EUROPEAN HAEMATOLOGY REVIEW – VOLUME 1 – SUMMER 2007

Optimal blood cell production and function is critical for healthy life. Too few or too many blood cells or the production of blood cells that do not function normally result in malignant and non-malignant disorders. These disorders can be fatal if not correctly treated. Blood cells are produced by a catenated pipeline initiated by haematopoietic […]

Approximately 30% of severe haemophilia A patients suffer an immune response to therapeutically administered factor VIII (FVIII). The formation of inhibitor antibodies is a serious complication in the treatment of haemophilia, and neutralisation of FVIII coagulation activity results in an inadequate response to FVIII infusion.1 Patients with severe haemophilia A have the highest incidence of […]

The development of inhibitors in children with haemophilia A presents a major problem in terms of both the success and the cost of treatment.1 Inhibitors develop in response to treatment with factor VIII (FVIII), which is administered to haemophilia A patients to overcome bleeding episodes. The risk of inhibitor development is highly dependent on disease […]

Haemophilia A (classic haemophilia) is an X-chromosome-linked bleeding disorder occurring in approximately one in 5,000–10,000 males worldwide.1,2 Haemophilia A is caused by a partial or total deficiency of functionally active coagulation factor VIII (FVIII). Haemophilia produces abnormal bleeding that may be mild, moderate or severe depending on the degree of FVIII deficiency. Individuals with severe […]

von Willebrand disease (vWD) and haemophilia A are the most frequent bleeding disorders in the general population, occurring in 66–100 people per million worldwide.1 vWD is caused by both qualitative and quantitative defects in von Willebrand factor (vWF) resulting in a dual defect in haemostasis, i.e. the abnormal platelet adhesion due to reduced and/or dysfunctional […]

Inherited Platelet Disorders

The Problem

In the case of an isolated chronic thrombocytopenia in an adult, the diagnostic procedure is well established. First, several causes must be ruled out. These include: • false thrombocytopenia diagnosis caused by platelet aggregation and clumping in the presence of ethylenediaminetetraacetic acid (EDTA) anticoagulant; • haematological malignancy and myelodysplastic syndromes; • viral infections (particularly HIV […]

Idiopathic thrombocytopenic purpura (ITP) is a primary acquired disease of adults and children characterised by transient or persistent decrease of the platelet count.1,2 Bleeding may be severe and is usually related to the platelet count. When platelet count is lower than 20–30×109/l, bleeding is manifested by variably extensive purpura and mucosal haemorrhages; occasionally, in the […]

Globally, multiple myeloma (MM) accounts for 0.8% of all cancer deaths, with a survival rate of 50% of those enrolled in clinical trials.1,2 It is the most common bone marrow cancer in Europe, with over 77,000 patients undergoing treatment at any one time.3 In the US, MM is the second most common haematological malignancy, affecting […]

Therapeutic goals vary in MDS, with some treatments aimed at symptom control while others aim to alter disease biology and improve survival. Both treatment approaches are relevant in different contexts, and the International Working Group (IWG) criteria have been developed to encompass a range of clinically pertinent measurements to monitor disease response. The evaluation of […]

The transfusion of allogeneic blood products is a complex medical therapy relying on materials that are often available only in short supply and carry a number of substantial risks for the recipient. Perhaps the most feared complication of transfusion is the transmission of infectious diseases. Patients receiving blood products are potentially exposed to any number […]

Immune thrombocytopenic purpura (ITP) is an autoimmune bleeding disorder characterised by antibody-induced platelet destruction.1,2 Intracranial haemorrhages are the most serious and life-threatening complications, with mortality of 0.2–1% in acute ITP and up to 3% per year in the chronic form.3,4 Almost all intracranial haemorrhages occur when platelet counts (PCs) are below 20×109/l, and most below […]

Prevalence of Cytomegalovirus Infection with the human herpesvirus cytomegalovirus (CMV) is a common occurrence, with an estimated 40–85% of the population in Europe and North America infected and harbouring the virus in a dormant form.1 The incidence of infection increases with age and is more common in women than in men, but infection rates depend […]

Normal blood cell production – haematopoiesis – is crucial for the maintenance of health.1 Haematopoiesis is initiated through rare populations of haematopoietic stem (HSC) and progenitor (HPC) cells that give rise to all blood-forming elements: HSCs/HPCs are found in the bone marrow of adults, where they are produced and nurtured. HSCs/HPCs are also found in […]

In June 2007, the World Health Organization (WHO) released its report on air travel and thrombosis, summarising the results of the WHO Research Into Global Hazards of Travel (WRIGHT) project.1 Venous Thrombosis

Pathogenesis of Venous Thromboembolism

Thalassaemias were traditionally believed to occur only in regions of the world where malaria is or was endemic, including the Mediterranean, the Middle East, South and East Asia, the Pacific and South China, with carrier rates varying from 2 to 25%. It is now known that haemoglobin disorders occur widely across the world, including in […]

Significant morbidity and mortality are found in the immune-mediated haematological disorders autoimmune haemolytic anaemia (AIHA) and idiopathic thrombocytopenic purpura (ITP). Despite the availability of several treatment options, relapsed or refractory disease is frequently encountered and treatment-related complications are of major concern.

Fanconi anaemia (FA) is a genetic, life-threatening disorder1,2 featuring progressive bone marrow failure, birth defects, leukaemia, increased incidence of solid tumours, spontaneous chromosomal instability and hypersensitivity to cross-linking reagents.1,2 Because of FA’s relationship to DNA damage hypersensitivity and its association with susceptibility to neoplastic transformation, the FA research field has gained much attention in recent […]

Helicobacter pylori is a microaerophilic, Gram-negative, spiral-shaped, flagellated bacterium that colonises the mucous layer of the human stomach.1 It has been causally linked with a diverse spectrum of gastrointestinal disorders, including gastritis, peptic ulcer disease, non-ulcer dyspepsia, gastric adenocarcinoma and mucosa-associated lymphoid-tissue lymphoma.2 Several investigators have studied whether H. pylori causes non-digestive diseases, but these […]