4th Year Additions Flashcards

1
Q

what is irradiated blood?

A

blood that has been treated with radiation to prevent transfusion associated graft versus host disease

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2
Q

who needs CMV negative blood?

A

pregnant women and foetus

granulocyte transfusions

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3
Q

what is anisocytosis?

A

variation in size of RBCs

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4
Q

what are target cells seen in?

A

iron deficiency anaemia

post-splenectomy

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5
Q

what are heinz bodies seen in?

A

G6PD deficiency

alpha thalassaemia

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6
Q

what are Howell-Jolly bodies seen in?

A

spleen problem

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7
Q

when does the reticulocyte increase?

A

haemolytic anaemia

acute blood loss

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8
Q

what are schistocytes?

A

fragments of RBCs and indicate damage in their journey through vessels

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9
Q

causes of schistocytes?

A
DIC
HUS
TTP
MAHA
haemolytic anaemia
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10
Q

what are sideroblasts seen in?

A

myelodysplastic syndrome

sideroblastic anaemia

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11
Q

what are smudge cells seen in?

A

CLL

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12
Q

what are spherocytes seen in?

A

hereditary spherocytosis

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13
Q

inherited haemolytic anaemias

A
hereditary spherocytosis
hereditary elloptocytosis
thalassaemia
sickle cell anaemia
G6PD deficiency
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14
Q

acquired haemolytic anaemias

A

AIHA
PNH
MAHA
prosthetic valve-related

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15
Q

how does tumour lysis syndrome cause AKI?

A

raised urate

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16
Q

what is MALT lymphoma associated with?

A

H. pylori

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17
Q

what is smouldering myeloma?

A

progression of MGUS

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18
Q

investigations for myeloma

A

BLIP

  • BJP
  • light chain assay
  • immunoglobulins in serum
  • protein electrophoresis of serum
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19
Q

what does thalidomide increase the risk of?

A

VTE

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20
Q

what is TTP (thrombotic thrombocytopenia purpura)?

A

small blood clots form throughout the body causing thrombocytopenia

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21
Q

what causes clot formation in TTP?

A

problem with protein ADAMTS13

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22
Q

RFs for development of TTP

A

obesity

pregnancy

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23
Q

management of TTP

A

plasma exchange with FFP

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24
Q

what is heparin-induced thrombocytopenia (HITT)?

A

development of Ab against platelets in response to exposure to heparin
target PF4

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25
Q

management of HITT

A

use different anticoagulant

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26
Q

anticoagulation choice in pregnancy?

A

LMWHs

if recurrent PEs consider IVC filter

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27
Q

female iron requirements daily

A

1.5mg/day

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28
Q

male iron requirements daily

A

1mg/day

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29
Q

pregnancy iron requirements daily

A

7.5mg/day

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30
Q

who usually gets Gaucher’s disease?

A

Jewish inheritance

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31
Q

genetic inheritance of Gaucher’s disease?

A

AR

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32
Q

what is Gaucher’s disease?

A

glucocerebrosidase accumulates and lipids cannot be broken down leading to deposits in organs

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33
Q

presentation of Gaucher’s disease

A

skeletal
blood
abdominal complaints

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34
Q

management of Gaucher’s disease

A

enzyme replacement therapy of GBA enzyme

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35
Q

who get’s Tay-Sachs disease?

A

Jewish people

36
Q

inheritance of Tay-Sachs disease?

A

AR

37
Q

what is Tay-Sachs disease?

A

genetic defect in hexosaminidase A gene which causes a deficit beta-hexosamindase A leading to accumulation of fatty deposits that damage brain and spinal cord

38
Q

presentation of Tay Sachs

A

6 months with loss of vision and hearing, dysphagia, muscle weakness

39
Q

three forms of Tay Sachs

A

infantile
juvenile
late onset/adult

40
Q

management of Tay Sachs

A

supportive + manage complications

41
Q

what is the inheritance pattern of Niemann-Pick disease?

A

AR

42
Q

what is Niemann-Pick disease?

A

abnormal sphinogmyelin degradation leading to cells being unable to metabolise cholesterol and lipids

43
Q

presentation of Niemann-Pick disease

A

contraction
difficulty walking
sleep disturbance

44
Q

management of Niemann-Pick

A

measure blood and skin biopsies to assess levels

45
Q

antibody in warm AIHA?

A

IgG

46
Q

antibody in cold AIHA

A

IgM

47
Q

types of blood transfusion reactions

A
  1. non-haemolytic febrile reaction
  2. minor allergic reaction
  3. anaphylaxis
  4. acute haemolytic reaction
  5. transfusion-associated circulatory overload (TACO)
  6. transfusion-related acute lung injury (TRALI)
48
Q

what causes a non-haemolytic febrile reaction?

A

antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from blood cells during storage??

49
Q

presentation of non-haemolytic febrile reaction

A

fever

chills

50
Q

management of non-haemolytic febrile reaction

A

slow or stop transfusion
paracetamol
monitor

51
Q

presentation of minor allergic reaction

A

pruritus

urticaria

52
Q

management of minor allergic reaction

A

temporarily stop transfusion
antihistamine
monitor

53
Q

management of anaphylaxis

A

stop transfusion
IM adrenaline
ABC support - oxygen + fluids

54
Q

what causes an acute haemolytic reaction?

A

ABO incompatibility

55
Q

presentation of acute haemolytic reaction

A

fever
abdo pain
hypotension

56
Q

management of acute haemolytic reaction

A

check blood, patients blood and send for DAT testing, repeat crossmatching

fluid resuscitation

57
Q

what causes transfusion-associated circulatory overload? (TACO)

A

excessive rate of transfusion

pre-existing heart failure

58
Q

presentation of TACO

A

pulmonary oedema

hypertension

59
Q

TACO management

A

slow or stop transfusion
consider IV loop diuretics (furosemide)
oxygen

60
Q

what causes transfusion-related acute lung injury (TRALI)?

A

non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability by host neutrophils activated by substances in donated blood

61
Q

presentation of TRALI

A

hypoxia
pulmonary infiltrates on CXR
fever
hypotension

62
Q

management of TRALI

A

stop transfusion
oxygen
supportive care

63
Q

what does cryoprecipitate contain?

A
factor VIII:C
von willebrand factor
fibrinogen
factor XIII
fibronectin
64
Q

what is the preferred anticoagulant in renal impairment?

A

apixaban

65
Q

can Coeliac disease cause hyposplenism?

A

yes

66
Q

what does raised LDH indicate?

A

haemolysis

67
Q

transfusion thresholds

A

Hb <70g/L without ACS

Hb <80g/L with ACS

68
Q

myeloma management pathway

A

suitable for transplant= brotezomib + dex
unsuitable= thalidomide + alklayting agent + dex

chemotherapy before stem cell transplant

69
Q

what is used to prevent relapses in myeloma?

A

bortezomib

70
Q

genetic mutation in acute promyelocytic leukaemia

A

t(15;17)

71
Q

what is seen on blood film in acute promyelocytic leukaemia

A

auer rods

72
Q

what genetic change is in mantle cell lymphoma?

A

t(11;14)

73
Q

VTE management if Wells score 0-1

A

check d-dimer
if negative consider an alternative diagnosis
if positive get scan within 4 hours

74
Q

d-dimer high but scan negative VTE management

A

stop interim anticoagulation and repeat 6-8 days later

75
Q

treatment time length for VTE?

A

3 months provoked

6 months unprovoked

76
Q

criteria for platelet transfusion

A

<10 x 10^9 no bleeding
<30 x10^ 9 minor bleeding
<100 x 10^9 in severe/ critical site

77
Q

presentation of acute intermittent porphyria

A

red urine on standing

neuro, abdo, etc

78
Q

what is Wiskott-Aldrich syndrome?

A

defect in WASP gene
x-linked recessive
low IgM

79
Q

types of Hodgkin’s lymphoma

A
nodular sclerosing (most common - lacunar cells)
mixed cellularity
lymphocyte predominant (best prognosis)
lymphocyte depleted (worst prognosis)
80
Q

what is the most common type of lymphoma?

A

large dffuse B cell lymphoma

81
Q

what is on microscopy in Burtkitt’s?

A

starry sky

82
Q

when in hydroxyurea used in sickle cell?

A

prevent further crisis

83
Q

what can packed red cells increase?

A

k+

84
Q

drug causes of G6PD deficiency

A

sulph drugs - sulphonamides, sulphasalazine and SUs

malaria prophylaxis

85
Q

what reverses rivaroxaban and apixaban?

A

andexanet alfa