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血管免疫母细胞性T细胞淋巴瘤:77例患者诊断时的临床和实验室特征

Angioimmunoblastic T-cell lymphoma: clinical and laboratory features at diagnosis in 77 patients.

作者信息

Lachenal Florence, Berger Francoise, Ghesquières Hervé, Biron Pierre, Hot Arnaud, Callet-Bauchu Evelyne, Chassagne Catherine, Coiffier Bertrand, Durieu Isabelle, Rousset Hugues, Salles Gilles

机构信息

From Hospices Civils de Lyon, Department of Internal Medicine (FL, ID, HR), Department of Pathology (FB), Department of Cytogenetic and Molecular Biology (ECB), and Department of Hematology (BC, GS), Centre Hospitalier Lyon Sud, Pierre-Bénite; Department of Internal Medicine (AH), Hospital Edouard Herriot, Lyon; Department of Hematology (HG, PB) and Department of Pathology (CC), Centre Léon Bérard, Lyon; and Université Claude Bernard Lyon 1 (FL, FB, AH, ECB, BC, ID, HR, GS), Lyon, France.

出版信息

Medicine (Baltimore). 2007 Sep;86(5):282-292. doi: 10.1097/MD.0b013e3181573059.

Abstract

We retrospectively analyzed 77 patients with pathologically diagnosed angioimmunoblastic T-cell lymphoma from a single city. There were 43 men and 34 women; the median age was 64.5 years (range, 30-91 yr). Average time between first symptoms of the disease and diagnosis was 3.6 months. At diagnosis, peripheral nodes were present in all but 1 patient, and were generalized in 90% of cases. Constitutional symptoms were reported in 77% of cases and spleen enlargement in 51%. A cutaneous eruption--morbilliform, urticarial, or more polymorphic--was present in 45% of patients; in one-third of them, the eruption occurred after drug administration. Other clinical manifestations included pleuritis (22%); arthralgia or arthritis (17%); ear, nose, and throat involvement (14%); central or peripheral neurologic manifestations (10%); and ascites (5%). Most patients presented with advanced disease at diagnosis (bone marrow involvement in 60% of cases). The main laboratory abnormalities were elevated lactate dehydrogenase levels (71%), inflammatory syndrome (67%), hypergammaglobulinemia (50%), anemia (51%), and lymphopenia (52%). Auto- or disimmune manifestations were reported in one-third of patients: autoimmune hemolytic anemia was present at diagnosis in 19% of patients and thrombocytopenic purpura in 7%. Documented vasculitis was described in 12% of cases. Clonality was analyzed in lymph nodes in 47 patients: T-cell and B-cell clones were found in 45 (96%) and 20 (45%) patients, respectively. Chromosomal abnormalities were identified in 62% of cases: trisomies 3, 5, 18, 19, additional X chromosome, and deletion of chromosome 7 were the most common abnormalities. The current study underlines the diversity of presenting manifestations of angioimmunoblastic T-cell lymphoma.

摘要

我们对来自同一个城市的77例经病理诊断为血管免疫母细胞性T细胞淋巴瘤的患者进行了回顾性分析。其中男性43例,女性34例;中位年龄为64.5岁(范围30 - 91岁)。从疾病首发症状到确诊的平均时间为3.6个月。确诊时,除1例患者外其余患者均有外周淋巴结肿大,其中90%为全身淋巴结肿大。77%的病例有全身症状,51%有脾肿大。45%的患者出现皮疹——麻疹样、荨麻疹样或更多样化的皮疹;其中三分之一的患者皮疹在用药后出现。其他临床表现包括胸膜炎(22%);关节痛或关节炎(17%);耳、鼻、喉受累(14%);中枢或周围神经系统表现(10%);以及腹水(5%)。大多数患者在确诊时已处于疾病晚期(60%的病例有骨髓受累)。主要实验室异常包括乳酸脱氢酶水平升高(71%)、炎症综合征(67%)、高球蛋白血症(50%)、贫血(51%)和淋巴细胞减少(52%)。三分之一的患者有自身或免疫失调表现:19%的患者在确诊时有自身免疫性溶血性贫血,7%有血小板减少性紫癜。12%的病例有记录在案的血管炎。对47例患者的淋巴结进行了克隆性分析:分别在45例(96%)和20例(45%)患者中发现了T细胞和B细胞克隆。62%的病例发现染色体异常:3、5、18、19号染色体三体、额外的X染色体以及7号染色体缺失是最常见的异常。本研究强调了血管免疫母细胞性T细胞淋巴瘤临床表现的多样性。

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