Ficuccilli F, Iacobini M, Beglieri M R, Papoff P, Mancuso M, Caradonna A, Donato A I, Colarizi P
Istituto di Clinica Pediatrica, Università degli Studi di Roma La Sapienza.
Minerva Pediatr. 1997 Dec;49(12):571-7.
Purpura fulminans is a rare form of disseminated intravascular coagulation characterized by rapidly progressive purpuric lesions, hypotension and, in some cases, fever. In neonates, purpura fulminans usually develops following deficiency of anticoagulant protein C or S, although acquired forms have been described. The management of disseminated intravascular coagulation is still controversial, with some studies finding a positive effect of anticoagulants and others showing no effect or even a detrimental one. Therefore, at present, management is limited to the treatment of underlying disease and replacement of clotting factors. Personal experience is reported on the efficacy of heparin in combination with antithrombin III in the treatment of purpura fulminans in two preterm neonates who did not have inherited deficiency of protein C or S and developed the disease possibly following prolonged labor (36 hours) in the first case, and maternal neoplasia, in the second. Both neonates presented with widespread cyanotic areas rapidly evolving in purpuric lesions and hemorrhagic bullae. Laboratory tests (prolonged prothrombin and partial thromboplastin time, fibrinogen and antithrombin III concentrations below normal ranges, d-dimer highly positive) were consistent with disseminated intravascular coagulation. In both cases anticoagulant treatment with heparin (50 UI/kg in bolus followed by 15 UI/kg/h) and antithrombin III was associated with resolution of disseminated intravascular coagulation and prompt amelioration of the purpuric lesions, without apparent side effects.
暴发性紫癜是一种罕见的弥散性血管内凝血形式,其特征为紫癜性病变迅速进展、低血压,部分病例伴有发热。在新生儿中,暴发性紫癜通常在抗凝蛋白C或S缺乏后发生,不过也有后天性形式的报道。弥散性血管内凝血的治疗仍存在争议,一些研究发现抗凝剂有积极作用,而另一些研究则显示无效甚至有有害作用。因此,目前的治疗仅限于治疗基础疾病和补充凝血因子。本文报告了个人经验,即肝素联合抗凝血酶III治疗两名早产儿暴发性紫癜的疗效。这两名早产儿均无遗传性蛋白C或S缺乏,第一例可能在产程延长(36小时)后发病,第二例与母亲患肿瘤有关。两名新生儿均出现广泛的发绀区,迅速发展为紫癜性病变和出血性大疱。实验室检查(凝血酶原时间和部分凝血活酶时间延长、纤维蛋白原和抗凝血酶III浓度低于正常范围、D-二聚体高度阳性)与弥散性血管内凝血相符。在这两例中,肝素(50 UI/kg静脉推注,随后15 UI/kg/h)联合抗凝血酶III的抗凝治疗均使弥散性血管内凝血得到缓解,紫癜性病变迅速改善,且无明显副作用。