haemo

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rdsmed
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227233
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haemo
Updated:
2013-07-16 05:35:25
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malignancies
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  1. What chemotherapy can be used for blood malignancies affecting the brain?
    BBB!
    • Methotrexate crosses the BBB
    • Intrathecal Chemo
    • Irradiate brain
  2. Incidence of leukaemia per 100 000?
    10
  3. Which sex is more likely to get leukaemia?
    Men
  4. China has a low incidence of which leukaemia in particular?
    CLL
  5. Is it acute or chronic leukaemia:
    Proliferation of all primitive stem cells
    Blasts accumulate?
    Acute
  6. Is it acute or chronic leukaemia:
    Malignant clonal differentiation that can't differentiate or mature
    Mature cells accumulate?
    Chronic
  7. In acute leukaemia, what percentage of peripheral blood cells are blasts?
    20%
  8. What is the incidence of AML:ALL in
    A) adults
    B) kids
    • A) 4AML:1ALL
    • B) 1AML:4ALL
  9. What are the signs of leukaemia?
    Signs of bone marrow failure

    • Infections
    • Anaemia
    • Bleeding
  10. What if there are auer rods in cells?
    Myelin lasted leukaemia
  11. What kind of anaemia is seen in acute leukaemia?
    Macrocytic anaemia
  12. What is the prognosis of acute leukaemia?
    • With treatment: 80% achieve remission
    • Without: 5 days
  13. Three stages of treatment of acute leukaemia?
    • Remission induction: chemo
    • Remission consolidation: chemo, radio, BMT
    • Remission maintenance
  14. CML is proliferation of all myeloid haematopoeitic cells but which line in particular?
    Granulocytes
  15. Age of onset of CML?
    55yr
  16. Incidence of CML per 100 000?
    1.8

    V rare!
  17. CML counts for what percentage of leukaemias?
    20%
  18. CML is caused by a part translocation of which chromosome, creating the BCR proto oncogene?
    Philadelphia chromosome from 22->9
  19. What percentage of CML are asympto?
    25%
  20. What organs/lymph nodes are enlarged in CML?
    No lymphadenopathy

    Hepatosplenomegaly (esp spleen)
  21. Symptoms of chronic leukaemia?
    • Bone marrow failure
    • Anorexia
    • Sweating
    • Lethargy
    • SOB
  22. What kind of anaemia do chronic leukaemias cause?
    • Normocytic
    • Normochromic

    -> haemolytic anaemia
  23. What is the commonest leukaemia (30% of them)?
    CLL
  24. Sex and age of onset of CLL?
    • 70yo
    • 2men:1woman
  25. Which leukaemia will have a monoclonal B cell band with CD19 and CD23 antigens and aberrant T cell antigen CD25?
    CLL
  26. Staging of CLL?
    A) 60%
    B) 30%
    C) 10%
    • A) no anaemia/thrombocytopenia and <3 lymphadenopathy
    • B) no anaemia/thrombocytopenia and >3 lymphadenopathy
    • C) anaemia or thrombocytopenia
  27. What chemo do you use for CLL?
    Alkylating agents

    • Chlorambucil
    • Cyclophosphamide with flu daring
  28. When would you do splenectomy for CLL?
    • If its massive
    • Or RBC count is v low
  29. Prognosis for stage B or CLL?
    6 yr
  30. What can be the difference between prolymphocytic leukaemia and CLL?
    • 25 % of Prolymphocytic is a variant with T cells
    • More likely to have massive splenomegaly than lymphadenopathy

    Poor prognosis
  31. What is hairy cell leukaemia?
    • Type of CLL
    • B cells
    • Neutropenia with hairy cells
    • Always splenomegaly never lymphadenopathy
  32. Sex ratios for hair cell CLL?
    6 female : 1 male
  33. What is a hairy cell?
    • B cell with
    • CD25 and CD103
    • Stains with CID phosphatase
    • Resistant to tartare staining
  34. Treatment and relative CLL prognosis of hairy cell leukaemia?
    Chemo: cladribine and deoxycoformycin

    Prog is better - yrs
  35. What is myelodysplastic syndrome?
    Clonal haematopoetic proliferation

    ->> AML
  36. Myelodysplastic syndromes are because of mutations in which 2 chromosomes?
    Name a cause
    5 or 7

    Radiation
  37. how many people in 100 000 over 70yrs have myelodysplastic syndromes?
    30
  38. what does the blood film of MDS show?
    • macrocytic dysplastic RBC anaemia
    • hypogranular neutropenia
    • +/- blasts in 20%
  39. treatment for MDS?
    • NO CURE
    • give RBC and PLT infusions, G-CSF and epo
  40. what is RARS?
    refractory anaemia with ringed sideroblasts
  41. what is a sideroblast?
    a nucleated RBC that cannot encorporate it's iron into Hb so it's granulated in a ring around the nucleus
  42. what is 5q syndrome?
    • 5q long arm deletion that leads to MDS
    • occurs in females with age
  43. RA
    RARS
    RCDS
    RAEB
    5q 
    are all classed as what syndromes
    MDS
  44. what is lymphoma?
    lymphoid proliferative
  45. what is Hodgekin's lymphoma?
    • B cell lymphoma:
    • with Reed Steinberg B cells surrounded by:
    • nornal T cells
    • plasma cells
    • eosinophils
  46. how many people per 100 000 get lymphoma?
    4
  47. what is the sex ration of lymphoma?
    age of onset?
    class?
    • 1.5 male: 1 female
    • 30yo
    • middle class
  48. what viruse is assoc in the PMH of lymphoma?
    EBV (mono)
  49. what is the commonest type of hodgekin's lymphoma?
    • nodular sclerosing
    • (classical type)
  50. what causes painless, rubbery cervical lymphadenopathy?
    hodgekin's lymphoma
  51. what causes a dry cough and SOB in hodgekin's lymphoma?
    mediastinal lymphadenopathy
  52. treatment for hodgekin's lymphoma?
    • radio
    • chemo: doxy, bleo, vinblastin, dacarbazine
    • autologous BMT
  53. prognosis of hodgekin's lymphoma?
    90%
  54. what is the Ann Arbor classification of HL?
    • I: HL with one other site or one node involved
    • II: HL with 2 other sites or nodes on the same side of diaphragm
    • III: nodes on both sides of diaphragm involved
    • IV: diffuse involvement of nodes
  55. what does A or B mean in classes of lymphoma?
    • A - no systemic symptoms
    • B - weight loss, drenching sweats
  56. what cell types can non-hodgekin's lymphoma be?
    • 70% are B cell
    • 300% are T cell
  57. what is the incidence of non-hodgekin's lymphoma in 100 000 over 75 yrs?
    63
  58. which type of non-hodgekin lymphoma is potentially curable: high or low grade?
    high grade
  59. treatment for high grade nonhodgekin lymphoma?
    • chemo: cyclophos, doxorubicin, vincristine, pred
    • radio
    • rituximab
    • autologous BMT

    (underlined is different to HL)
  60. treatment for low grade non hodgekin lymphoma?
    NO CURE

    • radio if stage 1 ann arbor
    • chemo: chlorambucil
    • rituximab
    • BMT
  61. what is non hodgekin lymphoma assoc with?
    • HIV and it's co infections
    • gastric lymphoma is assoc with h. pylori
    • immunodeficiency
  62. tests for leukaemia?
    • bloods
    • blood film
    • bone marrow aspirate
    • immunophenotyping for leukocytes antigens
    • uric acid level
    • HIV
  63. which is likely to be widely disseminated at presentation?
    HL
    non hodgekin lymphoma
    non hodgekin lymphoma
  64. which is likely to present with weight loss, sweats, itching?
    non hodgekin lymphoma
  65. low grade lymphoma survival?
    10yrs of relapsing/remitting
  66. what is paraproteinaemia?
    = gammopathy

    • clonal proliferation of antibodies¬†
    • can occur without underlying disease
  67. how do you detect paraproteinaemias?
    immunoelectrophoresis
  68. name some causes of polyclonal paraproteinaemia?
    • inflam
    • sarcoid
    • infection
    • autoimmune
  69. name some causes of monoclonal paraproteinaemias?
    • myeloma
    • lymphoma
    • RA
    • HIV
    • polymyalgia rheumatica
  70. what is MGUS?
    monoclonal gammopathy of uncertain significance

    aka benign monoclonal gammopathy
  71. what percentage of those over 80yrs have MGUS?
    why do you monitor these people?
    5%

    monitor because 20% will get myeloma
  72. MGUS is assoc with which malignancy?
    myeloma
  73. what is waldenstrom macroglobulinaemia?
    • lymphoplasma cytoid lymphoma
    • IgM paraprotein
    • low grade
  74. what is the treatment for waldenstrom macroglobulinaemia?
    • plasmaphoresis of IgM to reduce blood viscosity
    • alkylating chemo eg chlorabucil
    • rituximab
  75. how many people per 100 000 get multiple myeloma?
    4
  76. what is the sex ratio and the age of onset of multiple myeloma?
    which race does it affect the most?
    • 70yo
    • 2 male:1 female
    • afrocaribbean race
  77. what is multiple myeloma?
    • malignant proliferation of
    • B cells (derived from plasma cells)
  78. what monoclonal antibodies do myeloma B cells produce?
    (normal B cells produce polyclonal Abs)
    • 55% IgG
    • 21% IgA
    • 20% no paraproteinaemia
  79. other than paraproteinaemia, what do myeloma B cells produce?
    light chains
  80. what is Bence Jones proteinuria?
    when myeloma light chains are in the urine

    they are nephrotoxic!
  81. why is there bone lysis in myeloma?
    • myeloma B cells live in bone
    • cause increase osteoclast action
    • therefore bone resorption increases
  82. clinical picture of mutliple myeloma?
    • bone pain from lysis
    • hypercalcaemia
    • fractures
    • bone marrow failure symptoms
  83. diagnosis of multiple myeloma needs 2 of what 3 things?
    • malignant plasma cells in bone marrow
    • serum/urinary paraproteinaemia
    • skeletal lytic lesions
  84. which LFT is raised in multiple myeloma?
    ALP
  85. what is the 10yr survival rate for multiple myeloma?
    5%
  86. amyloidosis and myeloma?
    linked!

    amyloidosis is due to deposits of light chains
  87. name some causes of renal failure associated with myeloma?
    • bence jones proteinuria
    • NSAIDs
    • amyloid
    • hypercalcaemia
  88. what is urate nephropathy?
    uric acid crystals block renal tubules

    • seen in leukaemia due to tumour cell lysis (myeloma) and chemo
    • or gout
  89. treatment for hyperuricaemia?
    allopurinol
  90. why give GCs in myeloma?
    they reduce antibody production
  91. what chemo is used in myeloma?
    • thalidomide
    • alkylating agents
  92. treatment for bone lysis pain in myeloma?
    • radiotherapy
    • bisphosphonates¬†
    • dental review - jaw osteocronosis
  93. what causes failure of pluripotent stem cells and thus bone marrow hypoplasia and pancytopenia?
    primary idiopathic acquired apalastic anaemia
  94. where in the world is idiopathic acquired aplastic anaemia commonest?
    asia

    rare in europre
  95. what kind of anaemia is seen in aplastic anaemia?
    • macrocytic
    • low reticulocyte count
  96. what is a cure for young people with aplastic anaemia?
    • allogenic BMT
    • 90% if matched
  97. what percentage of people with aplastic anaemia die in the first year?
    50%
  98. complications of aplastic anaemia?
    • PNH
    • MDS
    • AML
  99. treatment for older people with acquired aplastic anaemia?
    • cyclosporin immunosuppression
    • anti-thymocyte globulin
  100. commonest cause of secondary aplastic anaemia?
    drugs
  101. name three types of myeloproliferative disorders?
    • mylofibrosis
    • essential thrombocytopenia
    • polycaethaemia ruba vera (PRV)
  102. age of onset of:
    a) PRV
    b) myelofibrosis
    c) essential thrombocythaemia?
    • a) 40yo
    • b) >50yo
    • c) 60yo
  103. what happens in myeolofibrosis?
    • hypercellular bone marrow
    • increased megakaryoctes -> increased GF
    • -> inc fibroblasts
  104. symptoms of myelofibrosis?
    • lassitude
    • weight loss
    • night sweats
    • splenomeg - painful due to infarcts
  105. prognosis of myelofibrosis?
    4 yr survival
  106. treatment for myelofibrosis?
    • RBCs for anaemia
    • folic acid
    • cytotoxics: hydroxycarbamide
    • splenectomy
    • BMT
  107. what gene mutation is assoc with myeloproliferative disease?
    • JAK2
    • chromosome 9
  108. what causes essential thrombocytopenia?
    • prolif of megakaryoctes
    • JAK2 mutation
    • risk factors: DM, HTN
  109. treatment for essential thrombocythaemia?
    hydroxycarbamide/anagrelide to stop megakaryocyte maturation

    radioactive phosphorus

    aspirin
  110. what is the PLT count like in essential thrombocythaemia?
    >1000
  111. how do you diagnose PRV?
    • haematocrit increases
    • JAK2
  112. how do you measure plasma vol?
    radio-labelled iodine
  113. what is the prognosis of PRV?
    what could it lead to?
    complications?
    • 65% -> myelofibrosis
    • also: MI, CVA

    10yr survival

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