The Studies on the Early Phase of Mlignant Lymphoma in Patients with Sjogren's Syndrome

(1) Ig VL基因单克隆性的研究：来自干燥综合征（SS）患者的f18 /2独特型在SS患者的血清和外周血淋巴细胞表面膜上经常检测到(1)。我们证明这个SF18/2独特型是Vg种系基因的产物，它是VK III亚群的成员(2)。然后，我们研究了SS患者外周血白细胞DNA样本中Vg基因的重排。在87%的SS患者中检测到重排的274kb片段。通过对这些片段进行测序，发现其中一个片段与Vg种系基因具有98.5%的同源性，另一个片段与CDR3区域的8个替代突变具有95.8%的同源性(3)。认为胚系基因Vg重排的高频率是引发SS患者淋巴细胞增生的因素之一。(2) IgVH-CDR3基因单克隆性的研究从50例SS患者的唇组织中制备DNA样本，用合适的引物进行PCR扩增IgVH-CDR3区域。在SS患者的50个唇活检样本中，7个显示单克隆或寡克隆条带(4)。6例SS患者主要唾液腺出现结节。这些结节已手术切除，病理证明是良性的。6份样品中有4份显示单克隆条带。这些条带的亚克隆和测序显示来自不同患者的2个共同的DNA序列。这些序列与恶性淋巴瘤或骨髓瘤患者中报道的基因具有密切的同源性(5)。2例泪腺肿大患者的4份泪腺样本中，3份泪腺单克隆带(6)。这些样本的DNA序列与唾液腺中发现的DNA序列不同。这些数据表明，B细胞单克隆增殖发生在小或大唾液腺和泪腺的慢性炎症部位，并在SS患者中发展为恶性

(1)Studies on the monoclonality of Ig VL genesSF18/2 idiotype derived from a patient with Sjogren's syndrome (SS) was frequently detected in sera and on the surface membrane of the peripheral blood lymphocytes from SS patients (1). We proved that this SF18/2 idiotype was a product of the Vg germline gene which is a member of the VK III subgroup (2). Then, we studied the rearrangement of the Vg gene in DNA samples from the peripheral blood leukocytes from SS patients. The rearranged 274kb fragments were detected in 87% of SS patients. By sequencing these fragments, it was shown that one has the 98.5% homology to the Vg germline gene and another has the 95.8% homology with 8 replacement mutations in the CDR3 region (3). It was considered that the high frequency of the rearrangement of the Vg germline gene is one of the factors to trigger the development of lymphoproliferation in SS patients.(2)Studies on the monoclonality of Ig VH-CDR3 genesThe DNA samples were prepared from the lip biop … More sy specimens of 50 SS patients and the IgVH-CDR3 regions were amplified by seminested PCR using appropriate primers.Among 50 samples of the lip biopsy from SS patients, 7 showed monoclonal or oligoclonal bands (4). Six patients with SS developed nodules in their major salivary glands. These nodules had been resected surgically and were proved to be pathologically benign. Four out of 6 samples showed monoclonal bands. The subcloning and sequencing of these bands showed 2 common DNA sequences from different patients. These sequences had the close homology to those genes reported in patients with malignant lymphoma or myeloma (5). Among 4 samples from lachrymal glands from 2 patients who showed swollen lachrymal glands, 3 showed monoclonal bands (6). The DNA sequences of these samples were different from those found in salivary glands. These data indicate that B cell monoclonal proliferation occurs in the chronic inflammation sites in minor or major salivary glands and lachrymal glands and develops to be malignant in patients with SS. Less