saniya Dhawan; Alun Gordon; Satbeer Singh; Elizabeth Hodges; Susan Davies; Robert Ayto Journal: Int J Hematol Date: 2019-03-19 Impact factor: 2.490
Authors: Francine M Foss; Steven M Horwitz; Monica Civallero; Monica Bellei; Luigi Marcheselli; Won Seog Kim; Maria E Cabrera; Ivan Dlouhy; Arnon Nagler; Ranjana H Advani; Emanuela A Pesce; Young-Hyeh Ko; Silvia Montoto; Carlos Chiattone; Alison Moskowitz; Michele Spina; Marina Cesaretti; Irene Biasoli; Massimo Federico Journal: Am J Hematol Date: 2019-11-25 Impact factor: 10.047
Authors: H C Kluin-Nelemans; M van Marwijk Kooy; P J Lugtenburg; W L J van Putten; M Luten; J Oudejans; G W van Imhoff Journal: Ann Oncol Date: 2011-01-06 Impact factor: 32.976
Authors: G S Falchook; F Vega; N H Dang; F Samaniego; M A Rodriguez; R E Champlin; C Hosing; S Verstovsek; B Pro Journal: Ann Oncol Date: 2009-02-23 Impact factor: 32.976