Bilgrami S F, Metersky M L, McNally D, Naqvi B H, Kapur D, Raible D, Bona R D, Edwards R L, Feingold J M, Clive J M, Tutschka P J
University of Connecticut Health Center, Farmington 06030-1315, USA.
Ann Pharmacother. 2001 Feb;35(2):196-201. doi: 10.1345/aph.10071.
To report the outcome as well as the clinical, radiographic, and pathologic features of idiopathic pneumonia syndrome (IPS) following autologous peripheral blood stem cell transplantation (aPBSCT).
A total of 271 patients with a variety of underlying malignancies received busulfan-containing myeloablative chemotherapy prior to aPBSCT; none of these patients received total body irradiation. Ten individuals developed IPS, with a median time of onset of 102 days after stem cell infusion. The major clinical and radiographic findings included an acute or subacute onset of dyspnea, cough, hypoxemia, and bilateral or unilateral infiltrates with or without pleural effusion. Pathologic findings consisted mainly of diffuse interstitial pneumonitis, organizing alveolitis, and cellular atypia. Nine patients diagnosed with IPS were treated with high doses of glucocorticoids parenterally. Despite heroic measures, eight patients died of IPS. The two remaining individuals recovered without experiencing significant long-term pulmonary sequelae.
Chronic low-dose busulfan therapy results in lung injury in 4-6% of patients after several years of treatment and once the cumulative dosage begins to approach 3g. High-dose, short-course busulfan (16 mg/kg)-containing conditioning chemotherapy prior to aPBSCT can also be complicated by IPS. IPS differs from lung damage due to chronic busulfan therapy by its earlier onset, an acute or subacute rather than indolent presentation, characteristic clinical and radiographic features, and lack of multinucleated giant cells on pathologic review. The pathophysiology of IPS secondary to high-dose busulfan-containing myeloablative regimens is not known, but cell-mediated immune reactions and release of cytokines may contribute to the lung injury. Mortality is high (80%) despite the use of heroic measures, including mechanical ventilation. Some patients, however, can respond to high doses of parenteral corticosteroid therapy.
IPS following high-dose, short-course busulfan-containing regimens exhibits unique clinical, radiographic, and pathologic features that differ from lung damage characteristic of chronic, low-dose busulfan therapy. Mortality from this complication is 80%, but some patients survive without long-term pulmonary sequelae following early treatment with glucocorticoids.
报告自体外周血干细胞移植（aPBSCT）后特发性肺炎综合征（IPS）的转归以及临床、影像学和病理学特征。
共有271例患有各种潜在恶性肿瘤的患者在aPBSCT前接受了含白消安的清髓性化疗；这些患者均未接受全身照射。10例患者发生了IPS，中位发病时间为干细胞输注后102天。主要临床和影像学表现包括急性或亚急性起病的呼吸困难、咳嗽、低氧血症以及双侧或单侧浸润影，伴或不伴有胸腔积液。病理表现主要为弥漫性间质性肺炎、机化性肺泡炎和细胞异型性。9例诊断为IPS的患者接受了大剂量糖皮质激素肠外治疗。尽管采取了积极措施，仍有8例患者死于IPS。其余2例患者康复，未出现明显的长期肺部后遗症。
慢性低剂量白消安治疗在数年治疗后且累积剂量开始接近3g时，会导致4%至6%的患者出现肺损伤。aPBSCT前的高剂量、短疗程含白消安（16mg/kg）预处理化疗也可能并发IPS。IPS与慢性白消安治疗所致的肺损伤不同，其起病更早，呈急性或亚急性而非隐匿性表现，具有特征性的临床和影像学特征，且病理检查缺乏多核巨细胞。高剂量含白消安清髓方案继发IPS的病理生理学尚不清楚，但细胞介导的免疫反应和细胞因子释放可能导致肺损伤。尽管采取了包括机械通气在内的积极措施，死亡率仍很高（80%）。然而，一些患者对大剂量肠外糖皮质激素治疗有反应。
高剂量、短疗程含白消安方案后的IPS表现出独特的临床、影像学和病理学特征，不同于慢性低剂量白消安治疗的特征性肺损伤。该并发症的死亡率为80%，但一些患者在早期接受糖皮质激素治疗后存活，无长期肺部后遗症。
