Disseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in <0.1% of Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.

Prothrombin complex concentrates are haemostatic blood products containing four vitamin K-dependent clotting factors (II, VII, IX and X). They are a useful, reliable and fast alternative to fresh frozen plasma for the reversal of the effects of oral anticoagulant treatments (vitamin K antagonists). They are sometimes used for factor II or factor X replacement in patients with congenital or acquired deficiencies. They are widely prescribed in Europe. Several retrospective and prospective studies have demonstrated their efficacy in normalizing coagulation and in helping to control life-threatening bleeding. Few side-effects, mainly thromboembolic events, have been reported. The link between these events and prothrombin complex concentrate infusion has, however, often been brought into question. The use of prothrombin complex concentrates in new promising indications such as the management of massive bleeding requires prospective studies providing a high level of evidence in a high-risk setting.

Objective: To investigate the incidence and clinical significance of deranged clotting results among patients with juvenile (nasopharyngeal) angiofibroma.

Methods: Twenty consecutive patients treated for juvenile angiofibroma between March 1998 and July 2002 in whom preoperative coagulation tests were performed were selected. Results were compared with normal laboratory values, and clinical and histological records were retrospectively analysed.

Results: Four out of 20 patients were found to have abnormal tests, consistent with concurrent consumptive coagulopathies. One patient was found to have a raised D-dimer level. This resolved following removal of the lesion.

Conclusions:The association between larger arterio-venous malformations and disseminated intravascular coagulopathy is well described (Kasabach–Merritt syndrome). Our findings suggest that low-grade consumptive coagulopathies may also complicate smaller juvenile angiofibromas, implying that preoperative coagulation screening tests may have a role in ensuring optimal perioperative haemostasis.

A 41-year-old multigravida presented at 32 weeks of gestation with polyhydramnios and an anencephalic fetus. Abnormal bleeding as a result of disseminated intravascular coagulation complicated an emergency Caesarean section for severe abdominal pain thought to be due to uterine rupture. Massive transfusion with blood products was necessary and the abdomen packed to control bleeding. The patient was transferred to the intensive care unit where she made a slow but complete recovery. Amniotic fluid embolism with atypical presentation of isolated coagulopathy is the likely diagnosis in this case. The case serves to demonstrate that amniotic fluid embolism may present with symptoms and signs other than the classical pattern of dyspnoea, cyanosis and hypotension.