Molecular biological study of lymphocytic stem cell leukemia

我们分析了16例CD 7+干细胞白血病患者的免疫相关基因，特别是T细胞受体（TcR）δ链基因，作为淋巴细胞干细胞白血病，和9例鼻窦恶性淋巴瘤。我们报道了在CD 7+干细胞白血病患者中，DDJ δ重排较T细胞胞浆CD 3 δ表达更早出现。细胞发育这些发现表明DDJdelta是T系干细胞的标志物，并表明TcRdelta基因重排发生如下：DD*DDJ*VDDJ。1994年，我们记录了16例CD 7+干细胞白血病患者中有5例表现出特定的临床、免疫表型和免疫基因型特征。我们将该亚组报告为T-前体白血病/肿瘤。T-前体细胞白血病/淋巴瘤具有以下特点：1）表型检查，原始细胞表型与前胸腺细胞I期和II期之间的早期T细胞表型相对应; 2）诊断时的临床特征，外周血中无原始细胞，胸腺瘤（4/5例）和淋巴结肿大（4/5例）; 5例完全缓解，4例复发，向混合型转化（4/4，T/B/M-2例，T/M-2例）; 3）免疫基因型检查，常见的存在Ddelta 2（D）Jdelta 1。在9例鼻腔鼻窦恶性淋巴瘤中，8例被认为是T细胞系，其中8例是自然杀伤（NK）细胞，大多数患者与胎儿NK细胞型cCD 3相对应。这一发现表明存在NK细胞和T细胞的共同前体。所有9例患者的淋巴瘤细胞均具有Epstein巴尔病毒的单克隆整合。

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.