haem

• What is the deficiency in Haemophilia B?

  Factor IX
• What is the deficiency in Haemophilia A?

  Factor VIII
• What do antibodies target in ITP?

  Glycoprotein IIb/IIIa complex
• What condition is associated with activated protein C resistance?

  Factor V Leiden
• What causes lead poisoning?

  Defective ferrochelatase function
• What mutation is found in Polycythaemia rubra vera?

  Mutation in JAK2
• What deficiency is associated with congenital sideroblastic anaemia?

  Delta-aminolevulinate synthase-2
• What defect is seen in acute intermittent porphyria?

  Porphobilinogen deaminase
• What deficiency is associated with hereditary angioedema?

  C1-INH
• What blood film abnormalities are associated with target cells?

  • Thalassaemia
  • Sickle-cell anaemia
  • Iron-deficiency anaemia
  • Liver disease
  • Hyposplenism
• What blood film abnormality is associated with hypersegmented neutrophils?

  Megaloblastic anaemia
• What do schistocytes indicate?

  Intravascular haemolysis
• What do Howell-Jolly bodies indicate?

  Hyposplenism
• What condition is indicated by tear-drop poikilocytes?

  Myelofibrosis
• What do Heinz bodies indicate?

  G6PD deficiency
• What does basophilic stippling indicate?

  Lead poisoning
• What blood film abnormalities are associated with hyposplenism?

  • Acanthocytes
  • Siderotic granules
  • Pappenheimer bodies
  • Target cells
• What blood film abnormality is associated with pencil poikilocytes?

  Iron-deficiency anaemia
• What are the features of anaphylaxis during blood transfusion?

  • Hypotension
  • Dyspnoea
  • Wheezing
  • Angioedema
• What are the symptoms of an acute haemolytic reaction?

  Fever, abdominal pain, hypotension
• What characterizes a non-haemolytic febrile reaction?

  Fever and chills
• What are the symptoms of transfusion-related acute lung injury (TRALI)?

  Pulmonary oedema, hypotension, fever
• What are the symptoms of a minor allergic reaction during transfusion?

  Pruritus and urticaria
• What are the symptoms of transfusion-associated circulatory overload (TACO)?

  Pulmonary oedema and hypertension
• What is the management for a minor allergic reaction during transfusion?

  • Temporarily stop the transfusion
  • Administer antihistamine
  • Monitor the patient
• What is the management for anaphylaxis during transfusion?

  • Stop the transfusion
  • Administer IM adrenaline
  • Provide supportive care including oxygen
• What is the management for an acute haemolytic reaction?

  • Stop the transfusion
  • Check patient and blood product identity
  • Send blood for direct Coombs test
  • Provide supportive care including fluid resuscitation
• What is the management for TRALI?

  • Stop the transfusion
  • Provide supportive care including oxygen
• What is the management for a non-haemolytic febrile reaction?

  • Slow or stop the transfusion
  • Administer paracetamol
  • Monitor the patient
• What is the management for TACO?

  • Slow or stop the transfusion
  • Provide supportive care including oxygen
  • Administer IV furosemide
• What causes an acute haemolytic reaction?

  ABO-incompatible blood transfusion
• What causes a minor allergic reaction during transfusion?

  Foreign plasma proteins
• What can cause anaphylaxis during transfusion?

  IgA deficiency with anti-IgA antibodies
• What causes a non-haemolytic febrile reaction?

  Antibodies reacting with white cell fragments
• What causes TRALI?

  Increased vascular permeability from activated neutrophils
• What causes TACO?

  Excessive rate of transfusion
• What conditions can cause massive splenomegaly?

  • Chronic myeloid leukaemia
  • Myelofibrosis
  • Malaria
  • Visceral leishmaniasis
  • Gaucher's syndrome
• What complications are associated with chronic lymphocytic leukaemia?

  • Anaemia
  • Transformation to high-grade lymphoma
  • Hypogammaglobulinaemia
  • Warm autoimmune haemolytic anaemia
• What complication can arise from chemotherapy in Burkitt's lymphoma?

  Tumour lysis syndrome
• Which drugs may cause haemolysis in G6PD deficiency?

  Ciprofloxacin, Sulphonamides, Primaquine