Health following recovery from immune thrombotic thrombocytopenic purpura: the patient's perspective.

The impact of residual symptoms following recovery from immune-mediated thrombotic thrombocytopenic purpura (iTTP) on activities of daily living during remission is not routinely discussed or evaluated by hematologists. This study used qualitative methodology to understand 3 issues from the patient's perspective: the most important symptoms during remission, the impact of these symptoms on their daily activities, and the effectiveness of communication with hematologists. Oklahoma and Ohio patients participated in either focus groups or individual interviews. Eligibility included age ≥18 years, ADAMTS13 deficiency (<10% activity) at diagnosis or relapse, and in clinical remission (≥1 year from episode). A nonprobabilistic purposive sampling approach was used. The most important symptoms were defined as symptoms mentioned across all 7 focus groups. The interviews supplemented focus group data. The analysis focused on describing the impact of symptoms and barriers to communicating with hematologists. A total of 44 patients participated (focus groups, N = 25; interviews, N = 19). The most important symptoms affecting the patients' daily activities were cognitive issues, anxiety, depression, and fatigue. These symptoms affected patients' ability to return to their previous level of functioning and created difficulties in relationships. A key communication barrier with their hematologists was forgetting to mention these symptoms. Although hematologists pronounce patients as recovered, iTTP remains a life-changing event. Patients often did not return to their previous functioning; relationships and careers were affected. However, patients may forget to discuss these concerns with their hematologist. To improve remission care, hematologists should incorporate patient-reported outcome measures evaluating these symptoms in remission visits.

Association of low serum albumin with venous thrombosis in pediatric patients.

OBJECTIVES: The incidence of venous thromboembolism (VTE) in children is increasing, attributed in part to increased utilization of central venous catheters (CVCs). Children with protein losing disorders (PLDs) and low serum albumin may have an increased incidence of thrombosis. We sought to determine the prevalence of PLDs and hypoalbuminemia at the time of diagnosis of VTE in pediatric patients and its relationship to central venous catheters. METHODS: We performed a single institution retrospective study of 65 consecutive hospitalized pediatric patients with an acute VTE. Data collected included clinical diagnoses, type of thrombosis, presence or absence of a CVC, and serum albumin level, if available. RESULTS: Of 65 patients with acute VTE, 51 % (33/65) had catheter-related thrombosis (CRT), including 71 % (19/27) of patients <12 years of age and 37 % (14/38) of patients aged 12 to 23 (P = 0.008). Eleven VTEs occurred in patients with a diagnosis of a PLD; of these, ten (91 %) were CRT and one (9 %) was a non-CRT (P = 0.003). Serum albumin levels obtained within four days of diagnosis of VTE were available for 38 patients. An albumin level below the lower limit of the age-adjusted normal reference range was documented in 27/38 (71 %) patients with VTE compared to 1011/3028 (33 %) of all pediatric patients admitted to the hospital during a two-year period (P < 0.0001). Albumin levels were low in 19/22 (86 %) patients with CRT compared with 8/16 (50 %) patients with non-CRT (P = 0.019). CONCLUSION: Low serum albumin levels are highly prevalent among pediatric patients with VTE, especially in those patients with CRT.

Endothelial VWF is critical for the pathogenesis of vaso-occlusive episode in a mouse model of sickle cell disease.

Vaso-occlusive episode (VOE) is a common and critical complication of sickle cell disease (SCD). Its pathogenesis is incompletely understood. von Willebrand factor (VWF), a multimeric plasma hemostatic protein synthesized and secreted by endothelial cells and platelets, is increased during a VOE. However, whether and how VWF contributes to the pathogenesis of VOE is not fully understood. In this study, we found increased VWF levels during tumor necrosis factor (TNF)-induced VOE in a humanized mouse model of SCD. Deletion of endothelial VWF decreased hemolysis, vascular occlusion, and organ damage caused by TNF-induced VOE in SCD mice. Moreover, administering ADAMTS13, the VWF-cleaving plasma protease, reduced plasma VWF levels, decreased inflammation and vaso-occlusion, and alleviated organ damage during VOE. These data suggest that promoting VWF cleavage via ADAMTS13 may be an effective treatment for reducing hemolysis, inflammation, and vaso-occlusion during VOE.

Eptacog beta efficacy and safety in the treatment and control of bleeding in paediatric subjects (<12 years) with haemophilia A or B with inhibitors.

INTRODUCTION: Eptacog beta is a new recombinant activated human factor VII bypassing agent approved in the United States for the treatment and control of bleeding in patients with haemophilia A or B with inhibitors 12 years of age or older. AIM: To prospectively assess in a phase 3 clinical trial (PERSEPT 2) eptacog beta efficacy and safety for treatment of bleeding in children <12 years of age with haemophilia A or B with inhibitors. METHODS: Using a randomised crossover design, subjects received initial doses of 75 or 225 µg/kg eptacog beta followed by 75 µg/kg dosing at predefined intervals (as determined by clinical response) to treat bleeding episodes (BEs). Treatment success criteria included a haemostasis evaluation of 'excellent' or 'good' without use of additional eptacog beta, alternative haemostatic agent or blood product, and no increase in pain following the first 'excellent' or 'good' assessment. RESULTS: Treatment success proportions in 25 subjects (1-11 years) who experienced 546 mild or moderate BEs were 65% in the 75 µg/kg initial dose regimen (IDR) and 60% in the 225 µg/kg IDR 12 h following initial eptacog beta infusion. By 24 h, the treatment success proportions were 97% for the 75 µg/kg IDR and 98% for the 225 µg/kg IDR. No thrombotic events, allergic reactions, neutralising antibodies or treatment-related adverse events were reported. CONCLUSION: Both 75 and 225 µg/kg eptacog beta IDRs provided safe and effective treatment and control of bleeding in children <12 years of age.

Use of infusion ports in patients with sickle cell disease: Indications and complications.

BACKGROUND: Peripheral venous access in patients with sickle cell disease (SCD) can become difficult over time due to frequent access and scarring. Infusion ports provide reliable central venous access. Deep venous thrombosis (DVT) and infections are complications associated with SCD and infusion ports. METHODS: We performed a 17.5-year single-institution retrospective chart review (January 2000 to July 2018) with literature review regarding use of infusion ports in patients with SCD. RESULTS: We identified 32 patients with infusion ports placed for a total of 63 devices (48 for chronic transfusion [CT] and 15 for poor venous access [PVA], not on CT) for a total of 99,272 catheter days. The mean age at first insertion was 8 years (range 1-20 years). Complications included malfunction, infection, thrombosis, difficult access, and pain over infusion port site. The rate of infection was 0.2 per 1000 catheter days. Thrombosis was identified in three devices (5%) in three patients (9%), with a rate of 0.03 per 1000 catheter days. There was no difference in complications by site in either the left or right subclavian vein (p = 1). The rate of premature removal was 0.36 per 1000 catheter days, which was higher among patients with infusion ports solely for PVA (0.87 per 1000 catheter days) compared with those placed for CT (0.29 per 1000 catheter days). CONCLUSION: Infusion ports in patients with SCD was associated with low rates of thrombosis, infection, and malfunction, and may be considered as an alternative to frequent intravenous access, especially in patients requiring CT.

The safety of activated eptacog beta in the management of bleeding episodes and perioperative haemostasis in adult and paediatric haemophilia patients with inhibitors.

INTRODUCTION: Haemophilia patients with inhibitors often require a bypassing agent (BPA) for bleeding episode management. Eptacog beta (EB) is a new FDA-approved recombinant activated human factor VII BPA for the treatment and control of bleeding in haemophilia A or B patients with inhibitors (≥12 years of age). We describe here the EB safety profile from the three prospective Phase 3 clinical trials performed to date. AIM: To assess EB safety, immunogenicity and thrombotic potential in children and adults who received EB for treatment of bleeding and perioperative care. METHODS: Using a randomized crossover design, 27 subjects in PERSEPT 1 (12-54 years) and 25 subjects in PERSEPT 2 (1-11 years) treated bleeding episodes with 75 or 225 µg/kg EB initially followed by 75 µg/kg dosing at predefined intervals as determined by clinical response. Twelve PERSEPT 3 subjects (2-56 years) received an initial preoperative infusion of 75 µg/kg (minor procedures) or 200 µg/kg EB (major surgeries) with subsequent 75 µg/kg doses administered intraoperatively and post-operatively as indicated. Descriptive statistics were used for data analyses. RESULTS: Sixty subjects who received 3388 EB doses in three trials were evaluated. EB was well tolerated, with no allergic, hypersensitivity, anaphylactic or thrombotic events reported and no neutralizing anti-EB antibodies detected. A death occurred during PERSEPT 3 and was determined to be unlikely related to EB treatment by the data monitoring committee. CONCLUSION: Results from all three Phase 3 trials establish an excellent safety profile of EB in haemophilia A or B patients with inhibitors for treatment of bleeding and perioperative use.

PERSEPT 3: A phase 3 clinical trial to evaluate the haemostatic efficacy of eptacog beta (recombinant human FVIIa) in perioperative care in subjects with haemophilia A or B with inhibitors.

INTRODUCTION: Surgical procedures in persons with haemophilia A or B with inhibitors (PwHABI) require the use of bypassing agents (BPA) and carry a high risk of complications. Historically, only two BPAs have been available; these are reported to have variable responses. AIM: To prospectively evaluate the efficacy and safety of a new bypassing agent, human recombinant factor VIIa (eptacog beta) in elective surgical procedures in PwHABI in a phase 3 clinical trial, PERSEPT 3. METHODS: Subjects were administered 200 µg/kg (major procedures) or 75 µg/kg eptacog beta (minor procedures) immediately prior to the initial surgical incision; subsequent 75 µg/kg doses were administered to achieve postoperative haemostasis and wound healing. Efficacy was assessed on a 4-point haemostatic scale during the intra- and postoperative periods. Anti-drug antibodies, thrombotic events and changes in clinical/laboratory parameters were monitored throughout the perioperative period. RESULTS: Twelve subjects underwent six major and six minor procedures. The primary efficacy endpoint success proportion was 100% (95% CI: 47.8%-100%) for minor procedures and 66.7% (95% CI: 22.3%-95.7%) for major procedures; 81.8% (95% CI: 48.2%-97.7%) of the procedures were considered successful using eptacog beta. There was one death due to bleeding from a nonsurgical site; this was assessed as unlikely related to eptacog beta. No thrombotic events or anti-eptacog beta antibodies were reported. CONCLUSION: Two eptacog beta dosing regimens in PwHABI undergoing major and minor surgical procedures were well-tolerated, and the majority of procedures were successful based on surgeon/investigator assessments. Eptacog beta offers clinicians a new potential therapeutic option for procedures in PwHABI.

Recognizing and managing hereditary and acquired thrombotic thrombocytopenic purpura in infants and children.

We describe how infants and children with hereditary and acquired autoimmune thrombotic thrombocytopenic purpura (TTP) initially present and how they can be promptly diagnosed and effectively managed. These are uncommon disorders that are commonly misdiagnosed and can be rapidly fatal. TTP is caused by a severe deficiency of the plasma protease, A disintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS13). Measurement of ADAMTS13 activity is becoming easily accessible. A common presentation of hereditary TTP is neonatal severe hemolysis and hyperbilirubinemia. However, the median age of diagnosis is not until 5.5 years. Plasma is effective treatment for exacerbations and for prophylaxis. Plasma may be replaced by recombinant ADAMTS13 when it becomes available. Acquired TTP is more frequent in older children, in whom it is more common in girls and is commonly associated with systemic lupus erythematosus. For acquired TTP, plasma exchange and immunosuppression are the current treatment for acute episodes; caplacizumab is now commonly used in adults and may replace plasma exchange.

Physical activity in children at risk of postthrombotic sequelae: a pilot randomized controlled trial.

Increased physical activity is protective against worsening of postthrombotic syndrome (PTS) in adults. We assessed patient eligibility, consent, adherence, and retention rates in a pilot trial of prescribed physical activity following venous thromboembolism (VTE) in children. Secondary objectives were to describe the within-subject changes in PTS, quality of life, and coagulation biomarkers before and after the intervention in each group. We enrolled and randomized patients between 7 and 21 years of age to the physical activity group or the standard care (education-only) group in a 1:1 allocation ratio. The physical activity group wore a Fitbit for 4 weeks to determine habitual activity and then increased activity over an 8-week "active" period, followed by a 4-week "do-as-you-wish" period. Two hundred thirty-five children were diagnosed with VTE; 111 patients were screened, of whom 40 (36%) met study eligibility criteria. Of these, 23 (57%) consented to participate and were randomized (Fitbit,11; standard group, 12). The trial was of greater interest to overweight and obese children, as they comprised 83% of consented patients. Only 33% adhered to the activity prescription, and 65% (15/23) completed the trial. The PTS scores (P = .001) improved in the physical activity group compared with the education-only group. It is feasible to enroll and randomize pediatric VTE patients to a prescribed physical activity regimen 3 months following VTE. Metrics for adherence to enhanced physical activity and retention were not met. These results provide the rationale to explore low adherence and retention rates before moving forward with a larger trial of exercise training following VTE. This trial was registered at www.clinicaltrials.gov as #NCT03075761.

Thrombin generation in children with sickle cell Anemia is Higher in the presence of platelets⋆ and ⋆.

Cellular and plasma interactions underlie hypercoagulability in sickle cell anemia (SCA). In healthy adults, thrombin generation (TG), a biomarker of hypercoagulability, is similar in plasma with and without platelets. Studies investigating TG in SCA using platelet-poor plasma (PPP) show conflicting results. There are no studies in SCA simultaneously comparing TG using platelet rich plasma (PRP) and PPP. This prospective study compares TG in children with SCA, at steady state, in PPP versus PRP and investigates the association of predefined clinical variables with the difference between PRP and PPP. Our secondary aim was to investigate derangements in the protein C and S pathway measuring TG with and without thrombomodulin (TM). In forty-three paired samples from SCA patients, aged 2-15 years, TG in the presence of platelets was 5.9 % higher [1239 nmol/(min*L) (SD: 224.1) vs. 1151 nmol/(min*L) (SD 223.3); p = 0.026]. The difference was highest in the 6-10 year age group (9.5 %; SD 14.1) followed by the 2-5 year age group (5.4 %; SD 21.4). In a multiple linear regression model, age, gender, current use of hydroxyurea, degree of hemolysis and severity of pain crises were not predictive of the difference between PRP and PPP. In PPP, TG reduction after TM addition was 7.4 % (SD 16.8), signifying activated protein C resistance. In conclusion, TG in children with SCA aged 2-10 years is higher in the presence of platelets. TG using PRP along with TM addition may be a useful biomarker of hypercoagulability in this population.

Thromboelastography-targeted management of severe coagulopathy and off-label use of four-factor prothrombin complex concentrate in an infant with massive bleeding.

: A 4-month-old girl initially presented to the pediatric ICU at 9 days old with multiorgan failure and cerebellar hemorrhage secondary to disseminated enteroviral infection and was eventually listed for liver transplant. Due to severe coagulopathy, she developed a hemothorax after line placement. Despite operative exploration and multiple recombinant factor VIIa doses, massive bleeding continued. The bleeding was finally controlled with thromboelastography-targeted platelet and cryoprecipitate transfusion in addition to four-factor prothrombin complex concentrate administration. This management strategy was invaluable in controlling bleeding from an iatrogenic cause.

Evidence-Based Strategies and Recommendations for Preservation of Central Venous Access in Children.

Children with chronic illness often require prolonged or repeated venous access. They remain at high risk for venous catheter-related complications (high-risk patients), which largely derive from elective decisions during catheter insertion and continuing care. These complications result in progressive loss of the venous capital (patent and compliant venous pathways) necessary for delivery of life-preserving therapies. A nonstandardized, episodic, isolated approach to venous care in these high-need, high-cost patients is too often the norm, imposing a disproportionate burden on affected persons and escalating costs. This state-of-the-art review identifies known failure points in the current systems of venous care, details the elements of an individualized plan of care, and emphasizes a patient-centered, multidisciplinary, collaborative, and evidence-based approach to care in these vulnerable populations. These guidelines are intended to enable every practitioner in every practice to deliver better care and better outcomes to these patients through awareness of critical issues, anticipatory attention to meaningful components of care, and appropriate consultation or referral when necessary.

Differentiation of Deep Venous Thrombosis Among Children With or Without Osteomyelitis.

BACKGROUND: Children with osteomyelitis are at risk for deep venous thrombosis (DVT). This study evaluates the characteristics of DVT among children to differentiate between those with and without osteomyelitis. METHODS: Children with DVT of any cause were studied between 2008 and 2016. Children with DVT and osteomyelitis were compared with those with DVT without osteomyelitis. Another comparison cohort included children with osteomyelitis but without DVT. Comorbidities, severity of illness (SOI), and clinical course were compared between cohorts. RESULTS: DVT was identified in 224 children, a prevalence of 2.5 per 10,000 children. Among those with DVT, 28 (12.1%) had osteomyelitis. The DVT rate among 466 children with osteomyelitis was 6.0%. Children with osteomyelitis and DVT had greater SOI (9.1 vs. 2.7), bacteremia rate (82.1% vs. 38.4%), methicillin-resistant Staphylococcus aureus rate (89.3% vs. 21.2%), surgeries per child (2.1 vs. 0.7), and intensive care unit admission rate (67.9% vs. 5.9%) than that of children without DVT (P<0.00001). Of 196 children who had DVT without osteomyelitis, 166 (84.7%) had comorbidities including defined hypercoagulability (27 or 13.8%). Children with DVT due to osteomyelitis were without comorbidities or hypercoagulability (P<0.00001). The rate of pulmonary embolism was similar for children with DVT with or without osteomyelitis (3/28, or 10.7% vs. 18/196, or 9.2%). CONCLUSIONS: Children with DVT and osteomyelitis differ substantially from other children with DVT by the absence of comorbidities or post-thrombotic syndrome. They also differ from children with osteomyelitis without DVT by higher SOI, methicillin-resistant S. aureus rate, and occurrence of intensive care. Awareness of for the characteristics of DVT among children with osteomyelitis will reduce delay to diagnostic ultrasound and improve anticoagulation management which must be carefully coordinated given the high rate of surgery of these children. LEVEL OF EVIDENCE: Level II-prognostic, retrospective cohort comparison.

Anticoagulation results in increased line salvage for children with intestinal failure and central venous thrombosis.

PURPOSE: The purpose of this study was to investigate whether anticoagulation (AC) results in thrombus resolution and increased line longevity in children with intestinal failure (IF) and catheter-associated central venous thrombosis (CVT). METHODS: A retrospective, single institution review was performed of children with IF who were dependent on parenteral nutrition with known CVT between 2006 and 2017. Frequency of catheter-related complications including infection, occlusion, and breakage were compared 18months prior to and after starting AC. Thrombus resolution during anticoagulation was also determined. Data were analyzed using Poisson regression. p-Values <0.05 were considered significant. RESULTS: Eighteen children had ≥1 CVT, with the subclavian vein most commonly thrombosed (44%). All children were treated with low molecular weight heparin, and 6 patients (33%) had clot resolution on re-imaging while receiving AC. Bloodstream infections decreased from 7.9 to 4.4 per 1000 catheter days during AC (p=0.01), and the number of infections requiring catheter replacement decreased from 3.0 to 1.0 per 1000 catheter days (p=0.01). There were no significant differences in line occlusions or breakages. CONCLUSION: Anticoagulation for children with intestinal failure and central venous thrombosis may prevent thrombus propagation, and decrease blood stream infections and line replacements. Further research is needed to determine optimal dosing and duration of therapy. LEVEL OF EVIDENCE: III; Retrospective Comparative Study.

Craniectomy and Traumatic Brain Injury in Children on Extracorporeal Membrane Oxygenation Support.

Severe trauma may cause refractory life-threatening respiratory failure requiring extracorporeal membrane oxygenation (ECMO). Concurrent traumatic brain injury, however, complicates the use of ECMO because of the major risk of intracranial bleeding with systemic anticoagulation. Craniotomy and/or craniectomy for hematoma evacuation during ECMO are extremely high-risk procedures secondary to ongoing anticoagulation, and there are only a few such case reports in the literature. We present the case of a child with multiple thoracic injuries and life-threatening respiratory failure supported on ECMO. She developed an intracranial hemorrhage while systemically heparinized that required emergent decannulation and bedside craniectomy for hematoma extraction. She survived with an excellent neurologic outcome. We also review the relevant literature regarding the use of ECMO in patients with polytrauma and the occurrence of craniectomy on extracorporeal support, with a focus on pediatric publications. Patients with polytrauma with brain injury can be supported on ECMO, but extreme precaution must be taken regarding anticoagulation. The intracranial complications of ECMO in this population are not infrequent, but our case report and review of the literature suggest that neurosurgical intervention should be considered in life-threatening conditions when no other alternatives are available.

Markers of coagulation activation, inflammation and fibrinolysis as predictors of poor outcomes after pediatric venous thromboembolism: A systematic review and meta-analysis.

BACKGROUND: Sequelae of venous thromboembolism (VTE) in children include recurrence, development of post thrombotic syndrome (PTS) when venous return from a limb is affected and chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism. Identification of laboratory-based risk factors may be useful for individualized risk assessment for VTE sequelae. Coagulation activation and inflammation may contribute to their pathophysiology. We performed a systematic review to investigate the association between biomarkers of coagulation activation, inflammation and fibrinolysis and adverse VTE outcomes in children and young adults. METHODS: A systematic search of electronic databases, PubMed (NIH), EMBASE (Ovid), Web of Science (Thompson Reuters), and SCOPUS (Elsevier) for studies published through November 2016 was conducted using "VTE" including MeSH terms for "coagulation activation," "inflammation" and "fibrinolysis," with no limit on publication date. A study was eligible for inclusion when it evaluated patients (< 21years) with VTE and biomarkers of coagulation activation, inflammation, and fibrinolysis and assessed for their association with development of adverse thrombotic outcomes. A modified Newcastle Ottawa Scale was applied to examine the quality of included studies. RESULTS: Our search strategy yielded 200 references. A total of 3 cohort studies representing 220 patients with VTE were included. Two authors independently assessed all references for inclusion. Three studies (2 prospective cohort and one mixed cohort study) were identified that reported on biomarkers of coagulation activation, inflammation and fibrinolysis, checked at least once after VTE diagnosis and assessed association with primary outcomes of recurrent VTE, PTS and CTEPH. Studies varied with regards to definition of outcomes, the type of biomarkers measured and time point of measurement. We were unable to meta-analyze results due to marked clinical heterogeneity and <3 studies available for each biomarker. Descriptively, a significant association was found for elevated plasma levels of FVIII and D-dimer for a compound outcome of PTS, recurrence and progression in one study, and positive lupus anticoagulant at DVT diagnosis and subsequent PTS by another study. No studies were found for CTEPH. CONCLUSIONS: Elevated D-dimer, FVIII and lupus anticoagulant show promise for predicting recurrent VTE and PTS in children and young adults. Further research is needed to elucidate whether these markers might be useful to predict development of adverse outcomes after VTE in children.

Coagulation Profile Is Not a Predictor of Acute Cerebrovascular Events in Pediatric Extracorporeal Membrane Oxygenation Patients.

We performed a retrospective matched case-control study evaluating whether the traditional coagulation profile predicts cerebrovascular events in children on extracorporeal membrane oxygenation (ECMO) in a 71 bed intensive care unit at a tertiary children's hospital. Between 2009 and 2014, 241 neonates and children were initiated on ECMO. The cumulative 5 year incidence of intracranial hemorrhage and infarct was 9.2% and 7.9%, respectively. Thirty-six cases were individually matched 1:1 with control subjects based on age, primary diagnosis, ECMO type, cannulation site, and the presence of pre-ECMO coagulopathy. In-hospital mortality was higher among the cases compared with control subjects (78 vs. 22%, p < 0.01). The median laboratory values that assisted with heparin anticoagulation monitoring (activated clotting time, partial thromboplastin time, and antifactor Xa) and the laboratory data that assisted with blood product administration (platelet count, prothrombin time, fibrinogen, and d-dimer) during the 24 and 72 hour periods before the cerebrovascular event did not show any significant difference between the hemorrhage group and their controls or between the infarct group and their controls. The traditional coagulation profile did not predict acute cerebrovascular events in our cohort. Other markers of neurologic injury on ECMO are yet to be elucidated. Prospective studies to determine better predictors of cerebrovascular complications in pediatric ECMO patients are required.

Pre-ECMO coagulopathy does not increase the occurrence of hemorrhage during extracorporeal support.

INTRODUCTION AND METHODS: Observational retrospective cohort study to evaluate the association between precannulation coagulopathy and the occurrence of hemorrhage during extracorporeal membrane oxygenation (ECMO) in neonatal and pediatric patients at a tertiary children's hospital. RESULTS: Of 241 patients supported with ECMO between January 2009 and December 2014, 175 (72.6%) had precannulation coagulation laboratory data and were included in the study. Of the eligible patients, 84 (48%) were identified as coagulopathic and 91 (52%) were noncoagulopathic. In the coagulopathic group, sepsis (27.3%) was the most common diagnosis leading to ECMO. Over half of the patients in both groups (55.9% of the coagulopathic and 52.7% of the noncoagulopathic group) developed hemorrhagic complications during ECMO support. The most frequent bleeding sites for both groups were the cannulation site (24%), the chest tube site (17%), and intracranial (10%). Pre-ECMO coagulopathy was not associated with higher incidence of hemorrhage during extracorporeal support (p = 0.76). CONCLUSIONS: Pre-ECMO coagulopathy was frequent in our cohort but did not increase the occurrence of hemorrhage during extracorporeal support. Although the identification of factors associated with hemorrhage is key to safely managing ECMO anticoagulation, the implication of precannulation coagulopathy seems to be minimal.