Introduction Haemophilia A is one of several genetic inherited clotting disorders, the other most common ones being haemophilia B and von Willebrand's disease. It typically presents in neonates and young…
Introduction Haemophilia B is an inherited X-linked recessive clotting disorder, which can cause excessive bleeding. Haemophilia B causes a similar, but generally less severe disease than haemophilia A. Most cases…
Introduction Iron studies are indicated in patients with anaemia, particularly those with microcytic anaemia. They may also be indicated in microcytosis (without anaemia), general lethargy and tiredness and malnutrition. Iron…
Introduction Leukaemia is a broad term, applied to a range of blood cell cancers. The leukaemias can be classified as acute or chronic, and sub-classified as myeloid or lymphoid. Thus, there…
Introduction Can be divided into two types; Hodgkin’s and non-Hodgkin’s. Lymphoid leukaemias also exist, and the distinction between these and leukaemia - especially chronic lymphocytic leukaemia - can be difficult.…
Introduction Anaemia is one of the most common presenting complaints to general practice. One of several ways in which anaemias can be classified, is by red cell size. As such,…
Marrow fibrosis and splenomegaly, de novo or following transformation of PV or ET Usually >50 years old Abnormal megkaryocytes produced in increased numbers. PDGF and TGFβ are released by megakaryocytes,…
Introduction Myeloma is a malignant disease of the plasma cells of the bone marrow. It is characterised by a proliferation of these plasma cells, and overproduction of an antibody (known…
Clonal proliferation of haematopoietic components in the bone marrow leads to an increase in numbers of one or more lineages of mature blood cells. Myeloproliferative disorders have the potential to…
Factor XI deficiency: mainly found in Ashkenazi Jews, recessive inheritance. Homozygotes <5% levels of factor XI. Factor XI concentrate given. Factor VII deficiency: autosomal recessive, variable bleeding severity, risk of…