Haematology Flashcards

1
Q

what are the features of anaemia?

A

fatigue, fainting, breathless, angina, intermittant claudication, pale skin and mucous membraines, tachycardic, cardiac failure, systolic flow murmer

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2
Q

Name some causes of MCV low anaemia

A

iron deficiency, chronic disease, thallasaemia

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3
Q

name some causes of MCV normal anaemia

A

acute blood loss, chronic disease, iron and folate deficiency

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4
Q

name some causes of MCV high anaemia

A

B12/folate deficiency, alcohol, liver disease, hypothyroid

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5
Q

causes of iron deficiency

A

blood loss, increased demand (growth/pregnancy), reduced absorption (coeliac/bowel dis), reduced intake

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6
Q

what Ix do you do for anaemia?

A
blood count and film
serum ferritin
serum iron and TIBC
serum transferrin
BM exam
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7
Q

how do you manage iron deficiency anaemia?

A

ferrous sulphate

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8
Q

causes of B12 deficiency?

A

vegans, pernicious anaemia, gastrectomy, crohns disease, coeliac

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9
Q

how is B12 absorbed?

A

binds to intrinsic factor which is released by gastric parietal cells and then absorbed in the ileum and stored in the liver

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10
Q

what is pernicious anaemia?

A

autoimmune condition with atrophic gastritis with loss of parietal cells

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11
Q

where do we find folate in our diet?

A

green vegetables, liver and kidney

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12
Q

name some causes of folate deficiency

A

damage to upper intestine (where it is absorbed) - crohns/coeliac
age, poverty, alcohol, pregnancy, lactation, methotrexate

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13
Q

what is aplastic anaemia?

A

BM failure, pancytopenia (deficiency of all cell elements of the blood and BM aplasia)

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14
Q

What is haemolytic anaemia?

A

destruction of RBCs with reduced circulating lifespan of 120 days. compensatory increase in BM activity with release of immature red cells

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15
Q

causes of haemolytic anaemia?

A

hereditary spherocytosis/elliptocytosis, thalassaemia, sickle cell, G6PD deficiency, autoantibodies, malaria

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16
Q

how can you manage haemolytic anaemia?

A

splenectomy

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17
Q

what are A and B thallasaemia?

A
A = reduced A chain synthesis
B = reduced B chain synthesis

Disorder in the gene for the chain = reduced rate of production of one or more globin chains

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18
Q

name some features of thallasaemia?

A

MINOR - genetic carrier state
INTERMEDIA - moderate anaemia, splenomegaly, bone deformities, leg ulcers, gallstones
MAJOR - severe anaemia, failure to thrive, recurrent infections, enlarged maxilla, hepatosplenomegaly

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19
Q

How do you manage thalassaemia? what needs to be given in addition to this Mx?

A

blood transfusions

give iron chelating agents too (desferrioxamine)

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20
Q

what is sickle cell?

A

autosomal recessive condition. HbS is a different shape when deoxygenated –> becomes insoluble and polymerises which increases rigidity of cells/sickle appearance/premature destruction and obstruction

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21
Q

what precipitates a sickle crisis?

A

hypoxia, dehydration, infection, cold

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22
Q

Features of sickle cell?

A

child is normal until until 6 months old (due to HbF)
OCCLUSION - acute hand and feet pain, avascular necrosis of the BM, bone pain in the long bones

ANAEMIA - enlarged spleen, BM aplasia, increased haemolysis with drugs/infection

LONG TERM - deformed long bones, splenic atrophy, retinal ischaemia, cerebral infarction

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23
Q

how do you Mx sickle cell?

A

avoid precipitating factors, folic acid supplements

24
Q

How do you Mx a sickle cell crisis?

A

paracetamol/NSAIDS

25
Q

complications of sickle cell?

A

pain, swollen and painful joints, acute sickle chest, CNS deficit, pigment gallstones

26
Q

what is autoimmune haemolytic anaemia and how do we diagnose it?

A

immune destruction of RBCs - we diagnose it with the COOMB’s TEST - tests for antibodies/complement on the surface of RBCs

27
Q

how do we manage autoimmune haemolytic anaemia?

A

prednisolone, splenectomy

28
Q

what causes polycythaemia?

A

increased EPO production produced by kidneys when they have signs of hypoxia

29
Q

what is the difference between primary and secondary polycythaemia?

A

primary - mutation on polycythaemia gene (JAK2)

secondary - due to smoking/hypoxia

30
Q

how do you investigate polycythaemia and what do the results show?

A

blood count (raised RBCs/raised PCV/Raised Hb)

31
Q

what are the symptoms of polycythaemia?

A

headache, dizzy, tinnitus, visual changes, angina, intermittant claudication, DVT

32
Q

how do you manage polycythaemia?

A

stop smoking/hypoxia (give O2)

venesection, low dose aspirin

33
Q

what is thrombocytopenia?

A

low levels of platelets

34
Q

what are some causes of impaired production of platelets (BM)

A

BM failure, megaloblastic anaemia, leukaemia, myelofibrosis, HIV infection

35
Q

name some causes of excessive platelet destruction?

A

autoimmune - ITP
SLE
post transfusion
DIC

36
Q

what is the main cause of ITP in children?

A

post viral

37
Q

what do you find in the bloods?

A

platelet autoantibodies

38
Q

management of ITP?

A

corticosteroids, IVIG, splenectomy

39
Q

features of ITP?

A

routine bloods
petichae, purpura
bleeding (e.g. epistaxis)
catastrophic bleeding (e.g. intracranial) is not a common presentation

40
Q

what is TTP?

A

widespread adhesion and aggregation of platelets - microvascular thrombosis and profound thrombocytopenia

41
Q

causes of TTP?

A

pregnancy, SLE, infection, cancer

42
Q

features of TTP?

A

purpura, fever, fluctuating cerebral dysfunction, haemolytic anaemia

43
Q

management of TTP?

A

plasma exchange

44
Q

what is Haemophilia A?

A

deficiency in factor 8, X linked recessive disease

45
Q

features of haemophilia A?

A

depends on how low the plasma factor 8 levels are
SEVERE - spontaneous muscle/joint bleeds (arthropathy)
MOD - severe bleeding following injury
MILD - bleeding with trauma/surgery

46
Q

Ix for haemophilia A and the results?

A

prolonged APTT, reduced factor 8 plasma levels

47
Q

Mx of Haemophilia A?

A

IV factor 8 recombinant as prophylaxis pre surgery
hep A/B vaccinations
avoid contact sport

48
Q

how are the haemophilias inherited?

A

x linked recessive

49
Q

what is haemophilia B?

A

deficiency in factor 9

50
Q

what is von Willebrand disease?

A

vWF deficiency leading to defective platelet function and factor 8 deficiency

51
Q

features of vonWB disease?

A

mucosal bleeding - GI/nose bleeds

prolonged bleeding after dental work

52
Q

what is shown on investigation of vWB disease?

A

prolonged bleeding time - due to defective platelet adhesion

53
Q

Mx for vWB disease?

A

factor 8 concentrate

54
Q

which clotting factors is vitamin K required for production of?

A

2, 7, 9, 10

55
Q

what is DIC?

A

widespread fibrin generation within blood vessels caused by coagulation pathway initiation.

  • consumption of platelets and coagulation factors
  • secondary activation of fibrinoysis
56
Q

name some causes of DIC?

A

sepsis, major trauma, tissue destruction, malignancy

57
Q

Features of DIC?

A

complete haemostatic failure, thrombotic events