Service de Pneumologie et Réanimation, Centre de Compétence Maladies Rares Pulmonaires, Hôpital Tenon, Paris, France.
Eur Respir J. 2009 Dec;34(6):1408-16. doi: 10.1183/09031936.00039309. Epub 2009 Jun 18.
Mucosa-associated lymphoid tissue-derived (MALT) lymphoma, a low grade B-cell extranodal lymphoma, is the most frequent subset of primary pulmonary lymphoma. Our objective was to evaluate the initial extent of disease and to analyse the characteristics and long-term outcome of these patients. All chest and pathological departments of teaching hospitals in Paris were contacted in order to identify patients with a histological diagnosis of primary pulmonary lymphoma of the MALT subtype. 63 cases were identified. The median age was 60 yrs. 36% of cases had no symptoms at diagnosis. 46% of patients had at least one extrapulmonary location of lymphoma. The estimated 5- and 10-yr overall survival rates were 90% and 72%, respectively. Only two of the nine observed deaths were related to lymphoma. Age and performance status were the only two adverse prognostic factors for survival. Extrapulmonary location of lymphoma was not a prognostic factor for overall survival or for progression-free survival. Treatment with cyclophosphamide or anthracycline was associated with shorter progression-free survival, when compared with chlorambucil. The survival data confirm the indolent nature of pulmonary MALT lymphoma. Better progression-free survival was observed with chlorambucil when compared with cyclophosphamide or anthracycline.
黏膜相关淋巴组织源性(MALT)淋巴瘤,一种低级别的 B 细胞结外淋巴瘤,是原发性肺淋巴瘤中最常见的亚型。我们的目的是评估疾病的初始范围,并分析这些患者的特征和长期预后。联系了巴黎所有教学医院的胸部和病理科,以确定组织学诊断为 MALT 型原发性肺淋巴瘤的患者。共确定了 63 例病例。中位年龄为 60 岁。36%的病例在诊断时无症状。46%的患者至少有一个结外淋巴瘤部位。估计 5 年和 10 年总生存率分别为 90%和 72%。9 例观察到的死亡中仅有 2 例与淋巴瘤有关。年龄和体能状态是生存的唯一两个不良预后因素。结外淋巴瘤部位不是总生存或无进展生存的预后因素。与苯丁酸氮芥相比,环磷酰胺或蒽环类药物治疗与无进展生存时间缩短相关。生存数据证实了肺 MALT 淋巴瘤的惰性性质。与环磷酰胺或蒽环类药物相比,苯丁酸氮芥观察到更好的无进展生存。