Hepatosplenic γ-δ T-Cell Lymphoma: Who Is on Your Speed Dial?

J Oncol Pract. 2019 Jun;15(6):307-312. doi: 10.1200/JOP.18.00594.

Abstract

Hepatosplenic γ-δ T-cell lymphoma, an exceptionally uncommon subtype of peripheral T-cell lymphomas, commonly presents with advanced-stage disease manifesting with hepatosplenomegaly, cytopenias, and constitutional symptoms. Management of this subset is challenging as a result of the unique presentation and refractory nature to conventional treatment approaches. There is a lack of consensus guidelines for up-front induction strategies, and the role of consolidative autologous or allogeneic stem-cell transplantation is controversial. Prospective studies are lacking, and treatment is often guided by literature on the basis of case series or single-institution studies, lending to expert opinions influencing treatment paradigms.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Cytogenetics
  • Hematopoietic Stem Cell Transplantation / methods
  • Humans
  • Liver Neoplasms / genetics
  • Liver Neoplasms / immunology
  • Liver Neoplasms / pathology*
  • Liver Neoplasms / therapy
  • Lymphoma, T-Cell / genetics
  • Lymphoma, T-Cell / immunology
  • Lymphoma, T-Cell / pathology*
  • Lymphoma, T-Cell / therapy
  • Male
  • Receptors, Antigen, T-Cell, gamma-delta / genetics
  • Receptors, Antigen, T-Cell, gamma-delta / immunology*
  • Splenic Neoplasms / genetics
  • Splenic Neoplasms / immunology
  • Splenic Neoplasms / pathology*
  • Splenic Neoplasms / therapy
  • Treatment Outcome

Substances

  • Receptors, Antigen, T-Cell, gamma-delta