Haematology 3 Flashcards

1
Q

what is myelofibrosis

A

clonal proliferation of abnormal megakaryocytes that release fibroblast stimulating factors e.g. PDGF (platelet derived growth factor) – myelofibrosis

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2
Q

symptoms of myelofibrosis

A

MASSIVE SPLENOMEGALY- extra medullary haemopoiesis
hyper metabolism - weight loss, fever, sweats, fatigue
anaemia, infections, bleeding

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3
Q

RBCs appearance in myelofibrosis

A

tear drop shaped RBCs

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4
Q

blood film myelofibrosis

A

leukoerythroblastic

  • immature RBC/WBC precursors
  • tear drop shaped RBCs
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5
Q

invesigations for myelofibrosis

A

trephine biopsy

blood film

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6
Q

tx myelofibrosis

A

transfusions for anaemia

allopurinol for high uric acid

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7
Q

what drug can cause increased neutrophils

A

steroids – disrupts margination + causes redistribution

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8
Q

mechanism of heparin

A

potentiates antithrombin

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9
Q

side effects of heparin

A

bleeding
HITT
osteoporosis

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10
Q

antidote for heparin

A

protamine sulphate

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11
Q

mechanism of warfarin

A

inhibits vitamin K

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12
Q

where is warfarin metabolised

A

liver

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13
Q

What is INR?

A

standardised PT time

too high = risk of bleeding

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14
Q

management if INR 4-8 but no bleeding

A

stop warfarin until INR <5

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15
Q

management if INR >3 + minor bleed

A

stop warfarin

1-2 mg oral vit K

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16
Q

management if big bleed on warfarin

A

stop warfarin
give vitamin K
give fresh frozen plasma or prothrombin concentrate

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17
Q

mechanism of dabigatran

A

thrombin inhibitor

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18
Q

mechanism of clopidogrel

A

ADP receptor antagonist

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19
Q

mechanism of rivaroxiban

A

factor Xa inhibitor

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20
Q

immediate ABO incompatibility reaction

A

within minutes
pyrexia, riggers, dyspnoea, hypotension
dark urine - intravascular haemolysis

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21
Q

delayed ABO incompatibility reaction

A

slower onset
less severe symptoms
Hb falls, jaundice, renal failure

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22
Q

elderly patient breathless after transfusion

- most likely?

A

circulatory overload

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23
Q

fever after transfusion otherwise well

- most likely?

A

febrile reaction

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24
Q

younger patient, breathless after transfusion otherwise well

- most likely?

A

acute lung injury

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25
Q

bacterial transfusion reaction

A

fever, shock, chills, vomiting, tachycardia, hypotension

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26
Q

which blood group can receive from any donor

A

AB

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27
Q

which blood group can be given to anyone

A

O

28
Q

how long does a sickle cell survive

A

10-20 days

29
Q

what virus can induce a aplastic anaemia in sickle cell patients

A

parvovirus B19

30
Q

how does hydroxycarbamide affect HbF

A

increases it

31
Q

findings in transfusion overload

A

hypertension
raised JVP
S3 may be present

32
Q

apotransferrin

A

unbound transferrin

33
Q

holotransferrin

A

transferrin bound to iron

34
Q

what transports iron

  • into duodenal enterocyte
  • out of duodenal enterocyte
A

Into - DMT

Out of- Ferroportin

35
Q

blood results in hereditary haemochromatosis

A

Increased serum ferritin
Increased transferrin saturation
Increased LFTs

36
Q

tx hereditary haemochromatosis

A

weekly venesection

37
Q

where is iron absorbed

A

duodenum

38
Q

what down regulates ferroportin to decrease iron uptake

A

hepcidin

39
Q

how do RBCs synthesise ATP

A
anaerobic glycolysis 
(no mitochondria so can't do aerobic)
40
Q

lifespan of normal RBC

A

120 days

41
Q

examples of chemo drugs that are specific to the cell cycle

A

methotrexate

hydroxyurea

42
Q

what does methotrexate inhibit

A

dihydrofolate reductase

43
Q

what does hydroxyurea impair

A

deoxynucleotide synthesis

44
Q

side effect of cis-platinum

A

nephrotoxicity

45
Q

side effect of anthracyclines

A

cardiotoxicity

46
Q

what does ritiximab target

A

CD20 B cells

47
Q

what is fanconi’s anaemia

A

inherited bone marrow failure
- unable to correct DNA damage

thrombocytopenia, neutrophilic, anaemia
short stature, cafe au lait spots, GI/cardio/renal defects

48
Q

Ix fanconis anaemia

A

chromosome fragility

49
Q

what is the bone marrow like in aplastic anaemia

A

hypocellular

50
Q

what is aplastic anaemia

A

autoimmune attack of stem cells causing marrow failure

can be congenital (fanconis), infection (EBV), drugs (methotrexate), toxins (benzene)

51
Q

what is the bone marrow like in myelodysplasia / megaloblastic anaemia

A

hypercellular

- bm producing new cells but they are deformed/underdeveloped – packed full of immature cells

52
Q

how can splenomegaly cause pancytopenia

A

increased destruction of RBCs

haemodilution due to increase plasma volume therefore relative decreased in WBCs + platelets

53
Q

symptoms of pancytopenia

A

thrombocytopenia == easy bruising, bleeding gums, epistaxis
anaemia == SOB, fatigue, dizziness, palpitations, pale
neutropenia == frequent infections

54
Q

macro-ovalocytes

A

B12 deficiency/pernicious anaemia

55
Q

what mitochondrial process needs iron

A

ATP production

56
Q

tx pernicious anaemia

A

lifelong B12 injections

57
Q

what drugs can increase risk of venous thromboembolism

A

tamoxifen
COC
olanzapine
HRT

58
Q

What is post thrombotic syndrome

A

complication following DVT

  • leg heaviness
  • itching
  • swelling
  • ulceration
59
Q

what virus can cause sequestration crisis in sickle cell + hereditary spherocytosis patients

A

parvovirus B19

60
Q

drugs that can cause haemolysis in G6PD patients

A

nitrofurantoin
ciprofloxacin
sulphonyureas

61
Q

how do blood transfusions affect potassium

A

risk of hyperkalaemia

62
Q

what are irradiated blood products

A

depleted T lymphocytes

- reduces graft v host disease

63
Q

how is heparin induced bleeding reversed

A

protamine sulphate

64
Q

what is given to replace fibrinogen

A

cryoprecipitate

65
Q

tx von villebrands disease

A

tranexamic acid

desmopressin

66
Q

tx ITP

A

1st line - steroids

2nd line - splenectomy

67
Q

do you treat B12 or folate first

A

b12- prevents subacute degeneration of spinal cord