Maintenance therapy with dose-adjusted 6-mercaptopurine in 15 cases of idiopathic pulmonary hemosiderosis
10.3760/cma.j.issn.1008-6315.2010.07.032
- VernacularTitle:巯嘌呤剂量个体化治疗儿童特发性肺含铁血黄素沉着症15例
- Author:
Xuequn LUO
;
Zhiyong KE
;
Libin HUANG
;
Xiaoqing GUAN
;
Xiaoli ZHANG
;
Yuan LIN
;
Yingchuan ZHANG
;
Tingting ZHANG
- Publication Type:Journal Article
- Keywords:
Idiopathic pulmonary hemosiderosis;
Childhood;
Diagnosis;
Treatment
- From:
Clinical Medicine of China
2010;26(7):761-764
- CountryChina
- Language:Chinese
-
Abstract:
Objective To review the diagnosis of idiopathic pulmonary hemosiderosis ( IPH),and to evaluate the efficacy of maintenance therapy with dose-adjusted 6-mercaptopurine (6MP) in IPH children. Methods The diagnosis of IPH was confirmed by in-patient examination and at least 1 year follow-up to exclude secondary causes of pulmonary hemorrhage. Fifteen children met the criteria of IPH and were enrolled. The age at diagnosis was 2-13 years ( median 7 years). Prednisone was administered at 2 mg/( kg·d) for 4 weeks in acute phase of the disease followed by taper. 6MP was also started at 60 mg/( m2·d) simultaneously and continued for 3 years. Results The diagnosis was delayed in most children, which was due to the lack of initial classical manifestation of the disease. The time between the onset of symptoms and diagnosis ranged from 2 weeks to 108 months ( median 8 months) . All the patients exhibited response to the initial treatment and prednisone was successfully tapered off. Only 1 of 8 patients with relative leucopenia (3 × 109/L -6 × 109/L) on 6MP maintenance recurred while 5 of 7 others recurred (P < 0. 05) during median 6-year (range 2. 5 - 9. 5 years) follow-up. Of the latter 5 patients who recurred,4 remained recurrence-free after adjusting the dose of 6MP upwards to keep relative leucopenia. Conclusions Diagnostic delayed is still a main problem in pediatric IPH. Most IPH children in our group tolerated maintenance treatment with 6MP and achieved long-term remission, and these suggested growth retardation on long-term steroids therapy could be avoided. Because of interindividual difference in 6MP metabolism, adjusting the dose of 6MP may be necessary for treatment of IPH children and avoid under-treatment or overtreatment in some children,and thus improve the prognosis. White blood count could be a simple and useful indicator to predict clinical response in most IPH children on 6MP.
