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Third Year Exam > Haem > Flashcards

Haem Flashcards

1
Q

sdefine ideroblastic anaemia

A

red cells fail to completely form haem

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2
Q

siderblastic anaemia causes

A

congenital: x-linked, rec or dom
acquired: drug/toxin related (alcoholism, lead, isoniazid), myelodysplastic syndromes, RA, carcinomas

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3
Q

sideoblastic anaemia blood results

A

increased ferritin
increased iron
increased transferrin

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4
Q

sideroblastic anaemia treatment

A

treat underlying

pyridoxine may help

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5
Q

thalassaemia inheritance

A

autosomal recessive

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6
Q

thalassamia definniton

A

abnormal haemaglobin production

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7
Q

a thalassaemia presentation

A

jaundice
fatigue
facial bone deformities

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8
Q

b thalassaemia presentation

A

severe symptomatic anaemia at 3-6 months age
frontal bossing
maxillary overgrowth
hepatosplenomegaly

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9
Q

a thalassaemia management

A

blood transfusions
stem cell transplant
splenectomy

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10
Q

b thalassamia management

A

regular blood transfusions

iron celating agents to reduce risk of iron overload

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11
Q

anaemia of chronic disease blood results

A

+/- feritin
decreased iron
decreased TIBC
decreased transferrin

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12
Q

aplastic anaemia blood results

A

anaemia
low plts
low neutrophils

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13
Q

primary aplastic anaemia causes

A

fanconis anaemia

idiopathic

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14
Q

secondary aplastic anaemia causes

A

drugs (NSAIDs, chloramphenicol), infection, PNH

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15
Q

aplastic anaemia features

A

anaemia
infeciton
easy bruising

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16
Q

aplastic anaemia management

A

any susp of infection - broad spec anti-bs

severe: stem cell transplant (<40)
severe: immunosupression (>40)

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17
Q

intravascular haemolytic anaemia causes

A

G6PD
autoimmine
acute transf reaction
mechanical injury

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18
Q

extravascular haemolytic anaemia causes

A

sickle cell

spherocytosis

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19
Q

G6PD inheritance

A

x linked

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20
Q

G6PD triggers

A

fava beans
illness
henna

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21
Q

G6PD characteristic features

A

bite and blister cells
heinz bodies
dark urine

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22
Q

G6PD causes

A

anti-malarials (primaquine)
ciprafloxacin
sulph-group drugs

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23
Q

autoimmune warm type mediated by

A

IgG

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24
Q

warm type autoimmune HA management

A

steroids +/- rtiux

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25
Q

cold type autoimmune HA mediated

A

IgM

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26
Q

hereditary spherocytosis inheritance

A

autosomal dominant

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27
Q

hereditary spherocytosis presentaiton

A

neonatal jaundice
gallstones
splenomlegaly

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28
Q

hereditary spherocytosis diagnosis

A

EMA binding + cryohaemolysis test

electrophoresis analysis

29
Q

hereditary spherocytosis management

A

acute: supportive, transfuse if necessary

long term: folate, splenectomy (if less than 5yo = curative)

30
Q

sickle cell disease definition

A

genetic condition where normal haemoglobin has a tendency to form abnormal haemoglobin molecules upon deoxygenisation

31
Q

sickle cell disease inheritance

A

autosomal recessive

glutamic acid replaced by valine on 6h position of B chain

32
Q

sickle cell blood film

A

sickle cells
target cells
howel joly (hyposlen)

33
Q

sickle cell testing

A

haemoglobin electrophoresis +/- genetic testing

34
Q

long term sickle cell treatment

A

hydroxyurea
vaccines
folic acid

35
Q

screening of primary haemostatic failure

A

platelet count

36
Q

extrinsic pathway factors

A

TF/VIIa

37
Q

extrinsic pathway tets

A

PT

38
Q

intrinsic pathway factors

A

VIII/IXa

39
Q

intrinsic pathway test

A

APTT

40
Q

VWD inheritance

A

autosomal dominant

41
Q

VWD stablises clotting factor

A

VIII

42
Q

VWD clotting result

A

prolonged bleeding time

may be prolonged APTT

43
Q

VWD management

A

TXA
desmopressin
factor VIII conc

44
Q

haemophilia inheritance

A

X-linked

45
Q

haemophilia A def

A

VIII

46
Q

haemophilia B def

A

IX

47
Q

haemophilia blood results

A

increased APTT

48
Q

haemophilia investigations

A

factor VIII and IX assay

49
Q

haemophilia treatment

A

mild - desmopressin

severe - factor VIII/IX infusions

50
Q

DIC blood results

A

increased PT/APTT
increased FDP (d-dimers)
decreased platelets
decreased fibrinogen

51
Q

DIC treatment

A

replacement therapy (platelet, plasma, fibrinogen transfusions)

52
Q

polythemia vera mutation

A

JAK2

53
Q

pruritis following hot baths

A

polythaemia vera

54
Q

arterial thrombosis treatment

A

aspirin

clopidogrel

55
Q

venous thrombosis treatment

A

heparin
warfarin
DOACS

56
Q

most common acute leukaemia in adults

A

AML

57
Q

most common childhood cancer

A

ALL

58
Q

definitive diagnosis of leukaemias

A

immunophenotyping

59
Q

characteristic AML biopsy findings

A

auer rods

60
Q

CML classic feautres

A

massive splenomegaly
weight loss
tiredness fever

61
Q

most common leukeamia in adults

A

CLL

62
Q

CLL characteristics

A

smudge cells

63
Q

hodgkins lymphoma characteristics

A

reed-sternberg cells
orderly lymph node spread
alcohol induced pain

64
Q

non-hodgkins lymphoma characteristics

A

B symptoms more common

painless, multiple sites

65
Q

myeloma pathophysio

A

plasma cell proliferation - abnormal clonal proliferation of post germinal B cells

66
Q

myeloma investigations

A

bence johns urine

peripheral blood film: rouleaux formation

67
Q

myeloma treatment

A

stem cell transplant

MPT if not suitable

68
Q

philidelphia chromosome assoc with what malignancy

A

chronic myeloid leukemia

Third Year Exam (11 decks)

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