The 2–5%γδT cells in healthy human peripheral blood recognize nonpeptide antigens,in contrast toαβT cells.1 DistinctγδT-cell malignancies have been identified,with hepatosplenic T-cell lymphoma(γδ-HSTL)being ...The 2–5%γδT cells in healthy human peripheral blood recognize nonpeptide antigens,in contrast toαβT cells.1 DistinctγδT-cell malignancies have been identified,with hepatosplenic T-cell lymphoma(γδ-HSTL)being a rare but aggressive(median survival 13 months)subset.2γδ-HSTL usually presents with hepatosplenomegaly without lymphadenopathy,anemia,and thrombocytopenia.It is histologically characterized by the involvement of the hepatic sinusoids,splenic red pulp and bone marrow interstitium,with or without leukemic presentation in peripheral blood.3 A standard treatment regimen is not well established,but therapy mostly includes various chemotherapeutic agents.Notably,interferon-α(IFN-α)has been proven to be effective in some patients withγδ-HSTL,but other patients require allogeneic or autologous stem cell transplantation.展开更多
基金supported by the Deutsche Forschungsgemeinschaft(DFG)through the Cluster of Excellence“Inflammation-at-Interfaces”EXC306-PN and EXC306-PM(D.K.,O.A.and C.K.)the Werner-und-Klara-Kreitz Foundation(JB).
文摘The 2–5%γδT cells in healthy human peripheral blood recognize nonpeptide antigens,in contrast toαβT cells.1 DistinctγδT-cell malignancies have been identified,with hepatosplenic T-cell lymphoma(γδ-HSTL)being a rare but aggressive(median survival 13 months)subset.2γδ-HSTL usually presents with hepatosplenomegaly without lymphadenopathy,anemia,and thrombocytopenia.It is histologically characterized by the involvement of the hepatic sinusoids,splenic red pulp and bone marrow interstitium,with or without leukemic presentation in peripheral blood.3 A standard treatment regimen is not well established,but therapy mostly includes various chemotherapeutic agents.Notably,interferon-α(IFN-α)has been proven to be effective in some patients withγδ-HSTL,but other patients require allogeneic or autologous stem cell transplantation.
