TITLE:
γδ-T Large Granular Lymphocyte Leukemia Associated Hemaphagocytic Syndrome Complicated with Multiple Organ Dysfunction
AUTHORS:
Suigui Wan, Chengcheng Zheng, Yang Lin, Hong Zhao, Li Su, Changqing Xia
KEYWORDS:
γδ-T Large Granular Lymphocyte Leukemia, Hemophagocytic Syndrome, Multiple Organ Failure
JOURNAL NAME:
Case Reports in Clinical Medicine,
Vol.3 No.4,
April
3,
2014
ABSTRACT: 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.