blood and immunity

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  1. 1. A 72 year old man presents with pedal oedema and a cough. On examinatino there is macroglossia and carpel tunnel syndrome.You hear lung crackles. You note proteinurea.What could this be?
    AL amyloidosis-
  2. 2. a 76 year old man has his spleen note the cut surface has a purple red colour, with flecked firm brown nodules. On microscopy, there is ectasis of the sinusoids.what is the cause of this?
    congestive splenomegaly-
  3. 3. A 68 year old male presents with renal impairmen. Serum shows raised IgM, which is monoclonal.What is this likely to be?
    waldenstrom's macroglobulinaemia-
  4. 4. A male child presents with recurrent infections between the ages of 4 months and 3 years of age.They have deficiency of B cells and plasma cells and immunoglobulins. Their tonsils are not palpable.What might you consider?
    X linked agammaglobulinaemia of Bruton-
  5. 5. A 9 month old child is diagnosed with Kostmann's syndrome.What might you expect them to present with?
    life-threatening infection- neutropenia
  6. 6. A 45 year old male presents with Addison's disease.what other autoimmune disease might you consider in them?
    Autoimmune gastritis-
  7. 7. a neonate has a cleft lip. On x ray you note the thymus is completely absent.which syndrome might you consider?
    diGeorge syndrome-
  8. 8. A 58 year old woman presents with a stroke. On bloods, you note thrombocytopaenia. No splenomegaly. Petechiae.What is this?
    Thrombotic thrombocytopaenic purpura.-
  9. 9. In neonates, when is there a natural trough in antibody levels and in which order do they appear?
    3-6 months- IgM, IgG, IgA
  10. 10. What is the commonest form of immunodeficiency, and what 2 kinds of infections are more common?
    IgA deficiency- Sinopulmonary infection, GI (giardia, salmonella etc)
  11. 11. Which congenital arches are not developed in digeorge and which 2 organs are absent?
    3rd and 4th branchial arches- thymus, parathyroid
  12. 12. What 5 conditions may precipitate reactive systemic amyloidosis and what kind of protein is involved?
    RA, Chronic infection, hodgkin's, IBD, bronchiectesis- serum amyloid A (an acute phase protein)
  13. 13. What 4 conditions might precipitate AL amyloidosis and what protein is it composed of?
    multiple myeloma, waldenstrom's macroglobulinaemia, heavy chain disease, primary amyloidosis- immunoglobulin light chains.
  14. 14. What is Felty's syndrome?
    RA with neutropaenia-
  15. 15. Which anticonvulsant can cause neutropaenia?
  16. 16. whiy hypoglycaemic causes neutropaenia?
  17. 17. which 3 infections can cause monocytosis most commonly?
    tuberculosis, rickettsiosis, malaria-
  18. 18. True or false, in Hodgkin's lymphoma the lymph node capsule is rarely breached.
    T - whereas with non-Hodgkin's this is not the case-
  19. 19. which gene translocation is associated with burkitt's lymphoma?
  20. 20. Which gene translocation is associated with follicular b cell lymphoma?
  21. 21. what is the most common type of non Hodgkin's lymphoma?
    B cell lymphoma-
  22. 22. True or false, extranodal presentation is more frequent in Hodgkin's lymphoma than non-Hodgkin's.
    F - the opposite is true.-
  23. 23. How does Down's syndrome affect your chances of leukaemia?
  24. 24. Which organism is associated with adult T cell leukaemia?
  25. 25. how is myelodysplasia defined?
    neoplastic disorders of the bone marrow. Increasing bone marrow failure. Quantitative and qualitative abnormalities of all 3 myeloid cell lines.-
  26. 26. which age and gender are usually affected by hairy cells?
    peak incidence 40-60 and males.-
  27. 27. how is myeloproliferative disorder defined?
    autonomous proliferations of one or more myeloid cells?-
  28. 28. what is the main complication of polycythaemia rubra vera?
  29. 29. how is myelofibrosis defined?
    proliferation of fibroblasts in the bone marrow. Corresponding extra-medullary haemopoiesis in the liver and spleen-
  30. 30. What is the main complication of myelofibrosis?
  31. 31. true or false, spleen can be reduced in size in primary thrombocythaemia?
    T - often dreduced due to thromboembolic infarction-
  32. 32. What is the paraprotein in multiple myeloma?
    Known as M component, usually IgG-
  33. 33. In what 3 ways does multiple myeloma damage the kidneys?
    Light chains produce amyloid, raised blood uric acid and hypercalcaemia-
  34. 34. which disease are rouleaux in the blood associated with?
    multiple myeloma-
  35. 35. you note hypersplenism on x ray. What compensatory mechanism might take place as a consequence?
    bone marrow hyperplasia-
  36. 36. which infectious causes can cause splenomegaly?
    typhoid, ebv, malaria etc-
  37. 37. which condition may result in thymic hyperplasia?
    myasthenia gravis-
  38. 38. what is the word for abnormalities of red cell size?
  39. 39. what is the word for abnormalities of red cell shape?
  40. 40. what conditions can cause microcytes?
    iron deficiency, thalassaemia-
  41. 41. what conditions cause round macrocytes?
    liver disease, alcohol, hypothyroidism-
  42. 42. what conditions cause pencil cells?
    iron deficiency-
  43. 43. what conditions cause target cells?
    iron deficiency, megaloblastic anaemia, haemoglobinopathy, liver diseae ,hyposplenism-
  44. 44. what conditions cause microspherocytes?
    heriditary spherocytosis, immune haemolytic anaemia, burns-
  45. 45. what conditions cause tear drop cells?
    myelofibrosis, marrow infiltration-
  46. 46. what conditions cause schistocytes?
    microangiopathic haemolytic anaemia-
  47. 47. what 2 groups of conditions cause macrocytic anaemia?
    megaloblastic (e.g. vit b12, folate) or haemolytic anaemia (reticulocytes)-
  48. 48. what is the commonest cause of iron deficiency anaemia?
    chronic blood loss - think GI loss or menorrhagia-
  49. 49. what are the 2 commonest defects of red cell membranes?
    heriditary spherocytosis, hereditary elliptocytosis-
  50. 50. what are the 2 commonest defects of red cell enzymes?
    glycolytic enzymes or hexose monophosphate shunt enzymes-
  51. 51. what are the 2 commonest haemoglobinopathies?
    thalassaemias, sickle cell disease-
  52. 52. what is the commonest cause of hereditary haemolytic anaemia in the UK and what protein is affected?
    hereditary spherocytosis - spectrin-
  53. 53. What is the main pathway of glucose production in red blood cells?
    Anaerobic metabolism to lactate via the Embden-Meyerhof glycolytic pathway-
  54. 54. what is glutathione required for in red blood cells and how is it produced/
    Hexose monophosphate shunt produces NADPH. It is required for maintaining haemoglobin in the reduced (ferrous) state-
  55. 55. An infant has congenital anaemia and raised unconjugated bilirubin. Blood film shows poikilocytosis and 'prickle cells'. What is this?
    Pyruvate kinase deficiency.-
  56. 56. What are bite and blister cells and Heinz bodies characteristic for?
    G6PD deficiency.-
  57. 57. Which protein is abnormal in sickle cell disease?
    B haemoglobin. (glutamate is substituted to valine)-
  58. 58. What kind of immunoglobulin is involved in warm autoimmune anaemia and where are cells destroyed?
    IgG - cells are destroyed in the spleen.-
  59. 59. what kind of immunoglobulin is involved in cold autoimmune anaemia?
    Igm - cells are destroyed by Kupffer cells in the liver.-
  60. 60. A handover sheet has 'microangiopathic haemolytic anaemia' jotted next to a patinet. Which 3 conditions might you consider?
    DIC, HUS, TTP-
  61. 61. What are 3 causes of cold autoimmune haemolytic anaemia?
    lymphoma, Ebv, mycoplasma pneumonia-
  62. 62. What are some causes of warm autoimmune haemolytic anaemia?
    CLL, SLE, drugs e.g. penicillin-
  63. 63. in which part of the gut is b12 mainly absorbed?
    terminal ileum, binding to IF.-
  64. 64. In which part of the gut is folic acid mainly absorbed?
  65. 65. In which part of the gut is iron mainly absorbed?
    duodenum and upper jejunum-
  66. 66. what is the commonest cause of thrombocytopenia?
    decreased production - due to aplastic anaemia, marrow infiltration or megaloblastic anaemia-
  67. 67. A patient presents with thrombocytopaenia and splenomegaly. What is the main cause for this?
    Autoimmune thrombocytopenic purpura - due to measles, CLL, SLE, drugs-
  68. 68. What is Bernard-Soulier syndrome?
    Rare platelet disease, causing life threatening haemorrhages. Deficiency glycoprotein receptors for binding von Willebrand's factor.-
  69. 69. What is Glanzmann's disease?
    Failure of membrane receptors for fibrinogen binding on platelets.-
  70. 70. How does aspirin cause defective platelet function?
    Irreversibly binds COX, impairing thromboxane A2 synthesis.-
  71. 71. How does uraemia affect platelet clotting?
    Increased bleeding, as abnormal arachidonate metabolism causes reduced synthesis of thromboxane.-
  72. 72. how is von Willebrand's disease inherited?
    autosomal dominant-
  73. 73. how are haemophilia A and haemophilia B inherited?
    X linked-
  74. 74. what are the 2 functions of von Willebrand factor?
    promotes platelet adhesion. Carries factor 8, protecting it from destruction.-
  75. 75. which factors are reliant on protein k?
    2,7,9,10, proteins c + s-
  76. 76. What malignancies can involve DIC?
    pancreatic, lung, prostate, acute promyelocytic leukaemia-
  77. 77. which obstetric complications result in DIC?
    amniotic fluid embolism, placental abruption, retained dead fetus-
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blood and immunity
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