A case report of childhood hepatosplenic γδT cell lymphoma and literature review / 中华实用儿科临床杂志

ABSTRACT

Objective To investigate the clinic characteristics of hepatosplenic γδT cell lymphoma (γδ HSTCL) and analyze the important role of morphology,immunology,cytogenetics and molecular(MICM) in the early diagnosis of γδHSTCL.Methods The clinical features of MICM of a rare case of childhood γδHSTCL had been analyzed,and the related literatures were reviewed.Results Bone marrow aspiration showed approximately 0.365 abnormal cells infiltrated.The immune phenotype analysis displayed 0.245 of the nuclear cells were abnormal ones,which possessed CD2/CD3/CD7/CD19/CD3s/cCD3 and TCRγδ expression.The immunohistochemical staining of spleen tissues showed clusters of differentiation (CD) 3/CD45 RO/CD8/Cytotoxic-granule associated protein (TIA-1)/leukocyte common antigen (LCA)/Granzyme B positive,CD4/CD20/T cell receptor (TCR) β/CD79 α/CD30/terminal deoxynucleotidy transferase (TdT) /CD10/ myeloperoxidase (MPO)/anaplasticlymphoma kinase (ALK) negative and 80.00％ of tumor cells displayed Ki-67 positive.The detection of spleen tissues by flow cytometry displayed that 72.26％ of the karyocytes were lymphocytes,with 88.90％ T lymphocytes.And 92.09％ of the T lymphocytes showed Ki-67/CD7dim,TCRγδ/CD2/CD8/CD34 positive,and TCRαβ/CD5/CD4/CD56 negative.And about 59.16％ of these karyocytes were abnormally matured T lymphocytes.The cytogenetic karyotype of bone marrow showed 46,XY.The detection of T cell receptor gene rearrangement showed TCRβ,TCRγ and TCRδ positive.The patient had been given prednisone of 60 mg/m2 when the diagnosis of γδHSTCL affirmed.But his parents discontinued chemotherapy for him and died.Conclusions MICM has shown a significant value in the diagnosis of γδHSTCL,a rare lymphoma in childhood.