Haematology P1 Flashcards
Which factors does heparin affect?
2, 9, 10, 11
Which factors does warfarin affect?
2, 7, 9, 10
Which factors are affected in DIC?
1, 2, 5, 8, 11
Which factors are affected in liver disease?
1, 2, 5, 7, 9, 10, 11
How is APTT, PT and bleeding time affected in haemophilia?
- increased APTT
- normal PT
- normal bleeding time
How is APTT, PT and bleeding time affected in von Willebrand’s disease?
-increased APTT -normal PT -increased bleeding time
How is APTT, PT and bleeding time affected in vit K deficiency?
-increased APTT -increased PT -normal bleeding time
What is acute intermittent porphyria (AIP)
-rare autosomal dominant -defect in porphobilinogen deaminase (enzyme in synthesis of haem) -toxic accumulation of delta aminolevulinic acid and porphobilinogen -more common in women
Presentation of AIP:
-abdominal: pain and vomiting -neuro: motor neuropathy -depression -HTN and tachycardia
Diagnosis of AIP:
-urine deep red on standing -raised urinary porphobilinogen (elevated between attacks and greater during acute) -assay of red cells for porphobilinogen deaminase -raised serum levels of delta aminolevulinic acid and porphobilinogen
Complications of antiphospholipid syndrome in pregnancy:
-recurrent miscarriage -IUGR -pre-eclampsia -placental abruption -pre-term delivery -venous thromboembolism
Management of antiphospholipid syndrome in pregnancy:
-low dose aspirin once pregnancy confirmed -LMWH once foetal heart on ultrasounds (discontinue at 24 weeks)
What is aplastic anaemia and peak incidence age?
-pancytopenia and hypo plastic bone marrow -peak incidence of acquired: 30yo
Features of aplastic anaemia:
-normochromic normocytic anaemia
-leukopenia with lymphocytes relatively spared
-thrombocytopenia
-may be presenting feature of acute lymphoblastic or myeloid leukaemia
-minority later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia
Causes of aplastic anaemia:
-idiopathic
-congenital: Fanconi, dyskeratosis congenita
-drugs: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
-toxin: benezene
-infections: parvovirus, hepatitis
-radiation
What test determines AIHA?
positive direct antiglobulin test (Coomb’s test)
Warm AIHA (MOA and management)
-antibody (usually IgG) causes haemolysis at body temperature and at extravascular sites e.g. spleen
-manage with steroids, immunosuppression and splenectomy
Causes of warm AIHA:
-autoimmune disease e.g. systemic lupus erythematosus
-neoplasia e.g. lymphoma, CLL
-drugs e.g. methyldopa
Cold AIHA:
-usually Igm
-haemolysis best at 4 degrees c
-mediated by complement and intravascular
-symptoms of Raynaud’s and acrocyanosis
-respond less well to steroids
Causes of cold AIHA:
-neoplasia e.g. lymphoma
-infections e.g. mycoplasma, EBV
Beta thalassaemia major: (incl features and management)
-absence of beta chains
-chromosome 11
-presents in first year of life with failure to thrive and hepatosplenomegaly
-microcytic anaemia
-HbA2 and HbF raised
-HbA absent
-manage with repeated transfusions (iron overload)
-infusion of desferrioxamine