blood and immunity

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jaz_walker
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274584
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blood and immunity
Updated:
2014-05-16 12:05:43
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pathology
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pathology
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pathology
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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 removed.you 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?
    phenytoin-
  16. 16. whiy hypoglycaemic causes neutropaenia?
    tolbutamide-
  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?
    8:14-
  20. 20. Which gene translocation is associated with follicular b cell lymphoma?
    14:18-
  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?
    Increased-
  24. 24. Which organism is associated with adult T cell leukaemia?
    HTLV-1-
  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?
    myelofibrosis-
  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?
    leukaemia-
  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?
    anisocytosis-
  39. 39. what is the word for abnormalities of red cell shape?
    poikilocytosis-
  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?
    jejunum-
  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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