Blood Coagulation & Fibrinolysis : An International Journal in Haemostasis and Thrombosis

The study was conducted to examine prevalence of pulmonary embolism in children with sickle cell disease (SCD) and identify potential risk factors associated with pulmonary embolism in a single tertiary paediatric centre. Children with SCD between 0 and 21 years of age from January 2010 to January 2021 were included. Pulmonary embolism was initially identified using International Classification of Diseases (ICD)-9 or 10 codes and confirmed with manual chart review of identified cases. Logistic regression analysis was performed to assess association between SCD specific and general thrombotic risk factors and pulmonary embolism...

Heparin-induced thrombocytopenia (HIT) is an uncommon but serious complication of exposure to heparin. Antibody optical densities (ODs) used to diagnose HIT exceeding 2 are highly suggestive of disease, whereas ODs less than 0.5 often 'rule out' HIT. Variation in the clinical care of patients with inconclusive ODs between 0.5 and 2 is likely. This single-centre, retrospective analysis evaluates the diagnosis, management and outcomes of those with antibody ODs between 0.5 and 2. We queried our institution's Healthcare Enterprise Repository for Ontological Narration (HERON) database to identify individuals with antibody ODs between 0...

Exposure to higher levels of steroid hormones, like that in pregnancy or during combined hormonal contraception, increases the risk of venous thromboembolism. Development of resistance to activated protein C (APC) thought to be the underlying pathomechanism of this prothrombotic state. This coagulation phenomena is largely to be explained by the hormone-induced impairment of the protein S/ tissue factor pathway inhibitor (TFPI) leading to a less efficient inactivation of factor Va and factor VIIIa by APC. APC resistance and decreased protein S/TFPI function were associated with the risk of first as well as recurrent venous thromboembolism...

A 44-year-old woman with obstetric antiphospholipid syndrome (APS) presented to our institution with suspected non ST-elevation myocardial infarction (NSTEMI). Shortly after coronary angiography, she developed diplopia, hypotension and chest pain with inferior ST elevation in 12-lead ECG. According to multidisciplinary evaluation, she promptly underwent systemic thrombolysis, with clinical and haemodynamic improvement. Eventually, a diagnosis of catastrophic APS was made, with multiorgan ischemic involvement confirmed by blood examinations and multimodal imaging techniques...

Although worldwide-known criteria of antiphospholipid syndrome include thrombotic and obstetric events, a moderate number of patients manifest with bleeding episodes during course of the disease, which is typically attributed to the long-term anticoagulation. However, these haemorrhagic manifestations sometimes are part of pathophysiological changes that might occur secondary to the disease that involves endothelial activation, platelets dysfunction and blood clot factors misfunction. Recognizing these mechanisms of bleeding is crucial not only due to the need of treatment change or adding, but also because of changes in the disease' prognosis...

Heavy menstrual bleeding is one of the most common causes of consultation in haematology. We present the clinical case of a 20-year-old woman referred by her gynaecologist due to heavy menstrual bleeding since menarche, complicated by iron deficiency anaemia. Haemostasis work-up was initially suggestive of a von Willebrand disease type 1. Genetic analyses by whole exome sequencing lead to a fortuitous discovery of haemophilia by identifying a heterozygous missense mutation in F8, exon 8 c.1127T>G:p.Val376Gly, previously reported in a patient with mild haemophilia A...

BACKGROUND: The postthrombotic syndrome (PTS) is a long-term complication of deep venous thrombosis (DVT). Increase knowledge on the PTS pathophysiology and novel biomarkers are needed in order to predict PTS development and to improve treatment results. The aim of this study was to analyze novel endothelium-biomarkers for PTS in patients with DVT out of the acute phase. METHODS: A case-control study was conducted. Inclusion criteria were symptomatic and confirmed DVT patients treated with anticoagulants for at least 3 months...

UNLABELLED: Direct oral anticoagulants (DOACs) interfere with many coagulation assays, mostly in lupus anticoagulant (LA) detection, causing false positive and negative results. Despite guidelines recommendations, LA testing may be important during anticoagulation when the clinician has to decide whether to prolong or discontinue the drug. OBJECTIVES: In this study, the effect of activated charcoal (DOAC-Stop, DS) as a DOAC-adsorbent was investigated on samples from DOACs treated and untreated patients...

Hemophilia, a congenital coagulopathy characterized by a deficiency in coagulation factor VIII (hemophilia A) or factor IX (hemophilia B), results in a tendency of bleeding proportional to the lacking factor. Most bleeds in patients with hemophilia occur in their joints and muscles, and because of these bleeding episodes, patients may end up developing musculoskeletal alterations resulting from hemophilic arthropathy, even receiving hemostatic treatment. The third edition of the World Federation of Hemophilia's Guidelines for the Management of Hemophilia defines 12 principles that encompass all the different types of multidisciplinary care required by people with hemophilia, one of these being clinical and epidemiological research...

The new coagulation factors have made possible to increase the life expectancy of patients with hemophilia to around 70 years of age. However, there are some comorbidities to take into account such as obesity, hypertension, cardiovascular or metabolic diseases. Improving dietary habits and promoting physical exercise are the two ways of preventing this. But, less than 50% of people with haemophilia actually do something in this sense, even though to practice physical activity or sport is save if you under treatment...

In haemophilia, screening protocols in the prevention and treatment of common lesions still require unification of criteria. Patients with haemophilia seek medical consultation exclusively for two reasons: because they have requested an appointment for a routine check-up (1-2 times a year in case of severe haemophilia) or because they have developed acute bleeding that requires treatment. The purpose of this paper is to emphasize the importance of an early differential diagnosis of joint damage and to review the techniques that allow an effective evaluation...

In addition to receiving the medical treatment they need, people with hemophilia or other coagulopathies must acquire the strategies required to deal with the physical, emotional and social challenges they will encounter at the different stages of their lives. To this end, it is necessary to offer support for any uncertainty that may arise for the patient or parents upon diagnosis of the disease, and to help them develop a healthy response pattern to bleeding episodes. First years and decades of life are essential because self-image is built and the foundations for socialization are laid, making it necessary to foster communication and self-esteem...

Bleeding into joints, known as hemarthrosis, is the most common kind of bleeding experienced by patients with hemophilia. Repeat bleeds into the same joint lead to the so-called hemophilic arthropathy. Patients with this condition tend to require surgery earlier and most frequently than the general population. Successful hemostasis is essential to carry out such procedures. Thanks to the advances made in the treatment of hemophilia, most surgical techniques can be performed safely and reliably. The present review shall focus on the international recommendations related to the performance of these surgical procedures...

Pain is one of the most common reasons for consultation and one of the most difficult tasks to handle for doctors. If it is chronic, the frequency of disorders increases associates that we cannot exclude from the treatment, and further complicates your treatment. The Asociation International Association for the Study of Pain (IAPS)1 defines the pain as "a sensory and emotional experience unpleasant associated with actual or potential tissue damage, or described in terms of such damage". So the pain is not only a nociceptive experience, but also involves emotional and subjective components, considering that It can even occur without a somatic cause justify...

It is essential that joint bleeds be treated in a hematologically and orthopedically optimal manner so as to arrest the bleeding as soon as possible and prevent potentially irreversible joint damage from setting in. The main goal of rehabilitation in the context of hemophilia is above all prevention and treatment of the consequences of musculoskeletal bleeding. Rehabilitation of acute joint bleeding episodes, that is, hemarthrosis, is based on three fundamental pillars: arthrocentesis, PRICE measures, and rehabilitation protocols...

The purpose of prophylaxis in hemophilic patients is to prevent bleeding. The latest guidelines of the World Hemophilia Federation recommend that all patients with a severe hemorrhagic phenotype should receive prophylactic treatment, defined as the regular administration of therapeutic products (either factor concentrates or nonfactor replacement treatments). These products are aimed at preserving hemostasis and preventing bleeding, especially into joints. The guidelines also stipulate that prophylaxis should allow patients with hemophilia to lead healthy and active lives, participating in most physical and social activities, similar to the nonhemophilic population...

This document focuses on the hematological treatment and prophylaxis that should be indicated in three groups of patients with hemophilia: Patients with inhibitors; Patients without inhibitors; and Patients receiving nonfactor replacement therapy. Inhibitors contribute to refractoriness to replacement treatment, leading to more severe bleeds. Immunotolerance and bypassing agents are essential to eradicate the inhibitors and bleeding control, respectively. Noninhibitors patients may receive standard half-life or extended half-life products, either on demand or prophylactically...

People with hemophilia tend to develop joint lesions secondary to the recurrent hemarthroses typical of their condition. These usually include chronic synovitis and arthropathy chiefly affecting their ankles, knees, and elbows. In addition, muscular hematomas, albeit less frequently, may also result in complications such as acute compartment syndrome, pseudotumors, bone cysts and peripheral nerve compression. Joint lesions may require some of the following surgical interventions: arthroscopic synovectomy (in cases of synovitis), arthroscopic joint debridement, radial head resection, opening-wedge tibial osteotomy, arthrodesis, arthrodiastasis (of the ankle), tendon lengthening (hamstrings, Achilles tendon), progressive extension of the knee by placing an external fixator in cases of flexion contracture of the knee, supracondylar femoral extension osteotomy in cases of knee flexion contracture and, eventually, a total joint arthroplasty when the affected joint has been destroyed and the patient experiences severe joint pain...

People with haemophilia tend to experience pain from an early age because of venipuncture and hemarthrosis. If pain is not properly managed, it can become chronic and bedevil patients throughout their lives. Therapies are currently available that have been shown to effectively treat the different types of pain and their causes. Patients with haemophilia tend to experience either nociceptive or mixed pain. Identification of the cause of pain by means of imaging techniques, and understanding the characteristics, location and intensity of the pain, are essential for a more targeted therapeutic approach...

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