Notes in Bleeding Disorders LECTURE 31

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Published	01/07/2025	bleeding disorder:problem with the blood {{c1::clotting}} process which can lead to abnormally heavy and prolonged bleeding (can be spontaneous)
Published	01/07/2025	causes of abnormal bleeding{{c1::vascular disorders}}{{c1::platelet disorders}}{{c1::coagulation pathway disorders}}
Published	01/07/2025	inherited vascular disorders:hereditary {{c1::haemorrhagic telangiectasia}}{{c1::collagen}} abnormalities (Ehlers-Danlos syndrome)
Published	01/07/2025	acquired vascular disorders:{{c1::scurvy}} (vitamin C decifiency){{c1::steroid}} treatment (cf Cushing's syndrome)age: {{c1::old age}}
Published	01/07/2025	hereditary haemorrhagic telangiectasiaInheritance:uncommon: {{c1::autosomal dominant}}What happens?What are the symptoms?{{c1::dilated microvascu…
Published	01/07/2025	collagen/connective tissue disordersexample:{{c1::e.g. Ehler's Danlos}} syndromesymptoms:easy {{c1::bleeding and bruising}}Describe collagen/conn…
Published	01/07/2025	scurvywhat is it?acquired {{c1::vitamin C}} deficiencysymptoms:bleeding {{c1::gums}}peri-follicular {{c1::haemorrhages}}{{c1::bruises}}
Published	01/07/2025	management of scurvyask about {{c1::alcohol abuse and diet}}give {{c1::vitamin C}}
Published	01/07/2025	platelet number disorders:low platelet count AKA {{c1::thrombocytopenia: <150 x 109/L}}symptoms:{{c1::epistaxis}}{{c1::GI}} bleeds{{c1::menorr…
Published	01/07/2025	disorders of platelet numbers:-inherited vs acquiredinherited causes are {{c1::rare}}acquired causes are {{c1::common}}:Some acquired causes include:{…
Published	01/07/2025	immune (idiopathic) thrombocytopenia purpura (ITP)describe its existence in children,adults, men/womenacute in {{c1::children}}, following {{c1::infec…
Published	01/07/2025	ITP treatmentWhen do we treat ITP?treat if {{c1::bleeding}} or platelet count {{c1::<30}}Possible treatments:{{c1::steroids}}IV {{c1::immunogl…
Published	01/07/2025	inherited disorders of platelet functionBernard-Soulier syndrome: mutation in {{c1::Gp1b-IX-V}}Glanzmann thrombasthenia: mutation in {{c1::Gp2b/3a}}de…
Published	01/07/2025	acquired disorders of platelet function can be due to?due to {{c1::drugs: aspirin, clopidogrel, NSAIDS}}due to {{c1::herbal}} remediesacquired {{…
Published	01/07/2025	platelet disorders are not usually evident with screening coagulation tests, {{c1::specialist}} tests are needed
Published	01/07/2025	which test is used to screen for abnormalities in intrinsic pathway of coagulation cascade?{{c1::APTT - activated partial thromboplastin time}}
Published	01/07/2025	which test is used to screen for abnormalities in extrinsic pathway of coagulation cascade?{{c1::PT - prothrombin time}}
Published	01/07/2025	Three features of haemophilia:What does haemophilia affect?>affects body's ability to control {{c1::blood clotting}}>serious {{c1::bleeding}}&nb…
Published	01/07/2025	haemophilia A = factor {{c1::VIII}} deficiency
Published	01/07/2025	haemophilia B = factor {{c1::IX}} deficiency (christmas disease)
Published	01/07/2025	clinical features of haemophiliasponatenous {{c1::bleeding}} into joints and musclechronic debilitating {{c1::joint}} diseaseunexpected post…
Published	01/07/2025	describe the inheritance of haemophilia{{c1::X-linked recessive inheritance}}
Published	01/07/2025	blood results in haemophilia A & B{{c1::prolonged}} APTT (tests factors VIII, IX, XI, XII{{c1::normal}} PT (tests factors II,V, VII, X)l…
Published	01/07/2025	treatment for haemophilia{{c1::recombinant DNA}} to produce products{{c1::prophylactic}} infusion of recombinant factor VIII or factor IX: -…
Published	01/07/2025	von Willebrand's factor-synthesised by?-role (2)synthesised by {{c1::endothelial cells and megakaryocytes}}acts as bridging molecules between {{c1::pl…
Published	01/07/2025	blood results in von Willebrand disease{{c1::normal/prolonged}} APTT{{c1::normal}} PT{{c1::low}} vWF level{{c1::low}} factor VIII{…
Published	01/07/2025	treatment of von Willebrand's disease{{c1::desmopressin}} (DDAVP) - increases synthesis/release of VWBF{{c1::anti-fibrinolytics}} (tranexami…
Published	01/07/2025	acquired haemophilia-how do you get it?{{c1::auto-antibodies}} produced (inhibitors) to clotting factorsrenders {{c1::factor}} deficientrari…
Published	01/07/2025	what to look for in acquired haemophilia casesunderlying driver which has lead to {{c1::antibody}} formation, for example:m{{c1::alignanc}}ya{{c1…
Published	01/07/2025	treatment of acquired haemophilia{{c1::complex}} to treat:{{c1::bypassing}} agents{{c1::immunosupression}}
Published	01/07/2025	What are the effects of liver disease?defects in synthesis of {{c1::clotting factors}}some degree of {{c1::'auto-anticoagulation'}}{{c1::bleeding}}&nb…
Published	01/07/2025	Causes of vitamin K deficiency:environmental factor: {{c1::dietary}}drug: {{c1::warfarin}}
Published	01/07/2025	Which factors are dependent on vitamin K?{{c1::dependent factors: X, IX, VII, II, protein S and C}}required for {{c1::gamma carboxylation}}
Published	01/07/2025	deficiency of vitamin K causes:{{c1::fat}} malabsorptionbiliary obstruction {{c1::(jaundice)}}newborn state - {{c1::haemorrhagic}} disease o…
Published	01/07/2025	warfarin acts as: {{c1::a vitamin K antagonist}}
Published	01/07/2025	causes of disseminated intravascular coagulation{{c1::septicaemia}}: infectionacute {{c1::promyelocytic leukaemia}}{{c1::cancer, trauma, pregnanc…
Published	01/07/2025	what is disseminated intravascular coagulation?release of {{c1::tissue factor}}breakdown of balance of: {{c1::coagulation and fibrinolysis}}{{c1::clot…
Published	01/07/2025	blood results in DICprolonged blood tests: {{c1::PT, APTT}} (all factors being used up in coagulation){{c1::low}} fibrinogen{{c1::low}}&nbsp…
Published	01/07/2025	treatment of DIC:treat {{c1::underlying cause}} (e.g. antibiotics for sepsis){{c1::blood products}} to correct coagulation abnormalitiesvita…
Published	01/07/2025	The APTT can be used as a screening test for the activity of which pathway? {{c1::intrinsic pathway}}
Published	01/07/2025	What type of inheritance is seen with Hereditary Haemorrhagic Telangiectasia?{{c1::autosomal dominant}}
Published	01/07/2025	Bernard Soulier disease is due to a mutation in which molecule?{{c1::GP1b}}
Published	01/07/2025	In the absence of bleeding, when might you treat ITP?{{c1::when platelet count <30}}
Published	01/07/2025	Haemophilia A is the deficiency of which factor?{{c1::factor VIII}}
Published	01/07/2025	In addition to protein S and C, the Vitamin K dependent clotting factors are:{{c1::Factors 10, 9 ,7, 2}}
Published	01/07/2025	>hereditary haemorrhagic telangiectasia>collagen abnormalities (Ehlers-Danlos syndrome)The above are examples of?
Published	01/07/2025	The following points describe which condition? dilated microvascular swelling: in skin, mucous membranes, internal organsnose bleeds, GI bleeds
Published	01/07/2025	epistaxisGI bleedsmenorrhagiabruisingpetechiae/purpuraThe above symptoms would indicate?
Published	01/07/2025	The following are signs of?spontaneous bleeding into joints and musclechronic debilitating joint diseaseunexpected post-operative bleedingfamily histo…
Published	01/07/2025	The following are results of:prolonged APTT (tests factors VIII, IX, XI, XIInormal PT (tests factors II,V, VII, X)low factor VIII or IX levels
Published	01/07/2025	normal/prolonged APTTnormal PTlow vWF levellow factor VIIInormal/low platelet numbers/disfunctionprolonged bleeding time
Published	01/07/2025	The followings are all signs of?prolonged PT, APTT (all factors being used up in coagulationlow fibrinogenlow plateletsraised D dimersraised fibrin de…
Published	01/07/2025	von Willebrand's diseasewhat is it?Deficiency of {{c1::Von willebrand factor}} and factor {{c1::VIII}} in plasmacommonest {{c1::inherited}}&…
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