Haematology Flashcards

1
Q

what is the mode of inheritance for G6PD deficiency?

A

X-linked recessive

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2
Q

what is the role of G6PD enzyme?

A

protects red blood cells from oxidative stress

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3
Q

what is thrombocytopenia?

A

deficiency of platelets in blood

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4
Q

triad of haemolytic uraemic syndrome

A

AKI, anaemia and thrombocytopenia

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5
Q

commonest cause of haemolytic uraemic syndrome in children?

A

E.coli 0157

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6
Q

what is haemolytic anaemia?

A

premature destruction of red blood cells

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7
Q

hereditary haemolytic anaemia conditions

A

sickle cell, thalassaemias, hereditary spherocytosis

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8
Q

acquired haemolytic anaemia conditions

A

autoimmune disorders, infections e.g. malaria, medication reactions or trauma

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9
Q

Describe intravascular haemolysis and the pathophysiology of it

A

Haemolysis occurs in bloodstream
leads to release of free haemoglobin
-excess dealt with:
combo with haptoglobin/ albumin
lost in urine
stored in epithelial cells

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10
Q

Describe extravascular haemolysis and the pathophysiology of it

A

haemolysis taking place outside bloodstream
-mainly in spleen and liver -> RBCs= phagocytosed
splenomegaly and hepatomegaly are typical

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11
Q

what type of anaemia does anaemia of chronic disease present with

A

microcytic or normocytic

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12
Q

what are pencil cells associated with?

A

iron deficiency anaemia
(mainly when caused by coeliac disease)

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13
Q

what are Howell-Jolly bodies?

A

remnants of RBC nuclei
* they’re Jolly cause they have extra stuff to others *

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14
Q

what do Howell-Jolly bodies indicate?

A

functional hyposplenism - spleen not functioning or true - splenectomy

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15
Q

What is DIC? and what do blood results look like?

A

disseminated intravascular coagulation
consumption of clotting factors
blood results
- thrombocytopenia
- schistocytes
- raised d-dimer (fibrin degradation product)
- clotting profile = increased prothrombin time and APTT, decreased fibrinogen

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16
Q

what is ferritin?

A

acute phase protein- increases in response to infection

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17
Q

iron studies of anaemia of chronic disease

A

low serum iron - inhibition of iron absorption
high ferritin -
low transferrin - reduced ability

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18
Q

what is the blood universal donor?

A

O-

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19
Q

What blood can rhesus +ve patients receive?

A

Rh -ve AND +ve

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20
Q

fatigue, headache and itchy after hot bath?

A

polycythaemia rubra vera

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21
Q

what should be given to patient on warfarin experiencing significant bleed?

A

Prothrombin complex concentrate (PTCC)- reverses anticoagulation and contains factors 2, 7, 9 and 10

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22
Q

which test confirms the diagnosis of Haemophilia A?

A

factor VIII assay

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23
Q

NHS management of neutropenic sepsis?

A

IV piperacillin with tazobactam (broad spectrum asap)

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24
Q

diagnosis of chronic lymphocytic leukaemia

A

elevated white cell count with absolute lymphocytosis
-smudge cells on blood film

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25
Q

mot common hereditary thrombophilia

A

Factor V leiden

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26
Q

acquired thrombophilias

A

anti-phospholipid syndrome

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27
Q

what is myelofibrosis

A

myeloproliferative disorder
often presenting with pancytopenia

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28
Q

signs and symptoms of myelofibrosis

A

weight loss
night sweats
splenomegaly
marrow failure
signs: poikilocytes, dry tap on bone marrow aspiration

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29
Q

management of major bleed and INR>8

A

stop warfarin, give vit K and prothrombin complex concentrate

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30
Q

complication of essential thrombocytosis

A

ischaemic stroke

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31
Q

investigations for tumour lysis syndrome

A

ECG, U&E, calcium, uric aid

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32
Q

primary part of coagulation that heparin exerts action on

A

activation of antithrombin III - inactivation of thrombin -> inhibits formation of stable blood clots

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33
Q

type of reaction associated with immediate, severe reaction following blood transfusion

A

type II hypersensitivity - involves IgG/ IgM

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34
Q

what factor initiates the coagulation cascade?

A

factor XII

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35
Q

deficiency in what factor results in Haemophilia A

A

factor VIII

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36
Q

what feature may be present in patient with excessive loss of plasma proteins

A

increased susceptibility to infection

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37
Q

what blood type can receive donations from all blood types

A

AB positive

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38
Q

multiple myeloma symptoms

A

CRAB - high Calcium, Renal insufficiency, Anaemia and Bone lesions

39
Q

high total iron binding capacity with low ferritin and low serum iron

A

iron deficiency anaemia

40
Q

low total iron binding capacity with normal/high ferritin and normal serum iron

A

anaemia of chronic disease

41
Q

management of essential thrombocythemia

A

low risk - (<40 y/o AND no history of thrombosis, no cardiovascular risk factors, platelet count <1500) = aspirin only
high risk - (> 60 OR previous history of thrombosis, diabetes, hypertension, platelet count >1500) = hydroxycarbamide and aspirin

42
Q

what are typical findings for myelofibrosis?

A

blood film analysis- tear drop poikilocytes (blood cells squeeze out of fibrotic marrow)
bone marrow aspiration- dry tap

43
Q

which clotting factor is most likely affected in patient with Von Willebrand disease?

A

factor 8 - Von Willebrand promotes platelet adhesion to damage blood vessels and stabilises factor 8
*remember - V III = V W *

44
Q

what is a common finding following acute traumatic event e.g. surgery ?

A

neutrophilia

45
Q

Massive splenomegaly and weight loss

A

chronic Myeloid leukaemia

46
Q

management of chronic myeloid leukaemia?

A

Imatinib - tyrosine kinase inhibitor -> inhibits the activity brought on by the Philadelphia chromosome

47
Q

prolonged PT is most likely caused by which factor deficiency?

A

factor VII

48
Q

APTT measures speed of clotting via which pathway? what factors are involved?

A

intrinsic and common pathways
Intrinsic = Factors VII, IX, XI and XII
common = I, II, V and X
*remember- APTT = play table tennis INSIDE = intrinsic *

49
Q

PT measures the speed of clotting via which pathway? what factors are involved?

A

extrinsic and common pathway
Extrinsic= III and VII
common = I, II, V and X
remember - PT = play tennis OUTSIDE = extrinsic

50
Q

typical findings for antiphospholipid syndrome

A

miscarriage, leg rash, raised APTT, low platelets
anticardiolipin antibodies

51
Q

typical presentation of fever, headache, thrombocytopenia, haemolytic anaemia and renal failure with schistocytes on blood film

A

thrombotic thrombocytopenic purpura

52
Q

Stage 1 Hodgkin lymphoma

A

single lymph node involvement

53
Q

stage II Hodgkin’s lymphoma

A

2 or more lymph node/ regions on the same side of the diaphragm

54
Q

stage III Hodgkin’s lymphoma

A

nodes on both sides of the diaphragm

55
Q

stage IV Hodgkin’s lymphoma

A

spread beyond the lymph nodes

56
Q

rubbery lymph nodes and ‘starry sky’ appearance on biopsy

A

Burkitt’s lymphoma - type of non-hodgkin’s lymphoma

57
Q

most worrying sign of blood transfusion reaction

A

hypotension - indicating anaphylaxis, acute haemolytic reaction or transfusion-related acute lung injury

58
Q

What conditions does polycyteamia rubra vera have potential to transform into

A

Myelofibrosis or Acute Myeloid Leukaemia

59
Q

Duration of anticoagulation treatment following a provoked or unprovoked VTE

A

provoked = 3 months
unprovoked = 6 months

60
Q

how to distinguish between intramedullary and extramedullary haemolysis

A

extramedullary- leads to release of protoporphyrin (unconjugated bilirubin)
symptoms = jaundice and gall stones
intramedullary - leads to fee Hb in circulation => reduced haptoglobin
symptoms= pink urine

61
Q

examples of intramedullary haemolysis

A

G6PD deficiency
alloimmune haemolysis

62
Q

H.pylori eradication treatment

A

omeprazole, amoxicillin and vancomycin
(associated with MALT - a type of Non-Hodgkin’s lymphoma)

63
Q

what is the definitive diagnostic test for sickle cell anaemia

A

haemoglobin high performance liquid chromatography of whole blood lysate (HPLC)

64
Q

management of polycythemia vera

A

low risk - aspirin
high risk - hydroxycarbamide

65
Q

what is factor V leiden most likelt to present with

A

pulmonary embolism or DVT
(they are LAIDen down with coagulants )

66
Q

Immunoglobulin involved in warm autoimmune haemolytic anaemia

A

IgG
(warm weather is Great)

67
Q

Immunoglobulin in cold autoimmune haemolytic anaemia

A

IgM
(cold weather is Miserable)

68
Q

disproportionate microcytic anaemia

A

think beta thalassaemia trait

69
Q

what does neutrophilia tend to indicate?

A

bacterial infection
other main causes:
- trauma
- surgery
- necrosis
- haemorrhage

less commonly
- corticosteroid use
- myeloproliferative disease

70
Q

what are protein C and S

A

serum anticoagulants

71
Q

what does warfarin do to protein C and S

A

reduces its concentration - period of time (1-2 days) after starting warfarin that patient is procoaguable
- particularly if have Protein C or S deficiency

72
Q

what virus can precipitate an aplastic crisis in a patient with sickle cell disease

A

parvovirus B19 infection
-causes slapped cheek syndrome

73
Q

what is most common process that causes pernicious anaemia

A

autoimmune destruction of parietal cells in stomach that secrete intrinsic factor -> unable to absorb vitamin B12 in ileum

74
Q

investigation findings for osteomalacia

A

low calcium , low phosphate and high ALP

75
Q

what is the most appropriate long-term prophylactic in a patient with sickle cell anaemia who has repeated painful crises

A

daily oral hydroxyurea

76
Q

new onset sever microcytic anaemia at 3-9 months old with frontal bossing

A

beta thalassaemia major

77
Q

history of petechiae, thrombocytopenia, normal APTT and PT

A

typical of Immune thrombocytopenic purpura

78
Q

most common inherited thrombophilia

A

factor V Leiden

79
Q

what blood test result would indicate that heparin treatment needs to be withdrawn

A

drop in platelets by more than 30%
- heparin induced thrombocytopenia

80
Q

presentation of beta thalassaemia trait

A

usually asymptomatic
- microcytic anaemia
- target cells on blood film
patient usually from Mediterranean countries or middle eastern

81
Q

smudge cells

A

chronic lymphocytic leukaemia

82
Q

schistocytes

A

intravascular haemolysis

83
Q

what cells cause graft vs host disease

A

caused by T cells in donor tissue

84
Q

tumour lysis syndrome

A

PUKE calcium
phosphate
uric acid
potassium (K) = all elevated
calcium = decreased

85
Q

indications for blood transfusion

A

symptomatic anaemia
and haemoglobin <8 g.dl

86
Q

heparin monitoring and reversal

A

APT - for unfractionated, not much monitoring needed for LMWH
reversal = protamine sulphate

87
Q

warfarin monitoring and reversal

A

INR and vitamin K

88
Q

mechanism of action of clopidogrel

A

anti-platelet - ADP receptor antagonist

89
Q

mechanism of action of abciximab

A

anti platelet - GP IIb/IIIa inhibitors

90
Q

mechanism of action of heparin

A

anti-coagulant - potentiates anti thrombin effect

91
Q

mechanism of action of warfarin

A

anti-coagulant - vitamin K antagonist

92
Q

mechanism of action of aspirin

A

irreversibly blocks COX in platelets
inhibits TXA2 synthesis and PGI2

93
Q
A