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+干细胞白血病患者作为淋巴细胞干细胞白血病的16例患者的免疫相关基因，特别是T细胞Delta链基因，以及九个患有鼻窦恶性淋巴瘤的患者。在T细胞发育中，细胞质CD3EPSILON的表达比表达。这些发现表明ddjdelta是T-Linege干细胞的标记，并表明TCRDELTA基因重排如下。 DD*DDJ*VDDJ.IN 1994年，我们记录了16例CD7+干细胞白血病患者中有5例显示了特定的临床，免疫表型和免疫原性特征。我们报道了该亚组为T-Encursor白血病/Lumphoma。 T-Exursor白血病/淋巴瘤具有以下特征。 1）在表型检查中，爆炸细胞的表型对应于雌性细胞I和II之间的早期T细胞的表型，2）在诊断时的临床特征上，在外周血中没有爆炸细胞，胸腺瘤（4/5例）和淋巴腺瘤（4/5例）；这些患者达到了完全缓解（5/5），但复发（4/5），表明转化为混合型（4/4，T/B/M-2病例，T/M-2病例，T/M-2病例），3）在免疫原型检查中，存在常见的ddelta2（d）Jdelta1。包括干细胞移植在内的更强化的化学疗法应被认为改变了T-促妇人白血病的预后不良。在九名鼻窦恶性淋巴瘤患者中，通常认为这是T细胞谱系的，通常认为8例来自自然杀伤剂（NK）细胞，这些患者来自许多患者（NK），这些患者与Fetal Nk Nk Nk nk nk类型相对应。这一发现表明NK细胞和T细胞都存在共同的前体。所有九名患者的淋巴瘤细胞均具有爱泼斯坦Barr病毒的单克隆整合。