[Disseminated intravascular coagulation associated with vascular abnormalities].

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.

Decreased factor XIII activity and haptoglobin may be markers of enhanced-fibrinolytic-type disseminated intravascular coagulation status: Case report.

Rationale: Aortic aneurysms are often accompanied by enhanced-fibrinolytic-type disseminated intravascular coagulation (DIC), but markers to adequately assess DIC status remain lacking.

Patient Concerns: An 80-year-old man scheduled for thoracic endovascular aortic repair for aortic aneurysm was diagnosed with enhanced-fibrinolytic-type DIC. The concern was whether surgery could be safely performed in a state of enhanced-fibrinolytic-type DIC with a bleeding tendency.

Transient lupus anticoagulant induced by adenovirus cystitis in a bone marrow transplant recipient.

Adenovirus-associated hemorrhagic cystitis (AdV-HC) is a serious complication of hematopoietic stem cell transplantation (HSCT) that requires hemostatic therapies to control severe hematuria. Here we present the case of a 65-year-old woman with acute myeloid leukemia who successfully underwent HSCT, but developed AdV-HC followed by adenovirus viremia. Marked prolongation of activated partial thromboplastin time (APTT) was observed, along with a decrease in all coagulation factors except prothrombin, raising suspicion of a coagulation factor deficiency.

Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024: significance of guidelines developed for each underlying disease.

Disseminated intravascular coagulation (DIC) is a severe condition characterized by systemic, persistent activation of coagulation in the presence of an underlying disease, leading to the formation of microthrombi in small blood vessels. In DIC, fibrinolysis is also activated alongside coagulation, but the extent of fibrinolysis varies significantly depending on the underlying condition. The classification of DIC types is crucial not only for understanding the pathophysiology involved, but also for selecting appropriate treatment strategies.

Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024: part 4-trauma, burn, obstetrics, acute pancreatitis/liver failure, and others.

Disseminated intravascular coagulation (DIC) is a complex condition with diverse etiologies. While its association with sepsis has been widely studied, less focus has been given to DIC arising from other critical conditions, such as trauma, burns, acute pancreatitis, and obstetric complications. The 2024 Clinical Practice Guidelines, developed by the Japanese Society on Thrombosis and Hemostasis (JSTH), aim to fill this gap and offer comprehensive recommendations for managing DIC across various conditions.

Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024. Part 3: solid cancers and 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.

Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024. Part 1: sepsis.

The Japanese Society on Thrombosis and Hemostasis (JSTH) published the first-ever disseminated intravascular coagulation (DIC) guidelines in 2009. Fifteen years later, the JSTH developed new guidelines covering DIC associated with various underlying conditions. These guidelines were developed in accordance with the GRADE system to determine the strength of the recommendations and certainty of the evidence.

Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024. Part 2: hematologic malignancy.

Disseminated intravascular coagulation (DIC) associated with hematologic malignancies, particularly acute promyelocytic leukemia (APL), is characterized by marked fibrinolytic activation, which leads to severe bleeding complications. Therefore, appropriate diagnosis and management of DIC are crucial for preventing bleeding-related mortality. However, to date, no clinical guidelines have specifically addressed hematologic malignancy-associated DIC.

How We Interpret Thrombosis with Thrombocytopenia Syndrome?

Platelets play an important role in hemostasis, and a low platelet count usually increases the risk of bleeding. Conditions in which thrombosis occurs despite low platelet counts are referred to as thrombosis with thrombocytopenia syndrome, including heparin-induced thrombocytopenia, vaccine-induced immune thrombotic thrombocytopenia, paroxysmal nocturnal hemoglobinuria, antiphospholipid syndrome, thrombotic microangiopathy (TMA), and disseminated intravascular coagulation. TMA includes thrombotic thrombocytopenic purpura, Shiga toxin-producing -associated hemolytic uremic syndrome (HUS), and atypical HUS.

Amyloid deposition through endocytosis in vascular endothelial cells.

No mechanistic lead is known for establishing AL amyloid deposits in organs. We here report an electron microscopic (EM) analysis in a case of intestinal AL amyloidosis before initiating treatment for amyloidosis. The dense deposits of amyloid fibrils are concentrated around the small blood vessels in the submucosal area of intestinal tissue.

Idarucizumab for Emergency Reversal of the Anticoagulant Effects of Dabigatran: Final Results of a Japanese Postmarketing Surveillance Study.

Introduction: Idarucizumab, a monoclonal antibody fragment that rapidly reverses the anticoagulant effects of dabigatran, was approved in Japan in September 2016, at which time an all-case, postmarketing surveillance (PMS) study was initiated to collect data on idarucizumab in Japanese patients. Interim results were published previously, and the final results are reported herein.

Methods: This multicenter, open-label, uncontrolled, non-interventional PMS study was conducted in Japanese patients who received idarucizumab at the approved dose (2 × 2.

Distinguishing immune-mediated thrombotic thrombocytopenic purpura from septic disseminated intravascular coagulation using plasma levels of haptoglobin and factor XIII activity.

Background: Both immune-mediated thrombotic thrombocytopenic purpura (iTTP) and septic disseminated intravascular coagulation (DIC) are life-threatening disorders developed by platelet-consuming microvascular thrombi and necessitate immediate therapeutic interventions. Although severe deficiencies of plasma haptoglobin in iTTP and factor XIII (FXIII) activity in septic DIC have been reported, few studies have focused on the possibility of using these markers to distinguish between iTTP and septic DIC.

Objectives: We investigated whether the plasma levels of haptoglobin and FXIII activity could be helpful for differential diagnosis.

Identification and Prognostication of End-of-Life State Using a Japanese Guideline-Based Diagnostic Method: A Diagnostic Accuracy Study.

Purpose: Prognostic uncertainty can be a barrier to providing palliative care. Accurate prognostic estimation for patients at the end of life is challenging. This study aimed to evaluate the accuracy of end-of-life diagnosis using our unique diagnostic method.

The efficacy and safety of caplacizumab in Japanese patients with immune-mediated thrombotic thrombocytopenic purpura: an open-label phase 2/3 study.

Caplacizumab is an anti-von Willebrand factor humanized single-variable-domain immunoglobulin fragment whose efficacy and safety in immune-mediated thrombotic thrombocytopenia purpura (iTTP) have been demonstrated in international studies. This prospective, open-label phase 2/3 study evaluated caplacizumab 10 mg administered daily during plasma exchange and for 30 days afterward, in combination with immunosuppressive treatment, in Japanese adults with a clinical diagnosis of iTTP (new or recurrent). The primary endpoint was prevention of iTTP recurrence; key secondary endpoints included time to platelet count response, time to organ damage normalization, and safety.

Effect of Anticoagulant/Antifibrinolytic Combination Therapy on Enhanced Fibrinolytic-Type Disseminated Intravascular Coagulation in End-of-Life Stage Solid Tumor Patients.

Thrombotic disorders such as venous thromboembolism and disseminated intravascular coagulation (DIC) are known complications of solid tumors. To date, no reports have described the treatment of enhanced fibrinolytic-type DIC caused by end-of-life stage solid tumors. We encountered three cases of end-of-life stage solid tumors complicated by enhanced fibrinolytic-type DIC with severe bleeding symptoms.

[Improving outcome of COVID-19-associated thrombosis: the need for evaluation of fibrinolytic activation].

Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 and is known to have thrombotic complications. Various-sized thrombosis occurs in the arteries and veins, especially in lung tissue. The prevention and treatment of thrombosis is an important issue that is directly linked to its prognosis.

Coagulopathy and Fibrinolytic Pathophysiology in COVID-19 and SARS-CoV-2 Vaccination.

Coronavirus Disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and is frequently complicated by thrombosis. In some cases of severe COVID-19, fibrinolysis may be markedly enhanced within a few days, resulting in fatal bleeding. In the treatment of COVID-19, attention should be paid to both coagulation activation and fibrinolytic activation.

Therapeutic Strategies for Disseminated Intravascular Coagulation Associated with Aortic Aneurysm.

Aortic aneurysms are sometimes associated with enhanced-fibrinolytic-type disseminated intravascular coagulation (DIC). In enhanced-fibrinolytic-type DIC, both coagulation and fibrinolysis are markedly activated. Typical cases show decreased platelet counts and fibrinogen levels, increased concentrations of fibrin/fibrinogen degradation products (FDP) and D-dimer, and increased FDP/D-dimer ratios.

Recombinant human erythropoietin attenuates hepatic dysfunction by suppressing hepatocellular apoptosis in lipopolysaccharide-induced disseminated intravascular coagulation in rats.

The aim of the present study was to clarify the effect of recombinant human erythropoietin (EPO) and low molecular weight heparin (LMWH) on a rat model of lipopolysaccharide (LPS)-induced disseminated intravascular coagulation (DIC). Experimental DIC was induced by sustained infusion of 5 mg/kg LPS for 4 h. EPO or LMWH was then administered to the LPS-induced DIC model.