Disseminated intravascular coagulation (DIC)

Learning outcome Describe the causes, pathophysiology, clinical and laboratory features of disseminated intravascular coagulation (DIC) Pathophysiology Disseminated intravascular coagulation (DIC) is a complex haemorrhagic disorder caused by uncontrolled activation of the coagulation cascade, usually upon inappropriate release of tissue factor (TF). Other factors such as bacterial components, snake venom and products of conception may alsoContinue reading “Disseminated intravascular coagulation (DIC)”

Vitamin K deficiency, liver disease & warfarin overdose

Learning outcome List the vitamin K dependant factors Explain the mechanism underlying vitamin K deficiency and bleeding Explain the role of warfarin as an anticoagulant and its action on the vitamin K redox cycle Describe the causes, clinical and laboratory features of vitamin K deficiency and warfarin overdose Vitamin K dependant factors and the vitaminContinue reading “Vitamin K deficiency, liver disease & warfarin overdose”

Haemophilia A & B

Learning outcome Describe the pathophysiology, inheritance, clinical and laboratory features of Haemophilia A and B Haemophilia A Pathophysiology Haemophilia A is a condition caused by reduced FVIII activity due to defective synthesis of the FVIII molecule. Its prevalence is estimated at 1 in 5,000 males. No significant geographical variation in its prevalence is reported. The condition isContinue reading “Haemophilia A & B”

von Willebrand’s disease (vWD)

Learning outcome Describe the pathophysiology, inheritance, clinical and laboratory features of von Willebrand disease Introduction The coagulation cascade consists of many interacting proteins that serve as activating proteases and co-factors that amplify the reaction. Functional mutations have been identified in nearly all of the proteins within the coagulation cascade, that results in an heritable bleedingContinue reading “von Willebrand’s disease (vWD)”

Immune thrombocytopenia purpura (ITP)

Learning outcome Describe the pathogenesis, clinical and laboratory feature of immune thrombocytopenia purpura. Pathophysiology Immune thrombocytopenia purpura (ITP) is a condition characterised by low platelet counts resulting from immune destruction of platelets. Auto-antibodies to platelet antigens develop (similar to that seen with red cells in autoimmune haemolytic anaemia) and coat the patient’s platelets that are subsequently removed by the splenicContinue reading “Immune thrombocytopenia purpura (ITP)”

Clinical and laboratory assessment of bleeding

Learning outcome Distinguish the use of common laboratory tests (Platelet count, APTT, PT, TT) to assess abnormalities of the coagulation cascade Clinical assessment and classification of bleeding disorders The clinical assessment of a patient with suspected bleeding disorder should include evaluation of; Age of onset and gender Extent and type of bleeding – skin, mucosal,Continue reading “Clinical and laboratory assessment of bleeding”

Normal haemostasis

Learning outcomes Explain the physiological process of haemostasis involving the blood vessel, platelet and coagulation factors List the coagulation factors that are associated with the intrinsic and extrinsic pathways of coagulation Introduction Haemostasis is the process that occurs when there is vascular injury. It is a complex dynamic process that involves interaction between the endotheliumContinue reading “Normal haemostasis”

Beta thalassaemia

Learning outcomes Relate disorders of globin change synthesis to the pathogenesis of thalassaemia and haemoglobin variants Describe the pathogenesis and clinical presentation of beta-thalassaemia Predict the risk of thalassaemia based on the genetic inheritance pattern of beta-thalassaemia List common molecular mutations associated with beta-thalassaemia in Malaysia Appraise the laboratory tests that are commonly used forContinue reading “Beta thalassaemia”

Alpha thalassaemia

Learning outcomes Relate disorders of globin change synthesis to the pathogenesis of thalassaemia and haemoglobin variants Describe the pathogenesis and clinical presentation of alpha-thalassaemia Predict the risk of thalassaemia based on the genetic inheritance pattern of alpha-thalassaemia List common molecular mutations associated with alpha-thalassaemia in Malaysia Appraise the laboratory tests that are commonly used forContinue reading “Alpha thalassaemia”

Globin gene regulation & thalassaemias

Learning outcomes Describe the structure and composition of haemoglobin Explain changes in globin chain synthesis from foetal life to adulthood Regulation of globin synthesis Haemoglobin is a tetramer composed of four polypeptide globin chains with a central heme component within each of the polypeptide. The heme component consists of an organic protoporphyrin ring with a central iron ion in the ferrous stateContinue reading “Globin gene regulation & thalassaemias”