HEMATOLOGY (US) – VOLUME 2 – WINTER 2009

This issue of US Hematology reflects the magnificently exciting scope of this field, which was so impressively rolled out during the 50th anniversary of the American Society of Hematology at its national meeting in San Francisco in December 2008.

What should physicians and researchers expect from a professional membership? At the very least, we hope for a venue for peer interaction, opportunities for career development, and a way to maintain quality standards and currency within our chosen fields. Several organizations exist for those of us in hemostasis and thrombosis, including the International Society on […]

Partial splenectomy has attracted increasing attention as an alternative to total splenectomy for the treatment of children with hereditary spherocytosis (HS) and other congenital hemolytic anemias. Total splenectomy, although quite effective for the treatment of HS, has been associated with a variety of significant complications, notably overwhelming post-splenectomy sepsis (OPSI).1,2 Other less recognized but potentially […]

Factor VIII (FVIII) and von Willebrand factor (vWF) are glycoproteins that circulate in plasma in a tightly bound complex. Structural defects or deficiencies in either glycoprotein are responsible for the development of the most common inherited bleeding disorders: hemophilia A and von Willebrand disease (vWD). These diseases manifest spontaneous bleeding in the severe form of […]

von Willebrand disease (VWD) is the most common inherited bleeding disorder and is characterised by low levels of and/or abnormal function in the plasma protein von Willebrand factor (VWF). Typically, laboratory investigation entails initial plasma testing of factor VIII coagulant (FVIII:C), VWF protein antigen (VWF:Ag) and VWF activity, which is classically assessed using the ristocetin […]

Intercellular Cross-talk Between Platelets and Leukocytes in Inflamation and Thrombosis

Vena cava filters are potentially life-saving devices that are used to prevent thrombi in the veins of the lower extremities and pelvis from accessing the lungs. They are usually inserted percutaneously into the inferior vena cava (IVC) below the renal veins. Both permanent and temporary filters are available. Filter insertion is recommended when anticoagulant therapy […]

Rationale for Second-generation Tyrosine Kinase Inhibitors Despite the very impressive data from the pivotal International Randomized Study of Interferon and STI571 (IRIS) that earned imatinib its place as a standard of care in the current management of chronic myeloid leukemia (CML), disease progressed by 7% over five years of therapy in chronic-phase (CP) patients.1 An […]

Since the introduction of the tyrosine kinase inhibitors (TKIs), management of chronic myeloid leukemia (CML) has undergone remarkable change. CML is characterized by the presence of the Philadelphia chromosome (Ph), which is formed by the reciprocal translocation of genetic material between chromosomes 9 and 22, t(9;22)(q34;q11). This translocation leads to the fusion of the BCR […]

The completion of the human genome project and additional ongoing efforts such as the international haplotype map (HapMap) project have promoted the exponential development of tools to investigate global genomic profiles at the DNA and transcript levels. Rapidly evolving technology now allows the interrogation of 1.8 million genetic markers (including almost one million single-nucleotide polymorphisms […]

Allogeneic stem cell transplantation (alloSCT) has been utilised in the management of leukaemia for several decades and has established its role in producing long-term remission. In the context of multiple myeloma (MM), alloSCT induces the highest rate of remissions, including molecular remission, resulting in long-term disease-free survival (DFS) in 20 years, but there is good […]

Solid organ transplant (SOT) and hematopoietic stem cell transplant (HSCT) recipients and their grafts are enjoying increased survival due to modern immunosuppressive regimens and aggressive supportive care.1 This success is tempered by an increasing incidence of malignancy following transplant.2 Malignancy is now identified among the three leading causes of mortality in the SOT population along […]

The human red cell surface is coated with a plethora of reactive substances known as blood group antigens, which form 30 systems and serve compatibility roles analogous to human leukocyte antigens (HLAs). Of this vast antigen repertoire, ABO and Rhesus (Rh) embody two of the most medically significant systems as a cornerstone of immunohematology in […]

As a unique electron donor and acceptor, iron is an essential element in most biological systems. However, its chemical reactivity can result in iron potentially becoming a toxin when present in excessive quantities; accordingly, total body iron must be tightly regulated. As humans have no physiological mechanism for the elimination of excess iron, body iron […]

The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis in one or more cell lines, leading to peripheral cytopenias with a tendency to develop into acute leukemia. Supportive care with red blood cell (RBC) transfusions is the mainstay of treatment, although newer ‘low-intensity strategies’ such as […]

Hemoglobinopathies such as thalassemia and sickle cell disease (SCD) impose a significant burden on global healthcare, with >300,000 afflicted infants born each year and approximately 5% of the global population carrying a potentially pathological hemoglobin gene.1 The anemia resulting from these conditions is commonly managed by red blood cell (RBC) transfusions in addition to treatment […]

Blood transfusion therapy and accompanying iron chelation have dramatically improved the quality of life for many patients with severe anemias. Diseases such as β-thalassemia, once fatal in early childhood, can now be managed as chronic conditions compatible with prolonged life. Today, life expectancy varies between 25 and 55 years, depending on patient compliance with medical […]

Pathophysiology of Paroxysmal Nocturnal Haemoglobinuria