Belen Burcu Fatma, Polat Meltem, Özsevik Sevinç Nursev, Soylu Esma
a Department of Pediatric Hematology, İzmir Katip Celebi University Tepecik Training and Research Hospital , İzmir , Turkey.
b Department of Pediatric Infectious Diseases, Gazi University Medical Faculty , Ankara , Turkey.
Pediatr Hematol Oncol. 2016 Feb;33(1):51-8. doi: 10.3109/08880018.2015.1106627. Epub 2016 Feb 26.
Weekly monitoring of absolute neutrophil count (ANC) under deferiprone therapy in thalassemia patients is recommended to avoid agranulocytosis adverse event. Actually, this recommendation may not be applicable in clinical setting. Our study aimed to establish incidence of neutropenia under deferiprone (DFP) monotherapy when it was monitored bimonthly due to socioeconomic conditions effecting local and refugee thalassemic patients including Syrian origin (SYR; n = 26) and Turkish origin (TR; n = 26) groups. Patients on DFP were followed up for 12 months. Fifteen neutropenic episodes were seen in 5 patients. All 5 patients (4 from SYR group and 1 from TR group) had splenomegaly and hypersplenism, and neutropenia ceased in 4 patients after splenectomy despite continuation of deferiprone. In the TR group, the frequency of patients who have neutropenia (absolute neutrophil count [ANC] <1500/mm(3)) was 3.8% (n = 1) in the 1st month, no patients in TR group had neutropenia until 10th month when again there was 1 patient with mild neutropenia. In SYR group, the frequency of patients who have neutropenia was 3.8% (n = 1), 7.7% (n = 2), and 11.5% (n = 3) in the 1st, 2nd, and 3rd months, respectively, and was found to be 3.8% (n = 1) between 6 and 12 months. Whether or not DFP therapy should be interrupted in case of mild neutropenia and the frequency of monitoring ANC in real-life conditions should be documented with further studies. Other causes of neutropenia in DFP-treated patients should also be kept in mind.
建议在地中海贫血患者接受去铁酮治疗期间每周监测绝对中性粒细胞计数（ANC），以避免粒细胞缺乏症这一不良事件。实际上，这一建议在临床环境中可能并不适用。我们的研究旨在确定在社会经济状况影响当地及难民地中海贫血患者（包括叙利亚裔[SYR；n = 26]和土耳其裔[TR；n = 26]组）的情况下，接受去铁酮（DFP）单药治疗且每两个月监测一次时中性粒细胞减少症的发生率。接受DFP治疗的患者随访12个月。5名患者出现了15次中性粒细胞减少发作。所有5名患者（4名来自SYR组，1名来自TR组）均有脾肿大和脾功能亢进，4名患者脾切除术后中性粒细胞减少症消失，尽管继续使用去铁酮。在TR组中，第1个月中性粒细胞减少（绝对中性粒细胞计数[ANC]<1500/mm³）的患者频率为3.8%（n = 1），直到第10个月TR组均无患者出现中性粒细胞减少，此时再次有1名患者出现轻度中性粒细胞减少。在SYR组中，第1、2和3个月中性粒细胞减少患者的频率分别为3.8%（n = 1）、7.7%（n = 2）和11.5%（n = 3），6至12个月期间为3.8%（n = 1）。对于轻度中性粒细胞减少症是否应中断DFP治疗以及现实条件下监测ANC的频率，应通过进一步研究加以记录。还应牢记DFP治疗患者中性粒细胞减少的其他原因。
