Haem Flashcards

1
Q

lmwh test

A

anti xa

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2
Q

what is the rapaport shunt?

A

2,3, dpg – less bound at same o2 so more o2 released from hb

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3
Q

dabigatran acts on

A

thrombin

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4
Q

what are the mechanical causes of acquired haemolysis?

A

DIC HUS

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5
Q

b12 made in the

A

methionine cycle

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6
Q

what does hydroxycarbamide do and what disease is it used in

A

induces production of HbF in sickle cell

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7
Q

how does dipyramidole work

A

phosphodiesterase inhibitor

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8
Q

what decreases iron absorption

A

tea calcium cereals

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9
Q

beta genes on what chromosome

A

11

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10
Q

what are the three main types of thalassaemia?

A

trait - one or two genes missing disease HbH - one left barts hydrops foetalis - none functioning so die in utero

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11
Q

what does B + mean

A

a reduced function of a beta gene

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12
Q

stages of granulopoeiis

A

myeloblast – promyelocyte – myelocyte – metamyelocyte – neutrophil

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13
Q

what is the rate limiting enzyme in the hexose monophosphate shunt?

A

g6pd

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14
Q

polychromasia

A

reticulocytes

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15
Q

mechanical damae

A

red cell fragments

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16
Q

aspirin works by

A

inhibiting cox which is nec to produce thromboxane a2

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17
Q

how much folic acid do you give in a low risk pregnancy

A

400ug

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18
Q

what are the two types of coombs test and what do they od

A

indirect and direct antiglobulin test which both test for antibody in plasma

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19
Q

what inheritance pattern do haemoglobinopathies have

A

aut rec

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20
Q

heparin works by

A

potentiating anti thrombin

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21
Q

what is sickle cell?

A

point mutation in codon 6 of beta globin gene that substitutes glutamine to valine

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22
Q

what are the three main types of macrocytic anaemia

A

megaloblastic non megaloblastic spurious

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23
Q

what are the two classes of haemoglobinopathies

A

structural variant thalassaemia

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24
Q

what is fibrinolysis?

A

breakdown of the fibrin clot

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25
Q

what is a spurious macrocytic anaemia

A

cells are a normal size however appear large due to cell agglutination

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26
Q

what is the main structural variant haemoglobinopathy?

A

HbS in sickle cell

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27
Q

what are the 4 general causes of haemolysis?

A

autoimmune alloimmune acquired genetic

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28
Q

what are the main causes of a macrocytosis

A

alcohol liver disease hypothyroid

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29
Q

what pathway produces folate

A

folate cycle

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30
Q

what are b12 and folate essential for

A

nuclear maturation

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31
Q

at the same po2 …………… bind more 02

A

HbF and myoglobin

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32
Q

oxidative damage

A

Heinz bodies

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33
Q

a b and o genes all on what chromosome

A

9

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34
Q

what type of mutations cause beta thalassaemia

A

point mutations

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35
Q

anaerobic glycolysis pathway of nadh generation

A

ebden myerhoff

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36
Q

what makes a pregnancy at higher risk of neural tube defects and hence require a larger dose of folic acid

A

obese mum diabetic mum sickle cell thalassaemia mum on antiepileptics

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37
Q

what can induce HbF production in sickle cell?

A

hydroxyccrabamide

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38
Q

hypersegmented neutrophils

A

megaloblastic

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39
Q

pappenheimer

A

thalassaemia

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40
Q

what is a thalassaemia

A

defect in globin chain synthesis

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41
Q

how do you distinguish between alpha and beta thalasaemia on testing

A

chromatography

42
Q

what does the folate cycle do?

A

U – T change

43
Q

hb curve shifted to the right by molecules that

A

bind to hb

44
Q

what does B0 mean

A

no beta gene

45
Q

what are the main genetic causes of haemolysi s/

A

hereditary spherocytosis G6PD deficiency sickle cell disease

46
Q

what does the methionine cycle do

A

produce methyl donors

47
Q

alpha genes on what chromosome

A

16

48
Q

gene in APS

A

beta 2 glycoprotein 1 affected

49
Q

what are the main causes of multiple clotting factor deficiency

A

DIC liver disease vitk deficient warfarin

50
Q

how does clopidogrel work

A

adp antagonist

51
Q

what is a remnant of dna from hyposplenism?

A

Howell jowly bodies

52
Q

oval macrocytes

A

megaloblastic

53
Q

…. are circulating versions of mast cells

A

basophils

54
Q

what is the issue in sickle trait

A

may sickle in severe hypoxia

55
Q

Heinz bodies

A

oxidative damage

56
Q

stages of erythropoiesis

A

pronormoblast – early normoblast – int normonlast – late normoblast – reticulocyte – erythrocyte

57
Q

warfarin acts on

A

vit k and protein c and s

58
Q

what is the inheritance pattern for hereditary spherocytosis

A

aut dom

59
Q

spherocytes sign of

A

membrane damage

60
Q

warfarin test

A

inr

61
Q

how much folic acid do you give in a high risk pregnancy

A

5mg

62
Q

target cells

A

thalassaemia

63
Q

HbS polymerises when

A

hypoxia

64
Q

what types of haemoglobin are affected in alpha thalassaemia?

A

HbA HbA2 HbF

65
Q

single factor clotting deficiencies follow which inheritance pattern

A

x linked

66
Q

what cells in the interstitium detect hypoxia

A

fibroblasts

67
Q

what inheritance pattern is sickle cell

A

aut rec

68
Q

what are the three types od beta thalassaemia

A

trait - one chromosome copy affected intermedia - both copies affected major - no copies at all

69
Q

thrombin causes

A

conversion of fibrinogen to fibrin

70
Q

what replenishes glutathione and what is the pathway called?

A

nadph generates this in the hexose monophosphate shunt

71
Q

causes of megaloblastic anaemia

A

b12 or folate deficiency drugs inherited conditions

72
Q

what increases iron absorption

A

alcohol

73
Q

baby dies around 6mths to a year - thalassaemia

A

beta

74
Q

what is the main cause of a normocytic anaemia

A

hypoproliferative (acute blood loss or early iron deficiency)

75
Q

what type of haemoglobin is produced in beta thalassaemia to compensate for the non function beta

A

HbA2

76
Q

reverse warfarin with

A

vit k and clotting factors

77
Q

unfractionated heparin test

A

aptt

78
Q

what are the causes of alloimmune disorders

A

immune - haemolytic transfusion reaction passive ab - haemolytic disease of the newborn

79
Q

baby dies in utero - thalassaemia

A

alpha

80
Q

rivaroxaban acts on

A

xa

81
Q

red cell fragments

A

mechanical damage

82
Q

what decreases oxidative stress

A

glutathione

83
Q

what is a megaloblast

A

abn large rbc precurser with an immature nucleus

84
Q

what are the possible causes of a hypochromic microcytic anaemia?

A

problem with the porphyrin ring problem with the haem group problem with the globin chain (thalassaemia)

85
Q

what are the causes of autoimmune haemolysis

A

cold - infections / lymphoproliferative disorders warm - autoimmune disorders

86
Q

how many beta genes are there

A

one per chromosome hence 2 in total

87
Q

what types of haemoglobin are affected in beta thalassaemia?

A

HbA

88
Q

what are the membrane causes of haemolysis

A

liver disease vit e deficiency

89
Q

what is the defect in macrocytic anaemia

A

nuclear defects with either a defect in cell divison or maturation

90
Q

main causes of dic

A

sepsis obs emergency malignancy massive trauma large blood loss

91
Q

reverse heparin with

A

protamine sulphate

92
Q

what is the defect in hypochromic microcytic anaemia

A

cytoplasmic defect

93
Q

causes of non megaloblastic anaemia

A

liver disease hypothyroid alcohol marrow failure

94
Q

what is hereditary spherocytosis?

A

increase in cell lysis which increases bilirubin and decreased hb

95
Q

diagnose sickle cell

A

chromatography or gel electrophoresis

96
Q

what substances help platelets aggregate

A

thrombin/ thromboxane a2 and adp

97
Q

what are the products of intravascular haemolysi s

A

haemoglobinaemia methaemalbininaemia heamglobinuria haemosidinuria

98
Q

what are the two classes of sickle cell?

A

sickle trait - one working one sickled sickle disease - both genes sickled

99
Q

how many alpha genes are there

A

2 per chromosome hence 4 in total

100
Q

does a reticulocyte have a nucleus?

A

no just remnants of rna

101
Q

what are the main types of single factor clotting deficiency

A

a - 8 b - 9

102
Q

what is methb?

A

fe3+ - has no oxygen carrying capacity