Vasil'ev V I, Probatova N A, Tupitsin N N, Varlamova E Iu, Logvinenko O A, Rodionova E B, Kovrigina A M, Kokosadze N V, Panin M G, Gaĭduk I V, Gorbunova T V, Kondrat'eva T T, Sholokhova E N, Simonova M V, Safonova T N, Radenska-Lopovok S G
Ter Arkh. 2006;78(1):45-52.
To develop algorithm of early diagnosis of extranodal lymphoma arising in patients with Sjorgen's disease (SD).
SD diagnosis was made in 457 patients treated in Rheumatology Institute clinic in 1999-2004, 38 (8.3%) females aged 19-82 had lymphoproliferative diseases. MALT-lymphomas were diagnosed in 15 (42.2%) patients. All the patients have undergone morphological, immunomorphological investigations of the salivary glands, postoperative material was analysed in some patients. In addition, the following investigations were made: ultrasonography of the salivary glands, lymph nodes, viscera; scintigraphy; trephine biopsy of the bone marrow; myelograms; CT of the chest, abdomena and brain; tests for monoclonal immunoglobulins in the serum and light chains in urine; biopsy of the parotid gland. Clinical, morphological and immunophenotypical characteristics of MALT-lymphomas were assessed by WHO classification. Lymphoma stages were classified according to Ann Arbor.
Parotid glands were affected with MALT-lymphoma most frequently. Predominant were extranodal lymphomas of the parotid submandibular, minor salivary glands of the lip and lacrimal glands of stage I E-II E. Extranodal lymphoma with nodal lesion of stage IV occurred less frequently. Untreated long existing MALT-lymphomas of the parotid glands may transform into B-large cell lymphomas deteriorating SD prognosis. The presence of long-term (> 12 months) massive enlargement of parotid/submandibular salivary and lacrimal glands, massive infiltration, monoclonal immunoglobulins in blood serum and their light chains in the urine predict development of MALT-lymphoma in SD.
In SD, MALT-lymphomas develop primarily in target organs--salivary and lacrimal glands. SD patients with persistent enlargement of the parotid glands need biopsy for early detection of malignant lymphoproliferation.
开发干燥综合征（SD）患者结外淋巴瘤的早期诊断算法。
1999年至2004年在风湿病研究所诊所接受治疗的457例患者被诊断为SD，其中38例（8.3%）年龄在19至82岁的女性患有淋巴增殖性疾病。15例（42.2%）患者被诊断为黏膜相关淋巴组织淋巴瘤（MALT淋巴瘤）。所有患者均接受了唾液腺的形态学、免疫形态学检查，部分患者对术后材料进行了分析。此外，还进行了以下检查：唾液腺、淋巴结、内脏的超声检查；闪烁显像；骨髓穿刺活检；脊髓造影；胸部、腹部和脑部的CT检查；血清中单克隆免疫球蛋白和尿液中轻链的检测；腮腺活检。根据世界卫生组织分类评估MALT淋巴瘤的临床、形态学和免疫表型特征。淋巴瘤分期根据Ann Arbor分类法进行。
腮腺最常受MALT淋巴瘤影响。主要为I E-II E期的腮腺、下颌下腺、唇部小唾液腺和泪腺的结外淋巴瘤。IV期伴有淋巴结病变的结外淋巴瘤较少见。未经治疗的长期存在的腮腺MALT淋巴瘤可能转变为B大细胞淋巴瘤，恶化SD的预后。腮腺/下颌下唾液腺和泪腺长期（>12个月）大量肿大、大量浸润、血清中单克隆免疫球蛋白及其尿液中的轻链提示SD患者发生MALT淋巴瘤。
在SD中，MALT淋巴瘤主要发生在靶器官——唾液腺和泪腺。腮腺持续肿大的SD患者需要进行活检以早期发现恶性淋巴增殖。
