To assess the prevalence of hemostasis abnormalities at the time of fetal death diagnosis. This retrospective single-center study included all fetal deaths between 22 and 42 weeks from July 2017 to December 2023, excluding multiple pregnancies, patients on anticoagulant therapy, and those with known pre-existing coagulation disorders. Hemostatic abnormalities were classified as: (1) probable disseminated intravascular coagulation (DIC), defined by a modified International Society on Thrombosis and Hemostasis (ISTH) probability score above 25, and (2) confirmed DIC, requiring clinical diagnosis with transfusion, intensive care admission, or maternal death. To evaluate this score and hemostasis abnormalities, platelet count, prothrombin time difference (PT), activated partial thromboplastin time (aPTT) and fibrinogen level are described. Maternal characteristics and obstetrical history were evaluated to identify a high-risk of DIC situation such as sepsis, placenta abruption and hypertensive disorders of pregnancy. Etiology of fetal death are also described, searching for a vascular etiology. Cases of confirmed DIC are detailed. Among 163 cases of fetal death, 12 women (7.4%) had probable DIC, and three women (1.8%) had confirmed DIC. When the initial blood test was normal, none of the subsequent blood tests revealed any abnormalities. The main causes of fetal death included vascular-related intrauterine growth restriction (42%), unexplained deaths (19%) and fetal malformations (14%). Two cases of confirmed DIC were associated with retroplacental hematomas, while the third involved severe intrauterine growth restriction (IUGR) with pathologic uterine Dopplers, suggesting a vascular etiology. The risk of confirmed DIC in the context of fetal death is low (1.8%) and primarily associated with retroplacental or vascular pathologies. These findings support a selective approach to hemostasis testing based on clinical context rather than routine systematic testing.

We report the first case of a fatal rupture of a flow-related iliac artery aneurysm (IAA) associated with an enormous arteriovenous malformation (AVM). A 47-year-old male presented with an extensive AVM involving the abdominal wall and left thigh, alongside a left IAA measuring 4.16 cm in diameter. Despite multiple AVM-targeted treatments and close IAA monitoring, the aneurysm ruptured after one year of follow-up, causing hemorrhagic shock. Emergency endovascular repair was performed, but the patient succumbed to uncorrected disseminated intravascular coagulation post-procedure. This outcome suggests the need for a more proactive management strategy for flow-related aneurysms in AVM patients.

This paper discusses the diagnostic findings and treatment options for DIC associated with vascular abnormalities based on the "Clinical practice guidelines for management of disseminated intravascular coagulation (DIC) in Japan 2024" released in early 2025. The guidelines define vascular abnormalities as aortic aneurysm, aortic dissection, vasculitis syndromes, and vascular malformations. DIC with enhanced fibrinolysis is a type of DIC often observed in association with vascular abnormalities. Key diagnostic tests for DIC include coagulation activation markers such as thrombin-antithrombin complexes and fibrinolysis activation markers such as plasmin-α2 plasmin inhibitor complex. Treatment consists of addressing the underlying disease, anticoagulant therapy, and combination therapy with anticoagulants and antifibrinolytics, or potentially observation, depending on bleeding symptoms and test results. Use of Factor XIII concentrates may also be considered. Treatment selection should consider the patient's condition and the treatment duration, with ongoing adjustments based on continuous testing. A well-established testing system and collaboration with experts are crucial in the treatment of DIC associated with vascular abnormalities.

This study discusses disseminated intravascular coagulation (DIC) associated with solid cancers and various vascular abnormalities, both of which generally exhibit chronic DIC patterns. Solid cancers are among the most significant underlying diseases that induce DIC. However, the severity, bleeding tendency, and progression of DIC vary considerably depending on the type and stage of the cancer, making generalization difficult. Moreover, during the process of creating these guidelines, it became apparent that despite solid cancers being a major underlying condition for DIC, there is a lack of high-quality research on DIC associated with solid cancers. Nevertheless, we developed recommendations for clinical questions (CQs) regarding the use of heparin and recombinant thrombomodulin. Additionally, statements concerning these five questions were provided. DIC associated with various vascular abnormalities, is characterized by hyperfibrinolytic activity and linked to underlying conditions such as aortic aneurysm, aortic dissection, vasculitis syndromes, and vascular malformations. These conditions must always be considered differential diagnoses when unexplained thrombocytopenia or bleeding tendencies are observed. Although no evidence was found to support the assignment of recommendation levels, three statements were made. However, traumatic vascular abnormalities have not been discussed in this context.

The alpha 1 and 2 chains of type IV collagen, encoded by the COL4A1 (MIM 120130) and COL4A2 (MIM 120090) respectively, play essential roles in the vascular basement membranes. Pathogenic variants in COL4A1/ COL4A2 are associated with autosomal dominant cerebral angiopathies. The clinical manifestations of COL4A1/COL4A2-related disorders include: aneurysms, intracerebral hemorrhage, polymicrogyria, porencephaly, heterotopia, periventricular leukomalacia, epilepsy, and neurodevelopmental disorders. COL4A1 pathogenic variants that are in exons 24 and 25 have been associated with hereditary angiopathy, nephropathy, aneurysms, and cramps. The multisystemic phenotypes of COL4A1/COL4A2-related disorders are increasingly being studied. Animal models have suggested that COL4A2-related disorders may also manifest with a variable combination of multisystemic abnormalities affecting the eyes, muscles, and kidneys. Okano and colleagues recently reported a case of recurrent episodes of rhabdomyolysis in a 2-year-old with COL4A1-related disorder raising fundamental questions on mechanisms of COL4A1/COL4A2 variants in muscle homeostasis. To date, rhabdomyolysis has not been associated with COL4A2-related disorder in humans. Rhabdomyolysis is a medical emergency, where there is elevated creatine kinase (CK) level in the blood and increased excretion of myoglobin in urine, due to skeletal muscle damage and release of intracytoplasmic proteins into systemic circulation. Rhabdomyolysis is a serious medical condition. It require intensive care management due to an increased risk of some life-threatening complications [including disseminated intravascular coagulation, renal failure, and severe hyperkalemia]. Herein, we report a case of rhabdomyolysis in an adult with COL4A2-related structural brain malformations (including polymicrogyria and heterotopia).