Fauci A S, Haynes B F, Costa J, Katz P, Wolff S M
N Engl J Med. 1982 Jan 14;306(2):68-74. doi: 10.1056/NEJM198201143060203.
Fifteen patients with lymphomatoid granulomatosis were studied prospectively over a 10-year period. Thirteen of the patients received the therapeutic protocol of cyclophosphamide (2 mg per kilogram of body weight per day) and prednisone (1 mg per kilogram on alternate days). Previous reports had indicated that mortality from lymphomatoid granulomatosis was as high as 90 per cent. Of the 13 patients who received the cyclophosphamide and prednisone protocol, seven had complete remissions lasting for 5.2 +/- 0.6 years (mean +/- S.E.M.) Six of the seven with disease in remission have received no therapy for 28.3 +/- 5.7 months. Malignant lymphomas developed in seven of the eight who died, and only two of the eight had therapy for an adequate period. Since virtually all patients who did not have complete remission went on to have malignant lymphoma, early recognition and prompt treatment during the lymphomatoid-granulomatosis phase of disease may not only lead to complete remissions but also percent the development of a lymphoid neoplasm.
在10年期间对15例淋巴瘤样肉芽肿病患者进行了前瞻性研究。其中13例患者接受了环磷酰胺（每天每千克体重2毫克）和泼尼松（隔日每千克体重1毫克）的治疗方案。先前的报告表明，淋巴瘤样肉芽肿病的死亡率高达90%。在接受环磷酰胺和泼尼松方案治疗的13例患者中，7例完全缓解，持续时间为5.2±0.6年（平均值±标准误）。7例病情缓解的患者中有6例在28.3±5.7个月内未接受任何治疗。8例死亡患者中有7例发生了恶性淋巴瘤，8例中只有2例接受了足够疗程的治疗。由于几乎所有未完全缓解的患者都会发展为恶性淋巴瘤，因此在淋巴瘤样肉芽肿病阶段尽早识别并及时治疗，不仅可能导致完全缓解，还可能预防淋巴样肿瘤的发生。
