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Molecular biological study of lymphocytic stem cell leukemia

淋巴细胞干细胞白血病的分子生物学研究

基本信息

批准号：
05670937
负责人：
KIMURA Nobuhiro
金额：
$ 0.83万
依托单位：
Fukuoka University
依托单位国家：
日本
项目类别：
Grant-in-Aid for General Scientific Research (C)
财政年份：
1993
资助国家：
日本
起止时间：
1993 至 1994
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-05670937/
关键词：
Ddelta2 (D) Jdelta1 cytoplasmic CD3 CD7+ stem cell leukemia NK cell Epstein Barr virus T-precursor leukemia 分子生物学 CD7陽性白血病遺伝子再構成 T細胞受容体

项目摘要

We analyzed the immunoassociated genes, specially T-cell receptor(TcR)delta chain gene, of sixteen patients with CD7+stem cell leukemia as lymphocytic stem cell leukemia, and of nine patients with sinonasal malignant lymphoma.In 1993, we reported that the DDJdelta rearrangement are frequently shown in the patients with CD7+ stem cell leukemia and occur more earlier stage than that of expression of cytoplasmic CD3epsilon in T-cell development. These findings indicate DDJdelta to be a marker of T-lineage stem cell, and suggest that TcRdelta gene rearrangement occur as following ; DD*DDJ*VDDJ.In 1994, we documented that five out of sixteen patients with CD7+ stem cell leukemia, showed the specific clinical, immunophenotypic and immunogenotypic characteristics. We reported this subgroup as T-precursor leukemia/lumphoma. The T-precursor leukemia/lymphoma has some characteristics as following ; 1) on phenotypic examination, the phenotypes of blast cells correspond to those of early T cells between prothymocyte stage I and II,2) on the clinical feature at diagnosis, without blast cells in peripheral blood, with thymoma (4/5 cases) and lymphoadenopaty (4/5 cases) ; these patients achieved complete remission (5/5) but had relapse (4/5) , indicating transformation to mixed type (4/4, T/B/M-2 cases, T/M-2 cases) , 3) on immunogenotypic examination, the presence of the common Ddelta2 (D) Jdelta1. The more intensive chemotherapy including the stem cell transplantation should be considered to alter the poor prognosis of T-precursor leukemia.In nine patients with sinonasal malignant lymphoma, which is generally considered to be T-cell lineage, 8 cases were suggested to be originated from Natural killer (NK) cell, which in many patients correspond to fetal NK cell type with cCD3. This finding suggests the presence of common precursor to both NK cell and T-cell. The lymphoma cells of all nine patients have the monoclonal integration of Epstein Barr virus.

我们分析了16例CD7+干细胞白血病和9例鼻腔恶性淋巴瘤患者的免疫相关基因，特别是T细胞受体(T-cell Receptor，TCR)Delta链基因。这些发现表明DDJDelta是T细胞系干细胞的标志，并提示TcRDelta基因重排的发生方式如下：1994年，我们报道了16例CD7+干细胞白血病中有5例表现出特殊的临床、免疫表型和免疫表型特征。我们将这个亚群报告为T-前体白血病/瘤。T-前体白血病/淋巴瘤具有以下特点：1)表型上，原始T细胞表型与早期T细胞表型一致，介于前胸腺细胞I~II期；2)临床特征：外周血中无原始细胞，伴胸腺瘤(4/5例)和淋巴腺瘤(4/5例)；这些患者完全缓解(5/5)，但复发(4/5)，提示转化为混合型(4/4，T/B/M-2例，T/M-2例)，3)免疫表型检查，存在共同的Ddelta2(D)Jdelta1。在9例被认为是T细胞谱系的鼻腔恶性淋巴瘤患者中，有8例被认为来源于自然杀伤细胞(NK)细胞，在许多患者中，自然杀伤细胞(NK)与胎儿NK细胞类型cCD3相对应。这一发现提示了NK细胞和T细胞的共同前体的存在。所有9例患者的淋巴瘤细胞均具有爱泼斯坦-巴尔病毒的克隆整合。