摘要
Hemophagocytic syndrome (HPS) usually presents as a complication of T/NK cell lymphoma. However, γδ-T large granular lymphocyte leukemia (LGL) associated HPS was rarely reported. Herein, we reported a case of γδ-T LGL associated HPS. A previously healthy 21-year-old Chinese man was admitted with high fever, severe pancytopenia and liver dysfunction. He developed disseminated intravascular coaggulation (DIC), shock, gastrointestinal bleeding, acute renal failure as well as central nervous system involvement. Bone marrow aspiration showed infiltration of large granular lymphocytes and hemophagocytic histiocytes. Immunophenotyping analysis by four-color flow cytometry showed the leukemic cells with large side scatter (SSC), CD3+, CD2+, CD8+, CD5-, CD7 partly+, CD4-, CD56-, CD57-. Polymerase chain reaction (PCR) amplification of TCRβ and γ chain gene rearrangement confirmed TCRγδ T cell clone. Thus, a diagnosis of γδ-T LGL associated HPS was established. The patient worsened rapidly and died of multiple organ failure.
Hemophagocytic syndrome (HPS) usually presents as a complication of T/NK cell lymphoma. However, γδ-T large granular lymphocyte leukemia (LGL) associated HPS was rarely reported. Herein, we reported a case of γδ-T LGL associated HPS. A previously healthy 21-year-old Chinese man was admitted with high fever, severe pancytopenia and liver dysfunction. He developed disseminated intravascular coaggulation (DIC), shock, gastrointestinal bleeding, acute renal failure as well as central nervous system involvement. Bone marrow aspiration showed infiltration of large granular lymphocytes and hemophagocytic histiocytes. Immunophenotyping analysis by four-color flow cytometry showed the leukemic cells with large side scatter (SSC), CD3+, CD2+, CD8+, CD5-, CD7 partly+, CD4-, CD56-, CD57-. Polymerase chain reaction (PCR) amplification of TCRβ and γ chain gene rearrangement confirmed TCRγδ T cell clone. Thus, a diagnosis of γδ-T LGL associated HPS was established. The patient worsened rapidly and died of multiple organ failure.
