Lymphoplasmacytic lymphoma/Waldenström macroglobulinemia with P53 deletion and TCR-delta rearrangement in a case.
10.3760/cma.j.issn.1003-9406.2015.05.014
- VernacularTitle:P53缺失和TCRδ重排克隆阳性的淋巴浆细胞淋巴瘤/华氏巨球蛋白血症一例
- Author:
Xiaofeng XU
1
;
Wei YANG
;
Xuejin ZHANG
Author Information
1. Department of Hematology, Zhejiang Provincial Hospital of Integrated Western and Chinese Medicine, Hangzhou, Zhejiang 310003, P.R. China. Email: zhangxuejin050@sina.com.
- Publication Type:Case Reports
- MeSH:
Aged;
Gene Rearrangement, delta-Chain T-Cell Antigen Receptor;
Genes, p53;
Humans;
In Situ Hybridization, Fluorescence;
Male;
Polymerase Chain Reaction;
Waldenstrom Macroglobulinemia;
drug therapy;
genetics
- From:
Chinese Journal of Medical Genetics
2015;32(5):674-678
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE To study the morphology, immunology, cyto- and molecular genetics of a patient with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM), deletion of P53 gene and rearrangement of clonal T cell receptors-delta (TCR-delta) gene. METHODS The cell morphology and immunocytochemistry were analyzed by bone marrow testing and biopsy. Cellular immunology was analyzed by flow cytometry. Genetic analysis was carried out by chromosome karyotyping, fluorescent in situ hybridization (FISH) and polymerase chain reaction (PCR). Immunoglobulin M (IgM) in serum and urine was assayed by immunofixation electrophoresis. And the effect of chlorambucil therapy was evaluated. RESULTS Bone marrow biopsy suggested that the patient was of B lymphocyte type and had abnormal increase of lymphocytoid plasma cells, which were CD38 and CD138 positive. The patient had a normal male karyotype. FISH and PCR analysis of peripheral blood samples suggested deletion of P53 gene and rearrangement of TCR-delta gene. Immunofixation electrophoresis has detected IgM-kappa in both serum and urine. The patient showed partial response to chlorambucil. CONCLUSION In addition to typical clinical features, bone marrow examination, flow cytometry, histochemistry and immunophenotyping, testing for P53 gene deletion and lymphocyte gene rearrangement can facilitate the diagnosis and treatment of LPL/WM.
