Intravascular Coagulation Research Articles

Risk Factors for Venous Thromboembolism in Acute Promyelocytic Leukemia

Background: Acute promyelocytic leukemia (APL) is frequently associated with disseminated intravascular coagulation (DIC), leading to potentially life-threatening bleeding. Compared to bleeding, thromboses are a less commonly encountered problem. Objective: The objective of our study was to identify the incidence and predictive value of demographic data, clinical–laboratory parameters, and thrombosis risk assessment models (RAMs) for venous thromboembolism (VTE) in patients with APL. Methods: This study was a retrospective study conducted on adult patients with APL who were treated between 2006 and 2024 at the Clinic of Hematology UCCS with all-trans retinoic acid (ATRA) and anthracycline. The demographic and clinical–laboratory data related to VTE were collected and analyzed alongside the predictive value of two RAMs proposed by Al-Ani and Paterno and colleagues. Results: Among the one-hundred-fifty-five adult patients with APL, VTE was diagnosed in twenty-eight cases (18.1%). The most common location for thrombosis was in the central venous catheter (CVC), which affected twelve (42.8%) patients. A total of six (21.4%) patients had deep vein thrombosis (DVT), one patient (3.6%) showed a pulmonary embolism (PE), and thrombosis at unusual sites was present in nine (32.1%) patients. Our analyses showed that neither Al-Ani’s RAM nor the RAM proposed by Paterno and colleagues were predictive for VTE in patients with APL. The C statistics value for the Al-Ani model was ROC = 0.514, and, for Paterno’s RAM, it was ROC = 0.521. The independent risk factors for VTE, identified via multivariate analysis, were CD114 expression (p = 0.005, OR = 6.4 IC 95%: [1.8–23.2]) and the absence of bleeding at presentation (p = 0.013, OR = 0.086 IC 95%: [0.01–0.59]). Conclusions: To the best of our knowledge, this is the first study showing that a higher expression of CD114 increases the risk of VTE. The absence of bleeding at presentation in patients with APL correlates with thrombosis. Further analyses are needed to confirm these findings and help to develop therapeutic strategies to prevent VTE complications. So far, no risk assessment model has been sufficient to stratify patients with APL according to their risk of VTE.

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. Therefore, we developed diagnostic and management algorithms for DIC based on a systematic literature review. Notably, these guidelines recommend using the JSTH DIC diagnostic criteria (2017 version) or the former Ministry of Health and Welfare DIC diagnostic criteria (1983 version) to diagnose DIC. Furthermore, in the management of DIC, it is essential to treat the underlying disease through transfusion of platelet concentrates and fresh frozen plasma, if necessary. A systematic review of antifibrinolytic and anticoagulant therapies concluded that tranexamic acid therapy is not strongly recommended for patients with APL undergoing treatment with all-trans retinoic acid (Grade 1C). The use of recombinant thrombomodulin is weakly recommended (Grade 2B), whereas the use of other anticoagulants, including heparin and serine protease inhibitors, is weakly not recommended (Grade 2C). Therefore, we hope that these guidelines will help physicians find the best possible solutions in clinical practice.

Fetal death: Expert consensus of the French College of Obstetricians and Gynecologists.

Fetal death is defined as the spontaneous cessation of cardiac activity after 14 weeks gestational age (GA). Regarding prevention of fetal death in the general population, it is not recommended to counsel or prescribe rest, aspirin, vitamin A, vitamin D, or micronutrient supplementation; systematically look for nuchal cord during prenatal screening ultrasound; or perform systematic antepartum monitoring by cardiotocography for the sole purpose of reducing the risk of fetal death. It is recommended to offer vaccination against influenza in epidemic periods and against SARS-CoV-2. Regarding evaluation in the event of fetal death, it is recommended that a fetal autopsy and anatomopathologic examination of the placenta be performed; chromosomal analysis be performed by microarray testing, rather than by conventional karyotype (with postnatal sampling of the fetal placental surface preferred for genetic purposes); testing for antiphospholipid antibodies be performed, with systematic Kleihauer-Betke testing and for irregular agglutinins; and summary consultation to discuss these examination results be offered. Regarding announcement and support, it is recommended that fetal death be announced without ambiguity, using simple words adapted to each situation, after which the couple should be supported with empathy across the different stages of their care. Regarding patient management in cases of fetal death, it is recommended that: in the absence of risks for disseminated intravascular coagulation or maternal demise, the patient's wishes regarding the timing between the fetal death diagnosis and labor induction should be considered; return home is possible, according to the patient's wishes; in all situations except maternal life-threatening emergencies, the preferred mode of delivery is vaginal, regardless of previous cesarean section(s); mifepristone 200 mg be prescribed at least 24 h before induction; and perimedullary analgesia be initiated at the start of induction if requested by the patient, regardless of GA. Of note, there is insufficient evidence to recommend either the administration route (i.e., vaginal or oral) of misoprostol or prostaglandin type. Regarding the risk of recurrence after unexplained fetal death: the incidence does not appear to be increased in subsequent pregnancies; in cases with a history of fetal death due to vascular problems, low-dose aspirin is recommended to reduce perinatal morbidity (otherwise, evidence is insufficient to recommend the prescription of aspirin); no optimal delay in initiating another pregnancy should be recommended based solely on a history of fetal death; fetal heart rate monitoring is not indicated based solely on a history of fetal death; although systematic labor induction is not recommended, induction may be considered depending on the context and parental request, and considering fetal age, benefits, and risks, especially before 39 weeks GA. Note that if the cause of fetal death is identified, management should be adjusted on a case-by-case basis. Regarding fetal death in a twin pregnancy, it is recommended that the surviving twin be examined immediately upon fetal death diagnosis; in a dichorionic twin pregnancy, preterm delivery induction is not recommended; in a monochorionic twin pregnancy, the surviving twin should be immediately evaluated for signs of acute fetal anemia, with weekly ultrasound monitoring for the first month, though immediate labor induction is not recommended.

Tracheal reconstructive surgery under ECMO for the treatment of congenital tracheal stenosis in the premature infant: case report

BackgroundCongenital tracheal stenosis (CTS) is a rare but life-threatening malformation of the trachea. Surgical reconstruction is the treatment of choice in symptomatic cases which is highly risky and is rarely performed in extremely premature infants. With this, reporting a case of CTS managed by tracheal reconstructive surgery under ECMO in a baby weighing 1.47 kg at 32 + 1 WOG was the first ever case in China.Case presentationA premature newborn with a very low birth weight (VLBW) was admitted to our institute for breathing difficulties, requiring mechanical ventilation, and experienced two unsuccessful attempts of extubation. The team performed tracheal reconstructive surgery supported by ECMO after identifying lower tracheal stenosis through a bronchoscopy examination. One month after the surgery, oxygen support was able to discontinue. The patient's entire hospitalization was incredibly challenging, marked by hemodynamic instability with persistent anemia, and disseminated intravascular coagulation (DIC), which were managed with great care. Despite the difficult stay, a follow-up bronchoscopy revealed no obstruction or tracheal stenosis, leading to a successful discharge.ConclusionAdvancements in diagnostic techniques and innovative management methods have made diagnosing and treating CTS easier, even in premature infants. Our case is the first in China to successfully undergo tracheal reconstructive surgery supported by ECMO, inspiring future achievements in the medical field.

Emergence of Invasive Group A Streptococcus Infection in an Infant: A Case Report

Introduction: Group A streptococcus (GAS) manifests as a spectrum of illnesses, ranging from mild to life-threatening. While relatively rare in infants, GAS infections can present with grave consequences. Case Report: An eight-month-old infant was found to have GAS bacteremia complicated by sepsis and disseminated intravascular coagulation, resulting in lower extremity myositis and tissue ischemia. Tissue ischemia progressed to dry gangrene requiring below-knee amputation followed by six weeks of antibiotics. Conclusion: This case serves as a reminder of the critical importance of vigilance, prompt recognition, and aggressive intervention in the management of invasive GAS infections in infants.

Heparin in sepsis: current clinical findings and possible mechanisms

Sepsis is a clinical syndrome resulting from the interaction between coagulation, inflammation, immunity and other systems. Coagulation activation is an initial factor for sepsis to develop into multiple organ dysfunction. Therefore, anticoagulant therapy may be beneficial for sepsis patients. Heparin possesses a variety of biological activities, so it has a broad prospect in sepsis. Previous studies suggested that patients with sepsis-induced disseminated intravascular coagulation and high disease severity might be suitable for anticoagulant therapy. With the development of artificial intelligence (AI), recent studies have shown that patients with severe coagulation activation represent the targeted patients for anticoagulant therapy in sepsis. However, it remains necessary to accurately define the relevant biomarkers indicative of this phenotype and validate their clinical utility by large randomized controlled trials (RCTs). Analyses of data from early small RCTs, subgroup analyses of large RCTs and meta-analyses have collectively suggested that anticoagulant therapy, particularly the use of heparin, may be an effective approach for managing sepsis patients. Concurrently, debate persists regarding the optimal selection of anticoagulants, proper timing, usage and dosage of administration that should be employed to assess treatment efficacy. The primary mechanisms of heparin are acting on heparan sulfate, histones, high mobility group box 1 and heparin-binding protein, which interfere with the regulation of inflammation, vascular permeability, coagulation, endothelial function and other biological activities. However, the underlying pathophysiological processes mediating the potential therapeutic effects of heparin in the context of sepsis remain incompletely understood and warrant additional rigorous investigation to establish the mechanism more conclusively.

Clinical experience in the study of thrombodynamics in pregnant women at risk of developing preeclampsia and during its manifestation

BACKGROUND: Need for early diagnosis of intravascular activation of coagulation in obstetric patients at risk of preeclampsia. AIM: The aim of this study was to assess the diagnostic value of the thrombodynamics test in patients at risk of developing preeclampsia and during its manifestation. MATERIALS AND METHODS: Dynamic thrombophotometry was performed in 56 pregnant women using a domestic “T2 Thrombodynamics Recorder” device (GemaKor Ltd., Russia). The first study group consisted of patients in the second to early third trimester of pregnancy (n = 45), who, due to hypercoagulability, according to the conventional coagulogram, received low-molecular-weight heparin therapy (calcium nadroparin 0.3 ml or enoxaparin sodium 0.4 ml subcutaneously). The thrombodynamics test was performed to evaluate the efficacy of anticoagulant therapy. The second study group involved patients with moderate (n = 11) and severe (n = 8) preeclampsia. RESULTS: The test was effective in monitoring the state of the hemostasis system in pregnant women receiving anticoagulant therapy — despite normal coagulogram test parameters, the increased rate of fibrin clot formation and / or spontaneous clots indicated increased anticoagulant therapy in 37.8% of patients. Among patients with extragenital pathology, the incidence of spontaneous clots, which should not form during normal coagulation, was observed in all pregnant women with chronic arterial hypertension combined with obesity (χ2 = 6.11; p 0.02), which characterizes the tendency to activate intravascular coagulation in such patients. Subsequently, moderate PE developed in 15.6% of pregnant women with predisposing extragenital pathology, there being no cases of severe preeclampsia. A comparison of thrombodynamics test parameters in patients with moderate and severe preeclampsia showed that in severe preeclampsia, there is a tendency to a higher rate of fibrin clot formation and a higher frequency of spontaneous clot formation (χ2 = 12.7; p 0.01). The test demonstrated high sensitivity (81.8%) and specificity (97.4%) of signaling the presence of severe preeclampsia. CONCLUSIONS: Demonstration of a clinical case of HELLP syndrome in a pregnant woman showed that confirmation of consumption thrombocytopenia due to activation of intravascular coagulation using the thrombodynamics test, in contrast to the global thromboelastography test and the conventional coagulogram test, allowed timely initiation of anticoagulant therapy and avoided the development of irreversible secondary microangiopathy.

Successful Management of Disseminated Intravascular Coagulation in Metastatic Castrate-Resistant Prostate Cancer With Lutetium-177: A Case Report and Review of the Literature

Successful Management of Disseminated Intravascular Coagulation in Metastatic Castrate-Resistant Prostate Cancer With Lutetium-177: A Case Report and Review of the Literature

A retrospective analysis of clinical features, management and outcomes in dogs and cats with Eastern Brown Snake envenomation (2016-2022).

Australian Eastern brown snakes (Pseudonaja textilis) can cause venom-induced consumptive coagulopathy (VICC) in envenomated dogs and cats due to toxin-induced consumption of clotting factors. The objective of this study was to describe presenting clinical signs, prevalence of VICC and haemorrhage, VICC resolution timelines and patient outcomes in a population of dogs and cats with Eastern brown snake envenomation (EBSE). Data from dogs and cats presenting with EBSE were retrospectively evaluated. Univariable and multivariable analyses were performed to test predictor variable effects on outcomes. Animals who were euthanased for financial reasons on presentation were excluded from treatment and outcome analysis. Two-hundred and forty dogs and 98 cats were included. On presentation, 66% (159/240) of dogs had lower motor neuropathy (LMN), 31% (74/240) had preparalytic collapse and 30% (72/240) had signs of haemorrhage. In cats, 94% (92/98) had LMN, and only 5% (5/98) had haemorrhage. Ninety-two percent of dogs (209/226) and cats (81/88) were diagnosed with VICC on presentation and median time to normalisation of coagulation tests was 24 hours. Median hospitalisation length was 1.5 days for dogs (lower quartile [LQ]-upper quartile [UQ]: 1.0-3.0) and 2 days for cats (LQ - UQ: 1.0-2.5). Dogs presenting with LMN and no history of preparalytic collapse had significantly longer hospitalisation times (median 2.25 vs. 1.0 days, P-value <0.001; median 2.0 vs. 1.0 days, P-value <0.001 respectively). Odds of survival was lower in dogs with LMN (odds ratio [OR]: 0.23) and in the pooled multivariable analysis of dogs and cats with haemorrhage (OR: 0.39). The administration of antivenom overall was found to confer a survival advantage; however analysis failed to show increased odds of survival with administration of more than 4000 units. Overall, 89% (187/210) of dogs and 75% (58/77) of cats survived to discharge.

Effect of hypofibrinolysis on clinical outcomes of patients with septic disseminated intravascular coagulation

BackgroundThis study investigated the utility of thromboelastometry (ROTEM) in assessing hypofibrinolysis among septic patients, specifically the association of hypofibrinolysis, as determined by ROTEM, with septic disseminated intravascular coagulation (DIC), organ dysfunction, and clinical outcomes. MethodsThis single-center, retrospective analysis included adult septic patients admitted to Saga University Hospital from 2013 to 2017, with available ROTEM data. Hypofibrinolysis was assessed using the lysis index at 60 min (LI60) in extrinsic thromboelastometry (EXTEM). Based on their LI60 values, patients were classified into three groups: Hyper (LI60 ≤ 85), Normal (LI60 86–96), and Hypo (LI60 ≥ 97). ResultsAmong the 63 cases analyzed, the Hypo group showed significantly higher APACHEII and SOFA scores than the Normal group, indicating greater disease severity. Similarly, DIC and sepsis-induced coagulopathy (SIC) scores were notably higher in the Hypo group. The diagnostic performance of LI60 for ISTH-overt DIC showed an area under the curve (AUC) of 0.954, with an optimal cutoff value of 97 %, achieving 100 % sensitivity and 83.3 % specificity. The odds ratio for ISTH-overt DIC was 2.894, indicating a strong association between elevated LI60 and occurrence of DIC. Hypofibrinolysis predicted 28-day mortality and high SOFA scores (≥ 10) with high specificity and negative predictive value (NPV). A Kaplan-Meier curve revealed that the Hypo Group showed significantly worse clinical outcomes than the Normal and Hyper groups. ConclusionFor septic patients, fibrinolysis suppression presenting as “hypofibrinolysis” (elevated LI60) is associated with poor prognosis and risk of higher organ dysfunction. Moreover, it is a significant predictor of adverse clinical outcomes in sepsis.

Disseminated intravascular coagulation and cirrhotic coagulopathy: overlap and differences. The current state of knowledge. Communication from the SSC of the ISTH

Disseminated intravascular coagulation and cirrhotic coagulopathy: overlap and differences. The current state of knowledge. Communication from the SSC of the ISTH

Platelet dysfunction in heatstroke-induced coagulopathy: A retrospective observational study

PurposeSevere heatstroke is often complicated by disseminated intravascular coagulation (DIC). The aim of this study was to evaluate platelet function assessed through thromboelastography (TEG) at the emergency department (ED) and DIC severity among patients with heatstroke. Materials and methodsWe performed a retrospective single-tertiary hospital cohort study. Patients admitted to an intensive care unit (ICU) with heatstroke were enrolled. Platelet function was evaluated as the difference between citrated functional fibrinogen-maximum amplitude (CFF-MA) and citrated rapid TEG-maximum amplitude (CRT-MA) at the ED. DIC was defined as a score ≥ 4 points based on the Japanese Association of Acute Medicine score. ResultsA total of 31 patients with heatstroke were enrolled. The median platelet count was 18.1 × 104/μL, (interquartile range [IQR]: 12.6–25.0 × 104/μL), and the median platelet function evaluated as CFF-MA-CRT-MA was 34.4 mm (IQR: 27.8–37.8 mm). Among several clinical and TEG-related parameters, the platelet function was strongly correlated with the DIC score (R = −0.63, p < 0.001) and significantly associated with DIC development (area under the receiver operating characteristic curve 0.87 [95 % confidence interval: 0.72–0.99]). ConclusionsThese results suggested that platelet dysfunction occurs in heatstroke-induced DIC, indicating that platelet function evaluation by TEG can provide complementary information and enhance our understanding about the subtypes of heatstroke.

Association of endotheliopathy with coagulofibrinolytic reactions and disseminated intravascular coagulation after trauma: a retrospective observational study

We carried out a retrospective observational investigation to explore the association of endotheliopathy with coagulofibrinolytic reactions and the progression of disseminated intravascular coagulation (DIC) in adult trauma patients. We measured syndecan-1 (SDC-1), an indicator of endotheliopathy, and biomarkers of coagulofibrinolysis in 100 trauma patients immediately transferred to Ehime University Hospital. We evaluated the correlations between the coagulofibrinolytic parameters and SDC-1. We also investigated the association between SDC-1 elevations and the development of DIC, and determined the discriminators of DIC development. The median SDC-1 concentration was 82.7 (43.5–178.1) ng/mL. DIC developed in 16 patients (16.0%), and SDC-1 concentrations were significantly higher in DIC patients than in non-DIC patients (218.8 [134.5–798.2] ng/mL vs. 67.2 [39.6–114.5] ng/mL, p < 0.001). Receiver operating characteristic curve analysis revealed that the circulating SDC-1 level effectively predicted the progression of DIC, with an area under the curve of 0.862 (95% confidence interval [CI], 0.789–0.936). The optimal cut-off value was determined to be 92.5 ng/mL, yielding a sensitivity of 100.0% and a specificity of 67.8% (p < 0.001). A simple logistic regression analysis showed that a circulating SDC-1 concentration of > 92.5 ng/mL was significantly correlated with DIC progression (odds ratio [OR], 31.67; 95%CI: 3.97–252.31, p = 0.001). Many coagulofibrinolytic parameters were significantly correlated with SDC-1. Estimating the discriminators of DIC development by the least absolute shrinkage and selection operator (LASSO) and elastic-net regression analysis identified markers of coagulofibrinolytic activation, such as thrombin-antithrombin complex (TAT) and tissue plasminogen activator (tPA). A multivariate logistic regression model using TAT, tPA, and SDC-1 demonstrated that TAT and tPA, but not SDC-1, were independent factors predicting the development of DIC (TAT per 10 µg/L: OR, 1.14, 95%CI: 1.05–1.24, p = 0.003; tPA per 100pg/mL: OR, 1.03, 95%CI: 1.01–1.05, p = 0.003; SDC-1 per 10ng/mL: OR, 1.00, 95%CI: 0.99–1.01, p = 0.973). Mediation analysis showed that SDC-1 elevation was predominantly associated with the development of DIC indirectly through the increase in TAT (proportion mediated = 96.1%, p < 0.001), while there was no significant indirect effect of SDC-1 elevation on the role of TAT elevation in DIC development was observed (p = 0.340). The primary pathogenesis of DIC in the acute phase of trauma is likely driven by coagulofibrinolytic activation. Endotheliopathy, as reflected by elevated circulating levels of SDC-1, is strongly associated with coagulofibrinolytic responses. Although endotheliopathy may contribute to the early development of DIC through coagulation activation, its role appears to be limited.

Blue rubber blister nevus syndrome: A case report

BACKGROUND Blue rubber blister nevus syndrome (BRBNS) is a congenital, rare disease characterized by venous malformations of the skin and internal organs, affecting all systems throughout the body. The pathogenesis is unknown. There is no consensus on the treatment of BRBNS. Most of the previously reported cases were mild to moderate with a good prognosis, and this case was a critically ill patient with severe gastrointestinal hemorrhage, disseminated intravascular coagulation (DIC), and severe joint fusion that was different from previously reported cases. CASE SUMMARY An 18-year-old man with early onset of BRBNS in early childhood is reported. He presented with recurrent melena and underwent malformed phlebectomy and partial jejunectomy and ileal resection. The patient had melena before and after surgery. After active treatment, the patient's gastrointestinal bleeding improved. This was a case of atypical BRBNS with severe gastrointestinal bleeding and severe joint fusion, which should be differentiated from other serious joint lesions and provide clinicians with better understanding of this rare disease. CONCLUSION This case of critical BRBNS with gastrointestinal hemorrhage, DIC and severe joint fusion provides further understanding of this rare disease.

THE ETIOLOGICAL PROFILE AND ALTERATIONS IN CEREBROSPINAL FLUID CHARACTERISTICS IN PATIENTS WITH BACTERIAL MENINGITIS

Currently, bacterial meningitis (BM) remains a form of life-threatening infectious pathology, with a mortality rate ranging from 4% to 35% and poor prognosis often accompanied by diverse specific and non-specific complications, including infectious toxic shock, cerebral edema, ventricular inflammation, hydrocephalus, acute cerebrovascular accidents, venous sinus thrombosis, disseminated intravascular coagulation (DIC) syndrome, respiratory distress syndrome (RDS) syndrome, inappropriate secretion of antidiuretic hormone, and systemic inflammatory response syndrome. In clinical practice, the early identification of the causative microorganism and the implementation of proper antibiotic treatment, as well as a comprehensive assessment of the patient's status and management, are crucial for minimizing a risk of related severe complications and mortality. The BM etiology is characterized by a highly variable nature, contingent upon a multitude of factors such as geographic location, temporal context, patient age, immunological status, and other variables. Over the past two to three decades, advancements in conjugate vaccines and the use implementation of antibiotic treatment have brought about alterations in the spectrum of pathogens responsible for meningitis. The objective of this study was to delve into the etiological profile and prognostic implications of cerebrospinal fluid (CSF) parameters among patients with diagnosed meningitis admitted to the Neuroinfectious Diseases Division of the Infectious Clinical Hospital №2 (Moscow) between 2022 and 2023. A retrospective analysis of medical records of meningitis patients admitted to the Infectious Clinical Hospital №2 during 2022–2023 was conducted. The data were analyzed using the k-means clustering algorithm implemented in the Loginom analytics software to create a scenario, as well as OLAP cubes. The study involved the analysis of medical records for 110 patients aged 18-89 years old. Among the BM pathogens identified, Neisseria meningitidis emerged as the most prevalent in this patient cohort, accounting for 68.2% of cases. Within the group of meningococcal meningitis cases, serovariants A and W135 were the most frequently detected, accounting for 49.3% and 33.3%, respectively. Significant alterations in cerebrospinal fluid parameters, characterized by elevated cell counts and lowered glucose levels, were observed among patients with purulent meningitis resulting from serogroup A Neisseria meningitidis infections. A combination of elevated baseline cell level along with high concentrations of proteins, and lactate, coupled with the presence of D-dimer in cerebrospinal fluid, has been associated with a severe disease progression and the emergence of critical and life-threatening sequelae.

Hemostasis and thrombosis risks and management in vascular anomalies.

The role of the hematologist in the management of vascular anomalies is evolving. Several vascular tumors and malformations are associated with complex coagulation derangements. Kaposiform hemangioendothelioma or tufted angiomas may present with a consumptive coagulopathy known as the Kasabach-Merritt phenomenon (KMP). The management of KMP is essential to reduce morbidity and mortality from this condition. Slow-flow vascular malformations (SFVM) are also frequently complicated by a coagulopathy requiring anticoagulation, especially during and after surgical procedures, and some of these conditions pose a high risk of venous thromboembolism. Pain in SFVM is also frequently responsive to anticoagulation as well. It is essential for a hematologist with expertise in vascular anomalies to assist in the management of these complex conditions as part of a multidisciplinary team to reduce morbidity and mortality. Through case-based discussions, we attempt to highlight the critical role of the hematologist in managing these anomalies.

Congenital Infantile Fibrosarcoma - A Potentially Fatal Condition.

Congenital infantile fibrosarcoma (CIF) is a rare prenatal malignant tumour that occurs in infants and can be difficult to distinguish from vascular malformation or haemangiomas. Early diagnosis and treatment are crucial for saving both limb and life. We report a 4-month-old infant with CIF of the left forearm and arm which presented with a bleeding ulcer over the swelling. During admission, the child developed disseminated intravascular coagulation (DIC) necessitating a life-saving above-elbow amputation. Level of Evidence: Level V (Therapeutic).

Machine learning models can predict cancer-associated disseminated intravascular coagulation in critically ill colorectal cancer patients.

Due to its complex pathogenesis, the assessment of cancer-associated disseminated intravascular coagulation (DIC) is challenging. We aimed to develop a machine learning (ML) model to predict overt DIC in critically ill colorectal cancer (CRC) patients using clinical features and laboratory indicators. This retrospective study enrolled consecutive CRC patients admitted to the intensive care unit from January 2018 to December 2023. Four ML algorithms were used to construct predictive models using 5-fold cross-validation. The models' performance in predicting overt DIC and 30-day mortality was evaluated using the area under the receiver operating characteristic curve (ROC-AUC) and Cox regression analysis. The performance of three established scoring systems, ISTH DIC-2001, ISTH DIC-2018, and JAAM DIC, was also assessed for survival prediction and served as benchmarks for model comparison. A total of 2,766 patients were enrolled, with 699 (25.3%) diagnosed with overt DIC according to ISTH DIC-2001, 1,023 (36.9%) according to ISTH DIC-2018, and 662 (23.9%) according to JAAM DIC. The extreme gradient boosting (XGB) model outperformed others in DIC prediction (ROC-AUC: 0.848; 95% CI: 0.818-0.878; p < 0.01) and mortality prediction (ROC-AUC: 0.708; 95% CI: 0.646-0.768; p < 0.01). The three DIC scores predicted 30-day mortality with ROC-AUCs of 0.658 for ISTH DIC-2001, 0.692 for ISTH DIC-2018, and 0.673 for JAAM DIC. The results indicate that ML models, particularly the XGB model, can serve as effective tools for predicting overt DIC in critically ill CRC patients. This offers a promising approach to improving clinical decision-making in this high-risk group.

Acute Fatty Liver of Pregnancy: A Rare Case Further Complicated with Disseminated Intravascular Coagulation

Introduction: An acute fatty liver of pregnancy is a rare and potentially deadly pregnancy complication. A case of acute fatty liver of pregnancy that is complicated with disseminated intravascular coagulation is hereby presented.Case Report: A 27-years old, 36 weeks pregnant female is presented with jaundice, elevated LDH and liver enzymes, and thrombocytopenia. An emergency obstetric ultrasonography examination found an unremarkable pregnancy without a placental abruption. The patient was managed conservatively and she delivered 6 days after the presentation. The neonate was treated in the neonatal intensive care unit due to a meconium aspiration. The patient suffered from a continued uterine bleeding and she was managed with the Sayeba’s technique. The laboratory work-up showed a disseminated intravascular coagulation with anemia and thrombocytopenia. The patient was treated with a packed red cell and fresh frozen plasma transfusion. The patient required an intensive care treatment. Discussion: An acute fatty liver of pregnancy is a potentially deadly complication of pregnancy and it requires a multidisciplinary, intensive support. The acute fatty liver of pregnancy symptoms are similar to those of a HELLP syndrome. An acute fatty liver of pregnancy requires a prompt delivery followed with a intensive support. Complications of an acute fatty liver of pregnancy include a hepatic failure and a disseminated intravascular coagulation which should be managed accordingly.Conclusion: An acute fatty liver of pregnancy requires a prompt diagnosis and treatment to achieve the best possible maternal outcome.Acute Fatty Liver of Pregnancy: Kasus Jarang Dengan Komplikasi Koagulasi Intravaskular DiseminataAbstrak Pendahuluan: Acute fatty liver of pregnancy adalah komplikasi kehamilan yang langka dan dapat mematikan. Laporan ini memaparkan sebuah kasus acute fatty liver of pregnancy dengan komplikasi koagulasi intravascular diseminata.Laporan Kasus: Seorang perempuan berusia 27 tahun, hamil 36 minggu datang dengan icterus, peningkatan kadar LDH dan enzim hati, serta trombositopenia. Pemeriksaan ultrasonografi obstetric di IGD menemukan kehamilan tanpa komplikasi dan tanpa solusio plasenta. Pasien ditatalaksana secara konservatif dan melahirkan 6 hari pasca-perawatan. Neonatus dirawat di ruang rawat intensif karena aspirasi meconium. Pasien mengalami perdarahan uterus dan ditatalaksana dengan kondom Sayeba. Pemeriksaan laboratorium menunjukkan adanya koagulasi intravaskular diseminata dengan anemia dan trombositopenia. Pasien ditatalaksana dengan transfuse packed red cell dan fresh frozen plasma. Pasien memerlukan perawatan intensif. Pembahasan: Acute fatty liver of pregnancy adalah komplikasi kehamilan yang dapat mematikan dan memerlukan tatalaksana intensif multidisipliner. Acute fatty liver of pregnancy memiliki gejala yang menyerupai sindrom HELLP. Acute fatty liver of pregnancy ditatalaksana dengan melakukan persalinan diikuti dengan terapi intensif. Komplikasi acute fatty liver of pregnancy meliputi gagal hepar dan koagulasi intravascular diseminata yang harus ditatalaksana dengan tepat.Kesimpulan: Acute fatty liver of pregnancy memerlukan diagnosis dan tatalaksana yang cepat untuk meningkatkan luaran maternal.Kata kunci: acute fatty liver of pregnancy, koagulasi intravascular diseminata, komplikasi kehamilan

Handheld Point-of-Care Devices for Snakebite Coagulopathy: A Scoping Review.

Venom-induced consumption coagulopathy (VICC) is a common complication of snakebite that is associated with hypofibrinogenemia, bleeding, disability, and death. In remote tropical settings, where most snakebites occur, the 20-minute whole blood clotting test is used to diagnose VICC. Point-of-care (POC) coagulation devices could provide an accessible means of detecting VICC that is better standardized, quantifiable, and more accurate. In this scoping review, the mechanistic reasons that previously studied POC devices have failed in VICC are considered, and evidence-based recommendations are made to prioritize certain devices for clinical validation studies. Four small studies have evaluated a POC international normalized ratio (INR) device in patients with Australian Elapid, Daboia russelii, and Echis carinatus envenoming. The devices assessed in these studies either relied on a thrombin substrate endpoint, which is known to underestimate INR in patients with hypofibrinogenemia, have been recalled due to poor accuracy, or have since been discontinued. Sixteen commercially available POC devices for measuring INR, activated clotting time, activated partial thromboplastin time, fibrinogen, D-dimer, and fibrin(ogen) degradation products have been reviewed. POC INR devices that detect fibrin clot formation, as well as a novel POC device that quantifies fibrinogen were identified, which show promise for use in patients with VICC. These devices could support more accurate allocation of antivenom, reduce the time to antivenom administration, and provide improved clinical trial outcome measurement instruments. There is an urgent need for these promising POC coagulation devices to be validated in prospective clinical snakebite studies.