Freund Ophir, Wand Ori, Hadad Yitzhac, Bergeron Anne, Fried Sabrina, Pomerantz Gidon, Shaffer Avshalom, Cohen-Hagai Keren, Osadchy Alexandra, Paz Dolev, Barel Nevo, Gershman Evgeni, Amit Odelia, Ram Ron, Bar-Shai Amir
The Institute of Pulmonary Medicine, Tel-Aviv Sourasky Medical Center, Tel Aviv, Israel.
Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Respiration. 2025 Jun 30:1-9. doi: 10.1159/000547175.
Hematological conditions, mainly allogeneic hematopoietic cell transplantation (allo-HCT) and chronic lymphocytic leukemia (CLL), have known associations with bronchiectasis. However, data on these unique clinical entities remain limited. We aimed to evaluate the clinical characteristics and outcomes of patients with hematology-related bronchiectasis.
This retrospective study included patients with bronchiectasis following allo-HCT and CLL. Groups were matched and compared to a control group of bronchiectasis from non-hematological conditions (n = 126). Clinical variables, radiologic features, and disease outcomes were analyzed.
Overall, 42 patients with bronchiectasis after allo-HCT (median age 59, 36% female) and 63 patients with bronchiectasis and CLL (median age 72, 40% female) were included. Both groups exhibited worse lung functions compared to the control. They had more isolations of Pseudomonas aeruginosa (PA) compared to the control group (21-31% vs. 9%) and less non-tuberculosis mycobacteria (3-5% vs. 21%). Allo-HCT and CLL patients also showed high rates of diffuse bronchiectasis distribution (38% and 40%), hypogammaglobulinemia (41% and 71%), and 1-year respiratory hospitalizations (50% and 54%). Only 26% of allo-HCT and 35% of CLL patients saw a pulmonologist, and only 14% performed routine airway clearance. Mortality was higher in both groups compared to controls. The key variables associated with mortality were bronchiolitis obliterans syndrome in the allo-HCT group (HR 11.1, 95% CI: 2.9-30.6) and PA isolation in the CLL group (HR 2.96, 95% CI: 1.3-6.8).
Hematology-related BE have distinct clinical and radiologic features with associated morbidity. These findings could help identify at-risk subgroups for early pulmonologist referral.
血液学疾病，主要是异基因造血细胞移植（allo-HCT）和慢性淋巴细胞白血病（CLL），与支气管扩张症存在已知关联。然而，关于这些独特临床实体的数据仍然有限。我们旨在评估血液学相关支气管扩张症患者的临床特征和预后。
这项回顾性研究纳入了allo-HCT和CLL后发生支气管扩张症的患者。将这些组与非血液学疾病所致支气管扩张症的对照组（n = 126）进行匹配和比较。分析临床变量、放射学特征和疾病预后。
总体而言，纳入了42例allo-HCT后发生支气管扩张症的患者（中位年龄59岁，女性占36%）和63例支气管扩张症合并CLL的患者（中位年龄72岁，女性占40%）。与对照组相比，两组的肺功能均较差。与对照组相比，他们分离出铜绿假单胞菌（PA）的比例更高（21%-31%对9%），而非结核分枝杆菌的比例更低（3%-5%对21%）。allo-HCT和CLL患者还表现出弥漫性支气管扩张分布的高发生率（38%和40%）、低丙种球蛋白血症（41%和71%）以及1年呼吸住院率（50%和54%）。allo-HCT患者中只有26%、CLL患者中只有35%看过肺科医生，只有14%进行了常规气道清理。与对照组相比，两组的死亡率均更高。与死亡率相关的关键变量在allo-HCT组是闭塞性细支气管炎综合征（HR 11.1，95%CI：2.9-30.6），在CLL组是PA分离（HR 2.96，95%CI：1.3-6.8）。
血液学相关支气管扩张症具有独特的临床和放射学特征以及相关发病率。这些发现有助于识别有风险的亚组以便早期转诊至肺科医生处。
