Clinical features and treatment outcomes of 14 patients with hepatosplenic γ δ T-cell lymphoma

J Cancer Res Clin Oncol. 2021 Nov;147(11):3441-3445. doi: 10.1007/s00432-021-03587-6. Epub 2021 Apr 15.


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.

Keywords: Hepatosplenic T-cell lymphoma; Intensive therapy; Retrospective analysis; Transplantation.

MeSH terms

  • Adolescent
  • Adult
  • Aminopyridines / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Benzamides / administration & dosage
  • Bortezomib / administration & dosage
  • Cyclophosphamide / administration & dosage
  • Doxorubicin / administration & dosage
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Intraepithelial Lymphocytes / immunology
  • Intraepithelial Lymphocytes / pathology*
  • Liver Neoplasms / drug therapy
  • Liver Neoplasms / immunology
  • Liver Neoplasms / pathology*
  • Liver Neoplasms / therapy*
  • Lymphoma, T-Cell, Peripheral / drug therapy
  • Lymphoma, T-Cell, Peripheral / immunology
  • Lymphoma, T-Cell, Peripheral / pathology*
  • Lymphoma, T-Cell, Peripheral / therapy*
  • Middle Aged
  • Prednisolone / administration & dosage
  • Retrospective Studies
  • Splenic Neoplasms / drug therapy
  • Splenic Neoplasms / immunology
  • Splenic Neoplasms / pathology*
  • Splenic Neoplasms / therapy*
  • Treatment Outcome
  • Vincristine / administration & dosage
  • Young Adult


  • Aminopyridines
  • Benzamides
  • Vincristine
  • Bortezomib
  • Doxorubicin
  • N-(2-amino-5-fluorobenzyl)-4-(N-(pyridine-3-acrylyl)aminomethyl)benzamide
  • Cyclophosphamide
  • Prednisolone

Supplementary concepts

  • VAP-cyclo protocol