EUROPEAN HAEMATOLOGY – VOLUME 2 – SUMMER 2008

This issue of European Haematology covers several topics that are of great interest to haematologists because they highlight recent advances in the field or discuss current controversies in the managament of patients.

Vipers (Viperinae) are members of the snake family that develops the most advanced venom-injecting organs, the long sheathed fangs that can be folded like switchblades. Some vipers possess pit organs, infrared detectors for locating warm-blooded prey in the dark. This specialised subfamily is termed crotalidae (Pit viper), separating them from viperidae (True viper). Vipers found […]

In addition, many patients may not be aware of having a bleeding abnormality, since relatives may have the same symptoms. Therefore, it is of the utmost importance to identify patients with underlying bleeding disorders before they undergo surgery. In particular, dentists, gynaecologists and paediatricians should investigate patients for potential bleeding abnormalities. In general, patients with […]

Haemophilia A is the most common plasmatic bleeding disorder, caused by congenitally reduced activity of coagulation factor VIII (FVIII). Affected patients suffer from a more or less severe bleeding tendency, mainly in the joints, muscles and organs, which, untreated, leads to haemophilic arthropathy and high mortality. To date, there is no cure for the disease. […]

The management and treatment of haemophilia is a complex process requiring a lifelong commitment from patients, family members and care providers alike. Recent decades have seen the goals of haemophilia treatment changing beyond solely maintaining prevention of acute bleeds towards the inclusion of improved patient quality of life by preventing severe debilitating outcomes such as […]

The X-chromosome-linked condition of haemophilia is a bleeding disorder that impairs the body’s natural control over coagulation. While there is currently no cure for haemophilia, the disorder can be effectively controlled with infusions of the deficient clotting factor, i.e. factor VIII (FVIII) in the case of haemophilia A and factor IX (FIX) in the case […]

Von Willebrand disease (VWD) is a common hereditary bleeding disorder that may affect as many as 1% of the general population.1 VWD is classified into three major categories based on the type of defect and degree of deficiency of von Willebrand factor (VWF). Type 1 is the most common, affecting approximately 75% of those who […]

In the past VWD1 was reported to be the most frequent form of VWD, accounting for approximately 70% of cases. A 2008 study based on the reappraisal of diagnoses of VWD1 after 10 years in 1,234 patients followed by 16 Italian centres has established that VWD1 accounted for only 671 of the 1,234 cases (55%). […]

When a blood vessel is injured, platelets adhere to the exposed subendothelium (platelet adhesion). The platelets are activated (platelet activation) and secrete their granule contents (platelet secretion). The granule contents include platelet agonists (adenosine diphosphate [ADP] and serotonin) that, by interacting with specific platelet receptors, contribute to the recruitment of additional platelets to form aggregates […]

Corticosteroids are typically used as initial treatment, but if platelet levels fall precipitously or the clinical situation is serious and there is a need to rapidly raise the count, intravenous immune globulins (including anti-D) may be administered. A variety of agents have been used when first-line treatments fail, but few of these are specifically licensed […]

In patients with a first episode of deep vein thrombosis (DVT) of the lower limbs, the standard for establishing the duration of oral anticoagulant therapy (OAT) is based on the nature of the DVT. It is currently three to six months for idiopathic DVT and three months for provoked thrombosis.1 Long-term anticoagulant treatment is highly […]

Risk Factors for Venous Thrombosis and a Model of Thrombotic Risk In 1856, Virchow postulated his triad proposing three main distinct mechanisms that may result in thrombosis: alterations in normal blood flow or stasis; alterations in the constitution of blood or hypercoagulability; and vascular damage.5 The first two mechanisms are thought to be most relevant […]

There is significant diversity in the availability of different parenteral iron preparations in Europe and the US. While non-dextran-containing iron formulations have been available in Europe for more than five decades, sodium ferric gluconate and iron sucrose have been available in the US only since the late 1990s and early 2000s, prior to which iron […]

Aptamers are oligonucleotides that bind to molecular targets in a manner conceptually similar to antibodies.1,2 Through the systematic evolution of ligands by exponential enrichment (SELEX) process, aptamers have been identified against a wide range of therapeutic targets.3–8 Synthetic ribonucleic acid (RNA) aptamers can be modified to have greatly enhanced plasma stability and longer circulating half-lives, […]

Human cytomegalovirus (CMV) is a common human pathogen that infects the majority of the world’s population. CMV is a large species-specific virus that co-evolved with its host for many thousands of years. Since CMV adapted to persist in immunocompetent individuals, there has been tremendous evolutionary pressure on the virus to develop mechanisms that enable it […]

Mother-to-child transmission (MTCT) of HIV causes significant morbidity and mortality among children. Our understanding of the epidemiology, pathogenesis, diagnosis and prevention of MTCT of HIV infection has improved dramatically in recent years, and the objectives of this review are to summarise current understanding of the prevention of MTCT of HIV.

In the initial phase of treatment in acute myeloid leukaemia (AML), patients receive chemotherapy with the intent to eradicate malignant blast cells from bone marrow and other tissues. Induction therapy usually induces the disappearance of microscopically detectable malignant cells (complete remission [CR]), and the ensuing consolidation therapy aims at preventing the subsequent recurrence of leukaemia […]

The majority of patients with MDS are anaemic. Up to 90% of patients will require regular transfusions. In 50–60% of patients, anaemia is severe (haemoglobin level below 10g/dl)1 and is associated with a decreased physical performance and poor quality of life,2 along with increased cardiac morbidity and mortality.3–5 The aim of blood transfusion therapy is […]

The term ‘proteome’ was proposed by Wilkins et al. in 1995 and referred to the whole set of proteins present in a cell or a biological fluid at a given time. The proteome is a fundamentally dynamic entity that reflects the best functional status of a biological system. There is increasing evidence that the large […]

What you should be measuring, how you should be treating and how the interaction between measurements and treatments affect patient outcome have long been subjects of debate in medical practice. For centuries there were few scientific links between measurements that could be taken from patients and the subsequent treatments employed, but as technology developed to […]

The safety of blood and its components continues to be a global problem. More than two-thirds of the world does not have access to safe blood.1 Each year unsafe blood transfusions in developing countries result in eight to 16 million hepatitis B virus (HBV) infections, 2.3–4.7 million hepatitis C virus (HCV) infections and 80,000–160,000 HIV […]

The bleeding disorder immune thrombocytopenic purpura (ITP) is a model for immunological research and new biological treatment modalities of chronic inflammatory and autoimmune disorders. In ITP, immunologically susceptible subjects are recognisable by disturbances of the immune response on various levels. Children have mostly a transient or persistent form (below 12 months’ duration) and rarely have […]

Advanced haemodynamic monitoring is of key importance in the management of children with circulatory failure.1 Therefore, the measurement of cardiac output in children could be of great benefit. Arterial pressure-based continuous cardiac output (APCCO) is an exciting technology that provides cardiac output (CO) monitoring based on the arterial pressure curve, and its use in children […]