Spontaneous Hemorrhage Research Articles

Cat scratch colon and cecal barotrauma perforation during colonoscopy with CO2 insufflation.

Cecal perforation from barotrauma is a rare complication of colonoscopy, even with the use of CO₂ insufflation, which is generally preferred over room air due to its rapid absorption and reduced patient discomfort. A 64-year-old woman with hypertension and dyslipidemia underwent a routine colonoscopy with CO₂ insufflation. Multiple diverticula were noted, and upon reaching the cecum, several parallel linear lesions with spontaneous bleeding, consistent with "cat scratch" colon, were observed. Hemostasis was achieved with 10 endoscopic clips. A subsequent CT scan revealed pneumoperitoneum and the clips in the cecum. The patient was managed conservatively with bowel rest and intravenous antibiotics, showing a favorable clinical course and was discharged on the fifth day. This case highlights the potential for cecal perforation due to barotrauma even with CO₂ insufflation during colonoscopy, emphasizing the need for awareness and prompt management of such complications.

Clinical and Humanistic Burden of Non-inhibitor HaemophiliaA in Five European Countries: Insights from the CHESSII Study.

HaemophiliaA (HA) is a congenital bleeding disorder caused by a deficiency/absence of factorVIII (FVIII) and characterised by frequent, acute and prolonged spontaneous or traumatic bleeding events, often leading to haemophilic arthropathy and progressive joint deterioration. HA severity is characterized by endogenous FVIII activity: mild (> 5-40%), moderate (1-5%), or severe (< 1%). HA poses a substantial clinical and socioeconomic burden on people with HA (PWHA), their caregivers, and society. This analysis evaluates clinical and patient-centric outcomes of a cohort of individuals with non-inhibitor HA sampled from France, Germany, Italy, Spain, and the UK in the 'Cost of Haemophilia in Europe: A Socioeconomic Survey II' (CHESSII) study. CHESSII was a cross-sectional burden-of-illness study collecting clinical and socioeconomic data on adult (≥ 18years) individuals with haemophiliaA or B of any severity with or without inhibitors from eight European countries. Descriptive analyses were conducted examining physician-reported demographics, clinical and health resource utilisation information. PWHA-reported health-related quality of life (HRQoL) using the EQ-5D-5L and Work Productivity and Activity Impairment (WPAI) were also examined. Outcomes were stratified by HA severity and reported at country level. Demographics and clinical characteristics of the cohort (N = 880) were generally consistent across countries. Individuals with severe HA experienced more frequent bleeding events and joint disease despite broad use of factor replacement therapy long-term prophylaxis. A minority of those with mild or moderate HA also experienced such challenges. HRQoL and workforce participation diminished, and chronic pain increased, with increasing HA severity. This analysis provides up-to-date insights on the impact of HA across five European countries. Increasing HA severity was generally associated with worse clinical outcomes, HRQoL and workforce participation. These findings suggest a place for continued evidence-based tailored treatment and clinical management approaches in addressing the residual burden of HA.

Arteriovenous malformation-associated aneurysms in the pediatric population: the University of Pittsburgh Medical Center experience.

Arteriovenous malformations (AVMs) are the most common cause of spontaneous intracranial hemorrhage (ICH) in children, often leading to devastating complications. The current literature from the adult AVM population suggests that both younger age and associated aneurysms carry an increased risk of hemorrhagic presentation. However, detailed analysis of pediatric AVM-associated aneurysms and their link to ICH is relatively unknown, with the literature largely consisting of case reports. This study aimed to determine whether AVM-associated aneurysms predispose pediatric patients to ICH. A retrospective cohort study of 238 pediatric patients with AVMs who presented to the Children's Hospital of Pittsburgh from 1988 to 2023 was performed. Hospital records, patient charts, and radiographic imaging studies were reviewed for patient demographic characteristics, presentation status, and AVM architecture. Of the 238 total patients, 44 (18.5%) children with AVM had associated aneurysms. There were 19 (38.8%) feeding artery aneurysms, 8 (16.3%) intranidal aneurysms, 21 (42.9%) postnidal aneurysms, and 1 (2.0%) unrelated aneurysm of 49 aneurysms. Five patients had venous varices. One hundred forty (58.8%) children presented with hemorrhage. Twenty-one of 44 (47.7%) patients with an AVM-associated aneurysm presented with hemorrhage, whereas 119 of 194 (61.3%) with a solitary AVM presented with hemorrhage (p = 0.10). On multivariate analysis, postnidal aneurysm (OR 0.36, p = 0.037) and an increased number of draining veins (OR 0.66, p = 0.049) were significantly associated with a decreased likelihood of hemorrhagic presentation. Deep venous drainage was associated with an increase in hemorrhagic presentation (OR 2.25, p = 0.0045) on multivariate analysis. Approximately one-fifth of children with AVMs in this study had accompanying aneurysms, and in this patient population, those with postnidal aneurysms and increased number of draining veins had a decreased incidence of hemorrhage on presentation. Feeding artery and intranidal aneurysms were not associated with an elevated risk of hemorrhagic presentation.

A Case of Page Kidney Secondary to Spontaneous Bilateral Retroperitoneal Hemorrhage

A Case of Page Kidney Secondary to Spontaneous Bilateral Retroperitoneal Hemorrhage

Protocol-based treatment of spontaneous hemorrhage from varicose veins prevents recurrence of bleeding

BackgroundSpontaneous hemorrhage from erosion of varicose veins through the skin is a serious and occasionally fatal complication of varicose vein disease. Various treatments, both acute and delayed, have been advocated. Our two-step clinical protocol was designed to prevent further hemorrhage without delay and to provide durable freedom from recurrent bleeding. MethodsAll patients referred to our surgical vein practice with hemorrhage from varicose veins were entered into a prospective registry. On presentation, all patients underwent diagnostic duplex ultrasound for venous reflux. Immediate treatment consisted of ultrasound-guided polidocanol/CO2 foam sclerotherapy of the bleeding varicosity and adjacent veins. Subsequent endovenous ablation of underlying incompetent axial veins, with concurrent microphlebectomy when indicated, was scheduled and performed within 8 weeks. ResultsFifty-nine patients were referred with recent hemorrhage from varicose veins over a period of 4 years. Fifty-six (95%) had an ultrasound-identified tributary varicosity underlying the point of bleeding, and three had a skin-surface erosion only and no ultrasound-identified underlying tributary. Of the 59 study patients, 52 underwent prompt polidocanol/CO2 foam sclerotherapy, targeted to both the underlying tributary (when present) as well as the cutaneous bleeding varicosity. The remaining seven either declined sclerotherapy or were ineligible. Underlying incompetence of axial (great, small, or anterior saphenous) veins was found in 54 patients (92%). Of these patients with truncal vein incompetence, 48 underwent ablation—5 declined and 1 was too frail for any further intervention. There were no recurrent bleeds in the interval between immediate sclerotherapy and scheduled ablation. During a mean follow-up of 2.2 years, four patients (7%) had late, recurrent hemorrhage: one had failed to return for their scheduled ablation, two were on chronic anticoagulation, and one had severe right heart failure. All four were retreated without further recurrence. ConclusionsA two-step protocol of immediate ultrasound-guided foam sclerotherapy, followed within 8 weeks by endovenous ablation of incompetent axial veins and concurrent microphlebectomy, provided rapid and efficient treatment with durable freedom from subsequent hemorrhage.

STUDY OF SERUM SODIUM LEVELS AND ITS CLINICAL SIGNIFICANCE IN DECOMPENSATED CHRONIC LIVER DISEASE PATIENTS ADMITTED IN A TERTIARY CARE CENTRE OF NORTH EASTERN STATE, TRIPURA

Introduction: Chronic Liver Disease is a major source of death and morbidity globally. Hyponatremia is one of the most common electrolyte abnormalities in chronic liver disease patients. Various studies have established a correlation between serum sodium levels and survival in these patients. Hyponatremia is a marker of severity in CLD patients and studies have shown its relationship with MELD score and Child Pugh score. The complications of DCLD and mortality also increases with hyponatremia. The aim of this study was to study the serum sodium levels in patients with DCLD and to establish its significance. Materials And Methods: Data was collected from 100 patients admitted in medical wards. Patients were divided into 3 groups based on serum sodium levels and the relevant parameters analyzed among the groups. OBSERVATIONS: Among 100 patients, 45 had serum sodium levels ≥136 mEq/L, while 32 had serum sodium levels between 131 and 135 mEq/L and 23 patients had serum sodium level ≤130. No patients had serum sodium levels greater than 145. Serum sodium levels were associated strongly with the severity of liver disease as assessed by Child Pugh and MELD scores. Serum sodium ≤130 indicated the existence of Hepatic Encephalopathy, Hepatorenal Syndrome and Spontaneous Bacterial Peritonitis. Patients with serum sodium less than 130 mEq/L had increased frequency of complications than those with ≥136 mEq/L. Patient with serum sodium levels ≤130 had increased mortality. Conclusion: Hyponatremia is more common in DCLD and low serum sodium levels are associated with increased frequency of complications such as hepatic encephalopathy, hepatorenal syndrome, spontaneous bacterial peritonitis and GI bleeding. Lower serum sodium levels were associated with increased MELD and Child Pugh score and mortality indicating the inverse relationship between serum sodium levels and severity of the disease.

Functional evaluation of joint in moderate to severe hemophilia patients treated with on-demand factor replacement: insights from a single hemophilia treatment center in Bangladesh

BackgroundHemophilia patients are prone to spontaneous or post-traumatic bleeding, particularly in joints and various other body sites. The high incidence of joint hemorrhages in hemophilia patients underscores the importance of routine joint assessments in clinical care to monitor the condition and evaluate the efficacy of hemophilia treatments.AimThis study aimed to assess the joint health of hemophilia patients receiving FVIII or FIX on-demand replacement therapy treatment at a tertiary care hospital in Bangladesh.MethodsThis single-center study involved 93 patients diagnosed with moderate (n = 72;77.4%) and severe (n = 21;22.6%) hemophilia. The assessment of joint health was carried out on six key joints—both elbows, knees, and ankles—using the Hemophilia Joint Health Score (HJHS) version 2.1. Clinical and laboratory data were meticulously documented in a structured questionnaire.ResultsAmong the 93 hemophilic patients, the majority had hemophilia A (96.8%). The median HJHS of the patients was 15 (IQR:9.5–20) with the knee joint being mostly affected (88.2%). A significant positive correlation was observed between HJHS and age (r = 0.385, p < 0.001) and total number of joints involved (r = 0.405, p < 0.001). No significant association was found between factor level, education status, and socio-economic status with HJHS score.ConclusionEarly assessment and monitoring of joint health are essential in clinical practice to prevent severe complications in hemophilia patients.

Emicizumab revolution in treating Hemophilia

HemophiliaA (H.A.) has beenanuncommon hereditary X-linked bleeding disorder indicated by clotting factorVIII (FVIII)deficiency, leading to impaired hemostasis. H.A. affects approximately one in 5,000male births in the U.S., often resulting in spontaneous joint bleeding, chronic pain, and an increased risk of cerebral hemorrhage. Prophylactic treatment with FVIII replacement or the bispecific antibody emicizumab has become a crucial strategy to prevent bleeding episodes and long-term tissue damage. Emicizumab, approved by the FDA &amp; EMA in2018, mimics FVIII activity by binding to activated factorIX (FIXa) &amp; factorX (F.X.), facilitating blood clotting regardless of FVIII inhibitors. The development of emicizumab involved extensive bioengineering to optimize its safety, stability, and efficacy, leading to its successful application in clinical trials. These trials demonstrated that emicizumab significantly decreases the annualized bleeding rate in studied cases with or without inhibitors, including severe H.A. cases, with a favorable safety profile and minimal adverse events. Emicizumab's subcutaneous administration, high bioavailability, and predictable pharmacokinetics make it a practical option for H.A. prophylaxis. Despite its benefits, emicizumab can interfere with specific coagulation assays and may develop anti-drug antibodies (ADAs) in rare cases, potentially reducing its efficacy. Ongoing research and post-marketing surveillance continue to assess its long-term safety and effectiveness, making emicizumab a transformative treatment in managing hemophilia A.

Predictors of liver disease outcomes in individuals with hemophilia and HCV infection

AbstractHemophilia is an X-linked congenital bleeding disorder for which factor replacement is life-saving but complicated by the sequelae of chronic hepatitis C virus (HCV) infection acquired decades ago. Although antiviral therapy clears HCV and reduces end-stage liver disease (ESLD), it may not reverse cirrhosis or prevent hepatocellular cancer (HCC). This was a retrospective cohort study of 121 men with hemophilia and HCV infection cared for at the Hemophilia Center of Western Pennsylvania to determine the incidence and predictors of ESLD and HCC. ESLD and HCC predictors were analyzed using Fisher exact test, and HCV-associated outcomes by Kaplan-Meier time-to-event and Cox proportional hazards regression analyses. At a mean 54 years (36-80) duration of HCV, ESLD occurred in 24 (19.8%), 0.365 per 100 person-years (py); and HCC in 7 (5.8%), 0.106 per 100 py. All 46 (38.0%) alive when HCV antiviral therapy became available, received it. Overall, 31 (25.6%) were HIV+. The leading causes of death were ESLD in 11 (32.3%), bleeding in 9 (26.5%), and HCC in 6 (17.6%). Major risk factors for ESLD included platelets <100 × 103/μL (odds ratio [OR], 6.009; P = .012) and HIV infection (OR, 3.883; P = .001). The major predictors of HCC were ESLD (OR, 11.476; P = .003) and platelets <100 000/μL (OR, 6.159; P = .014). No antiviral-treated subject developed ESLD, P = .001. For men with hemophilia, the sequelae of chronic HCV infection were significant. The major risk factors for ESLD were platelets <100 000/μL and HIV infection. Despite antiviral therapy, ESLD is the most significant predictor of HCC, and ESLD is the leading cause of death.

Spontaneous hepatic hemorrhage as presentation of metastasized papillary thyroid carcinoma: a case report

BackgroundSpontaneous hepatic hemorrhage is a rare condition, most commonly diagnosed in patients with hepatocellular carcinoma or hepatic adenomas, and is seldom caused by metastatic disease. In this case report, we present a patient with spontaneous hepatic hemorrhage due to hepatic metastasis of papillary thyroid carcinoma, an exceptionally rare occurrence.Case presentationThe patient was a 77-year-old white male with a history of atrial fibrillation treated with apixaban. He presented at a local hospital with abdominal pain and nausea. A CT scan revealed a hepatic lesion in segment 3 with an adjacent hematoma. He was referred to our tertiary center and treated conservatively. Further evaluation revealed an intrathoracic goiter containing a tumorous process diagnosed as a papillary thyroid carcinoma (PTC), and the patient subsequently underwent thyroidectomy. A biopsy of the hepatic lesion confirmed it as a PTC metastasis. Due to worsening abdominal pain and anorexia, the patient underwent subacute hepatic segmental resection. Postoperatively, he developed iodine-refractory disease with disseminated metastasis and passed away 22 months after the initial admission.ConclusionsTo our knowledge, this is the first recorded case of metastasized papillary thyroid carcinoma presenting with spontaneous hepatic hemorrhage—adding to the list of rare causes for this condition.

Preparation and structural characterization of a sulfated octasaccharide with heparin-like anticoagulant activity

Heparins are sulfated polysaccharides with a heterogeneous mixture derived from animal tissues, subject to supply limitations and the risk of animal virus residues. Patients using heparin also face the risks of spontaneous bleeding and thrombocytopenia. Here we reported an efficient riclinoctaose-based approach for rapid chemical synthesis of a structurally defined heparin-like anticoagulant sulfated octasaccharide (SRO). We used sulfur trioxide-pyridine, sulfur trioxide-trimethylamine, and sulfur trioxide-triethylamine complexes as solvents for one-pot O-sulfation and determined the optimal conditions for synthesizing SRO. Sulfur trioxide-trimethylamine provided reasonable control over the degree of substitution between 1.85 and 1.88, revealing a single molecule with a theoretical molecular weight of 2952.96 g/mol. The structural features of the SRO were carried out by Fourier transform infrared spectroscopy and one- and two- dimensional 1H and 13C NMR analysis, revealing sulfation repeatedly present at the fixed positions of C-6/C-2/C-3 and reducing terminals. The anticoagulant activity of SRO was demonstrated by efficiently blocking coagulation in the blood of mice and human. SRO dose-dependently decreased ferric chloride-induced experimental thrombosis in mice. Like heparin, SRO specifically inhibits coagulation factor Xa, but significantly reduces the risk of bleeding compared to heparin. Therefore, we named it octaparin. These results support that octaparin is expected to replace animal-sourced heparin.

Risk of spontaneous intracerebral hemorrhage associated with NOACs compared with aspirin and warfarin: A long-term single hospital follow-up study

BackgroundNon-vitamin K antagonist oral anticoagulants (NOACs) are currently the mainstay treatment for preventing thrombosis-induced ischemic stroke in patients with atrial fibrillation (AF), deep vein thrombosis (DVT), or previous infarction. However, such management may potentially induce antithrombotic-associated intracranial hemorrhage, leading to significantly adverse clinical outcomes. To investigate the risk of spontaneous intracranial hemorrhage (sICH) in patients under therapeutic anticoagulation. MethodsThis retrospective cohort study used a database established by Kaohsiung Veterans General Hospital to estimate the risk of first onset sICH in patients with AF, DVT or previous stroke who were 18 years old or older, and who had been on at least three months continuous long-term treatment with the oral anticoagulants aspirin, warfarin, or NOACs. In addition, we used propensity-score matching to minimize bias and Cox proportional hazards ratio to compare the risk of sICH among patients prescribed these anticoagulants. ResultsWe analyzed the data of 546 patients (182 aspirin users, 182 warfarin users, and 182 NOAC users). 180 (20 taking aspirin, 74 warfarin, and 86 NOACs) developed new onset sICH before seven years. No new onset cases were found after 7 years. Importantly, those taking NOACs were found to be at a higher risk of early onset hemorrhage (47.80 %) compared to the groups taking aspirin (11.10 %) and warfarin (47.80 %) with a median time-to-occurrence being 2.50, 4.00, and 4.40 years, respectively. ConclusionsThough NOACs prevented ischemic stroke, they were used with a higher risk of early onset spontaneous ICH at our large medical center.

Spontaneous bilateral adrenal haemorrhage: 2 cases occurring in pregnancy

Spontaneous bilateral adrenal haemorrhage: 2 cases occurring in pregnancy

Biomarkers Involved in the Pathogenesis of Hemophilic Arthropathy.

Hemophilia, which is a rare disease, results from congenital deficiencies of coagulation factors VIII and IX, respectively, leading to spontaneous bleeding into joints, resulting in hemophilic arthropathy (HA). HA involves complex processes, including synovial proliferation, angiogenesis, and tissue remodeling. Despite ongoing research, factors contributing to HA progression, especially in adults with severe HA experiencing joint pain, remain unclear. Blood markers, particularly collagen-related ones, have been explored to assess joint health in hemophilia. For example, markers like CTX-I and CTX-II reflect bone and cartilage turnover, respectively. Studies indicate elevated levels of certain markers post-bleeding episodes, suggesting joint health changes. However, longitudinal studies on collagen turnover and basement membrane or endothelial cell markers in relation to joint outcomes, particularly during painful episodes, are scarce. Given the role of the CX3CL1/CX3XR1 axis in arthritis, other studies investigate its involvement in HA. The importance of different inflammatory and bone damage biomarkers should be assessed, alongside articular cartilage and synovial membrane morphology, aiming to enhance understanding of hemophilic arthropathy progression.

Understanding the Importance of Blood-Brain Barrier Alterations in Brain Arteriovenous Malformations and Implications for Treatment: A Dynamic Contrast-Enhanced-MRI-Based Prospective Study.

The major clinical implication of brain arteriovenous malformations (bAVMs) is spontaneous intracranial hemorrhage. There is a growing body of experimental evidence proving that inflammation and blood-brain barrier (BBB) dysfunction are involved in both the clinical course of the disease and the risk of bleeding. However, how bAVM treatment affects perilesional BBB disturbances is yet unclear. We assessed the permeability changes of the BBB using dynamic contrast-enhanced MRI (DCE-MRI) in a series of bAVMs (n = 35), before and at a mean of 5 (±2) days after treatment. A set of cerebral cavernous malformations (CCMs) (n = 16) was used as a control group for the assessment of the surgical-related collateral changes. The extended Tofts pharmacokinetic model was used to extract permeability (Ktrans) values in the lesional, perilesional, and normal brain tissues. In patients with bAVM, the permeability of BBB was higher in the perilesional of bAVM tissue compared with the rest of the brain parenchyma (mean Ktrans 0.145 ± 0.104 vs 0.084 ± 0.035, P = .004). Meanwhile, no significant changes were seen in the perilesional brain of CCM cases (mean Ktrans 0.055 ± 0.056 vs 0.061 ± 0.026, P = .96). A significant decrease in BBB permeability was evident in the perilesional area of bAVM after surgical resection (mean Ktrans 0.145 ± 0.104 vs 0.096 ± 0.059, P = .037). This benefit in BBB permeability reduction after surgery seemed to surpass the relative increase in permeability inherent to the surgical manipulation. In contrast to CCMs, BBB permeability in patients with bAVM is increased in the perilesional parenchyma, as assessed using DCE-MRI. However, bAVM surgical resection seems to reduce BBB permeability in the perilesional tissue. No evidence of the so-called breakthrough phenomenon was detected in our series. DCE-MRI could become a valuable tool to follow the longitudinal course of BBB damage throughout the natural history and clinical course of bAVMs.

Spontaneous rupture and hemorrhage of renal epithelioid angiomyolipoma misdiagnosed to renal carcinoma: a case report

BackgroundRenal epithelioid angiomyolipoma is a rare and unique subtype of classic angiomyolipoma, characterized by the presence of epithelioid cells. It often presents with nonspecific symptoms and can be easily misdiagnosed due to its similarity to renal cell carcinoma and classic angiomyolipoma in clinical and radiological features. This case report is significant for its demonstration of the challenges in diagnosing epithelioid angiomyolipoma and its emphasis on the importance of accurate differentiation from renal cell carcinoma and classic angiomyolipoma.Case presentationA 58-year-old Asian female presented with sudden left flank pain and was initially diagnosed with a malignant renal tumor based on imaging studies. She underwent laparoscopic radical nephrectomy, and postoperative histopathology confirmed the diagnosis of epithelioid angiomyolipoma. The patient recovered well and is currently in good health with regular follow-ups. This case highlights the diagnostic challenges, with a focus on the clinical, radiological, and histopathological features that eventually led to the identification of epithelioid angiomyolipoma.ConclusionsEpithelioid angiomyolipoma is easily misdiagnosed in clinical work. When dealing with these patients, it is necessary to make a comprehensive diagnosis based on clinical symptoms, imaging manifestations, and pathological characteristics.

Successful embolization with subsequent pancreaticoduodenectomy for intraductal papillary mucinous neoplasm hemorrhage: a case report and review of literature.

Although various complications associated with intraductal papillary mucinous neoplasms have been reported, including acute pancreatitis, duct perforation, and fistula formation, spontaneous bleeding, especially life-threatening bleeding, is infrequent. In this case, emergency pancreatic resection might be one of the therapeutic options, which is associated with poor postoperative outcomes. An 87-year-old woman presented to our hospital with severe anemia (hemoglobin, 4.5g/dl). Contrast-enhanced computed tomography revealed a large cystic lesion in the pancreatic head measuring 15cm, with some solid components and an adjacent hematoma, suggestive of intra-cystic hemorrhage of the intraductal papillary mucinous neoplasm. The patient was hemodynamically unstable and had hypotension. After transcatheter arterial embolization, the patient became hemodynamically stable. Subsequently, an elective pylorus-preserving pancreaticoduodenectomy was successfully performed. Preoperative embolization was effective for subsequent elective pancreaticoduodenectomy in patients with severe intraductal papillary mucinous neoplasm bleeding.

Laboratory Evaluation of Interferences Associated with Factor XIa Inhibitors Asundexian and Milvexian in Routine Coagulation Assays.

Direct oral anticoagulants (DOACs) are increasingly used for the treatment of thrombosis. While inhibitors of factor IIa and factor Xa have shown effectiveness, the risk of bleeding remains a significant concern. Recently, direct factor XIa inhibitors-including asundexian and milvexian-have emerged as potential anticoagulation therapies, based on clinical observations that patients with factor XIa deficiencies seldom present with spontaneous bleeding tendencies. The interferences associated with DOACs in routine and specialised coagulation assays are well-described; however, the interferences associated with emerging FXIa inhibitors are largely uncharacterised. Here, we briefly report the impact of asundexian and milvexian in routine coagulation assays using in vitro plasma-based systems. Asundexian and milvexian induce concentration-dependent prolongations in APTT-based assays with curvilinear regressions, which may be suitable for the measurement of pharmacodynamic effects at peak levels ex vivo. We also report differential sensitivities of APTT-based assays-particularly at higher FXIa inhibitor concentrations-highlighting the clinical need for an extensive evaluation of interferences associated with FXIa inhibitors in coagulation assays.

Switching hemophilia A patients to rVIII-SingleChain: The Iberian experience.

The real-world outcomes of lonoctocog alfa (rVIII-SingleChain), a long-acting factor VIII (FVIII) with a favorable safety and efficacy profile in trials, were assessed in patients with hemophilia A in Iberian (Spain and Portugal). This was a retrospective study involving patients switching to rVIII-SingleChain from other FVIIIs in 7 Spanish and Portuguese hospitals. The efficacy and safety of replacement therapies were compared between 12 months before switching and the period from switching to the end of the study. Twenty-nine patients (median age 25 years; severe hemophilia A, 37.9%) were recruited. Before switching, 12 were on prophylaxis and were followed-up for a median of 12 months. After switching, 17 received prophylaxis with rVIII-SingleChain and were followed-up for a median of 41 months. Those with ≤2 weekly infusions increased from 37.5% before switching to 60.7% after switching to rVIII-SingleChain. The median monthly consumption was 312 IU/kg with prior FVIIIs and 273 IU/kg with rVII-SingleChain. Six spontaneous bleeds were reported in each period in the prophylaxis patients. In the entire cohort, 50 bleeds were reported with prior FVIIIs and 33 were reported after switching to rVIII-SingleChain. Patients requiring ≤1 dose for hemostasis increased from 44.0% with prior FVIIIs to 60.6% with rVIII-SingleChain. Responses were rated good/excellent in 95.4% of cases. No safety concerns were reported. Patients who switched to rVIII-SingleChain prophylaxis had excellent bleeding control and reduced infusion frequency in regular clinical practice, with the subsequent increase in quality-of-life.

Neonatal cardiorespiratory failure caused by splenic hemorrhages: a case report on a fatal and a rescued case

Two cases of neonatal splenic hemorrhage with acute cardiorespiratory failure are described in this report. The first case involves a full-term neonate who was found unresponsive without any witnesses and could not be successfully resuscitated. A postmortem diagnosis revealed a splenic hemorrhage. Second case is an extremely premature neonate who experienced a witnessed cardiovascular collapse on the 14th day of life. Rapid cardiovascular support was administered, resulting in a positive outcome. While splenic hemorrhage is commonly associated with traumatic events, these cases highlight the need of considering spontaneous splenic hemorrhages as a potential cause of acute neonatal compromise, even in the absence of birth-related trauma (e.g., asphyxia, prolonged labor, clavicle fractures, brachial plexus injuries). This report emphasizes the importance of including splenic hemorrhage timely in the differential diagnosis of neonatal cardiorespiratory instability, especially in the absence of more common diagnoses, and discusses the challenges associated with its recognition and treatment.