haemoglobinpathies and haemostasis W6 and 7 Flashcards
what is haemoglobinopathies
synthesis of abnormal haemoglobin
what is a normal adult haemoglobin level
95-98%
structure of haemoglobin?
hb = 4 globin + haem
structure of haemoglobin?
hb = 4 globin + haem - contains Fe
what is thalassemia
reduced synthesis of haemoglobin
what is alpha thalassemia
deficiency in production of alpha haemoglobin chains due to a gene deletion in alphaglobin gene
what is beta thalassemia
deficiency in production of beta haemoglobin chains due to a point mutation in betaglobin
what are both alpha and beta thalaseemia
autosomal recessive
what does HPLC detect
different haemoglobin
what is primary haemostasis
vasoconstriction and platelet plug formation
what is secondary haemostasis
plates plug reinforced by conversion of fibrinogen to fibrin, coagulation cascade
what is the role of the von willebrand factor
platelets agglutinate to damages vascular sub-endothelium
what are factors affecting haemostasis
anticoagulants
liver disease
vitamin K deficiency
what are factors affecting haemostasis
anticoagulants
liver disease
vitamin K deficiency
what are natural coagulants
proteins C, S
antithrombin
what are hereditary diseases that lead to abnormal bleeding
Haemophilia A and B
what is disseminated intravascular coagulation (DIC)
- fibrin generated
- pro-coagulant clotting factors and platelets are used for no reason
what is the treatment for DIC
blood transfusions
cryoprecipitate
heparin used to control thrombosis
why are RH antibodies important
used to detect compatibility if two blood groups are mixed
what is given to women to prevent haemolytic disease in children
K-
