Munn S E, Olliver L, Broadbent V, Pritchard J
Department of Dermatology, King's College Hospital, London, United Kingdom.
Med Pediatr Oncol. 1999 Apr;32(4):247-9.
Because prostaglandin (PG) E2 has been identified in the bone lesions of Langerhans cell histiocytosis (LCH), we speculated that indomethacin, a potent PG inhibitor, may be useful in patients with symptomatic LCH involving the bony skeleton.
We used indomethacin to treat patients in whom we wanted to avoid steroids or chemotherapy, or in whom these treatments did not provide complete symptom relief. Ten children with bony LCH between 1984 and 1995 were treated; six had single-system bone disease and four had multisystem disease involving the bony skeleton and other organs.
The dose of indomethacin ranged from 1 to 2.5 mg/kg/day (9-200 mg/day) in divided doses and was given for 1-16 weeks (mean, 6 weeks). Eight patients had a complete response to treatment, defined as complete resolution of symptoms for 4 weeks. One patient was withdrawn from treatment because of concern regarding the potential of indomethacin to induce seizures and a second patient, with suppurative skin lesions overlying a lytic skull defect, did not respond.
Indomethacin is a useful therapy for LCH involving the bony skeleton and may have a role as first-line treatment in single-system bone disease. Whether it has a specific role in slowing disease progression or merely acts as an analgesic has not yet been established.
由于在朗格汉斯细胞组织细胞增多症（LCH）的骨病变中已发现前列腺素（PG）E2，我们推测吲哚美辛（一种有效的PG抑制剂）可能对有症状的累及骨骼的LCH患者有用。
我们使用吲哚美辛治疗那些我们想要避免使用类固醇或化疗的患者，或者那些这些治疗不能完全缓解症状的患者。1984年至1995年间，对10例患有骨LCH的儿童进行了治疗；6例患有单系统骨病，4例患有累及骨骼和其他器官的多系统疾病。
吲哚美辛的剂量为1至2.5mg/（kg·天）（9至200mg/天），分剂量给药，给药1至16周（平均6周）。8例患者对治疗有完全反应，定义为症状完全缓解4周。1例患者因担心吲哚美辛诱发癫痫而退出治疗，另1例患者在溶骨性颅骨缺损上方有化脓性皮肤病变，治疗无效。
吲哚美辛是治疗累及骨骼的LCH的一种有效疗法，在单系统骨病中可能作为一线治疗方法。它在减缓疾病进展中是否有特定作用，还是仅仅起到止痛作用，尚未明确。
