Gülen F, Demir E, Tanaç R, Aydinok Y, Gulen H, Yenigün A, Can D
Division of Allergy , Department of Pediatrics, Faculty of Medicine, Ege University, Izmir, Turkey.
Minerva Pediatr. 2006 Dec;58(6):571-4.
Thalassaemia major is a severe chronic hemolytic disease, resulted with iron overload mainly due to regular blood transfusions. Iron overload may lead to serious organ toxicity and even fatal complications, if no iron excretion is achieved by a chelating agent. First introduced in 1976 as s.c. treatment for thalassaemia major, desferrioxamine (DFO) has substantially improved the life expectancy in the disease. While DFO can cause local allergic reactions including redness, itching, pain and lumps, on rare occasion anaphylactic reactions can occur. The mechanism of anaphylaxis like reactions is not well understood. In this case report, we presented a 10 years-old girl with thalassaemia major who had to stop DFO therapy after appearing of systemic allergic reactions with hypotension, tachycardia, pruritus and urticaria against this drug. Serum IgE level was normal, specific IgE and skin prick tests were negative. Intradermal test was resulted with positive reaction to DFO. The patient was hospitalized and desensitization protocol was initiated with rapid s.c. infusions per 15 min. The protocol was stopped at the 17th cycle because of local reaction reappeared. After that, DFO was further diluted and was restarted with lower dosage and longer infusion period. Then, DFO dosage was increased and the dilutions and infusion times were decreased gradually. By this desensitization programme, the patient would continue to use DFO chelation safely for 10 months.
重型地中海贫血是一种严重的慢性溶血性疾病，主要由于定期输血导致铁过载。如果没有通过螯合剂实现铁排泄，铁过载可能导致严重的器官毒性甚至致命并发症。去铁胺（DFO）于1976年首次作为治疗重型地中海贫血的皮下用药，显著提高了该疾病患者的预期寿命。虽然DFO可引起局部过敏反应，包括发红、瘙痒、疼痛和肿块，但罕见情况下会发生过敏反应。过敏样反应的机制尚不清楚。在本病例报告中，我们介绍了一名10岁的重型地中海贫血女孩，她在出现针对该药物的全身性过敏反应，伴有低血压、心动过速、瘙痒和荨麻疹后，不得不停止DFO治疗。血清IgE水平正常，特异性IgE和皮肤点刺试验均为阴性。皮内试验对DFO呈阳性反应。患者住院并开始脱敏方案，每15分钟进行一次快速皮下输注。由于局部反应再次出现，该方案在第17个周期停止。此后，DFO进一步稀释，并以较低剂量和较长输注时间重新开始使用。然后，逐渐增加DFO剂量，并减少稀释度和输注时间。通过这种脱敏方案，患者能够安全地继续使用DFO螯合治疗10个月。
