Haem

Question 1

lmwh test

Answer 1

anti xa

Question 2

what is the rapaport shunt?

Answer 2

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

Question 3

dabigatran acts on

Answer 3

thrombin

Question 4

what are the mechanical causes of acquired haemolysis?

Answer 4

DIC HUS

Question 5

b12 made in the

Answer 5

methionine cycle

Question 6

what does hydroxycarbamide do and what disease is it used in

Answer 6

induces production of HbF in sickle cell

Question 7

how does dipyramidole work

Answer 7

phosphodiesterase inhibitor

Question 8

what decreases iron absorption

Answer 8

tea calcium cereals

Question 9

beta genes on what chromosome

Answer 9

11

Question 10

what are the three main types of thalassaemia?

Answer 10

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

Question 11

what does B + mean

Answer 11

a reduced function of a beta gene

Question 12

stages of granulopoeiis

Answer 12

myeloblast – promyelocyte – myelocyte – metamyelocyte – neutrophil

Question 13

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

Answer 13

g6pd

Question 14

polychromasia

Answer 14

reticulocytes

Question 15

mechanical damae

Answer 15

red cell fragments

Question 16

aspirin works by

Answer 16

inhibiting cox which is nec to produce thromboxane a2

Question 17

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

Answer 17

400ug

Question 18

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

Answer 18

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

Question 19

what inheritance pattern do haemoglobinopathies have

Answer 19

aut rec

Question 20

heparin works by

Answer 20

potentiating anti thrombin

Question 21

what is sickle cell?

Answer 21

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

Question 22

what are the three main types of macrocytic anaemia

Answer 22

megaloblastic non megaloblastic spurious

Question 23

what are the two classes of haemoglobinopathies

Answer 23

structural variant thalassaemia

Question 24

what is fibrinolysis?

Answer 24

breakdown of the fibrin clot

Question 25

what is a spurious macrocytic anaemia

Answer 25

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

Question 26

what is the main structural variant haemoglobinopathy?

Answer 26

HbS in sickle cell

Question 27

what are the 4 general causes of haemolysis?

Answer 27

autoimmune alloimmune acquired genetic

Question 28

what are the main causes of a macrocytosis

Answer 28

alcohol liver disease hypothyroid

Question 29

what pathway produces folate

Answer 29

folate cycle

Question 30

what are b12 and folate essential for

Answer 30

nuclear maturation

Question 31

at the same po2 …………… bind more 02

Answer 31

HbF and myoglobin

Question 32

oxidative damage

Answer 32

Heinz bodies

Question 33

a b and o genes all on what chromosome

Answer 33

9

Question 34

what type of mutations cause beta thalassaemia

Answer 34

point mutations

Question 35

anaerobic glycolysis pathway of nadh generation

Answer 35

ebden myerhoff

Question 36

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

Answer 36

obese mum diabetic mum sickle cell thalassaemia mum on antiepileptics

Question 37

what can induce HbF production in sickle cell?

Answer 37

hydroxyccrabamide

Question 38

hypersegmented neutrophils

Answer 38

megaloblastic

Question 39

pappenheimer

Answer 39

thalassaemia

Question 40

what is a thalassaemia

Answer 40

defect in globin chain synthesis

Question 41

how do you distinguish between alpha and beta thalasaemia on testing

Answer 41

chromatography

Question 42

what does the folate cycle do?

Answer 42

U -- T change

Question 43

hb curve shifted to the right by molecules that

Answer 43

bind to hb

Question 44

what does B0 mean

Answer 44

no beta gene

Question 45

what are the main genetic causes of haemolysi s/

Answer 45

hereditary spherocytosis G6PD deficiency sickle cell disease

Question 46

what does the methionine cycle do

Answer 46

produce methyl donors

Question 47

alpha genes on what chromosome

Answer 47

16

Question 48

gene in APS

Answer 48

beta 2 glycoprotein 1 affected

Question 49

what are the main causes of multiple clotting factor deficiency

Answer 49

DIC liver disease vitk deficient warfarin

Question 50

how does clopidogrel work

Answer 50

adp antagonist

Question 51

what is a remnant of dna from hyposplenism?

Answer 51

Howell jowly bodies

Question 52

oval macrocytes

Answer 52

megaloblastic

Question 53

…. are circulating versions of mast cells

Answer 53

basophils

Question 54

what is the issue in sickle trait

Answer 54

may sickle in severe hypoxia

Question 55

Heinz bodies

Answer 55

oxidative damage

Question 56

stages of erythropoiesis

Answer 56

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

Question 57

warfarin acts on

Answer 57

vit k and protein c and s

Question 58

what is the inheritance pattern for hereditary spherocytosis

Answer 58

aut dom

Question 59

spherocytes sign of

Answer 59

membrane damage

Question 60

warfarin test

Answer 60

inr

Question 61

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

Answer 61

5mg

Question 62

target cells

Answer 62

thalassaemia

Question 63

HbS polymerises when

Answer 63

hypoxia

Question 64

what types of haemoglobin are affected in alpha thalassaemia?

Answer 64

HbA HbA2 HbF

Question 65

single factor clotting deficiencies follow which inheritance pattern

Answer 65

x linked

Question 66

what cells in the interstitium detect hypoxia

Answer 66

fibroblasts

Question 67

what inheritance pattern is sickle cell

Answer 67

aut rec

Question 68

what are the three types od beta thalassaemia

Answer 68

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

Question 69

thrombin causes

Answer 69

conversion of fibrinogen to fibrin

Question 70

what replenishes glutathione and what is the pathway called?

Answer 70

nadph generates this in the hexose monophosphate shunt

Question 71

causes of megaloblastic anaemia

Answer 71

b12 or folate deficiency drugs inherited conditions

Question 72

what increases iron absorption

Answer 72

alcohol

Question 73

baby dies around 6mths to a year - thalassaemia

Answer 73

beta

Question 74

what is the main cause of a normocytic anaemia

Answer 74

hypoproliferative (acute blood loss or early iron deficiency)

Question 75

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

Answer 75

HbA2

Question 76

reverse warfarin with

Answer 76

vit k and clotting factors

Question 77

unfractionated heparin test

Answer 77

aptt

Question 78

what are the causes of alloimmune disorders

Answer 78

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

Question 79

baby dies in utero - thalassaemia

Answer 79

alpha

Question 80

rivaroxaban acts on

Answer 80

xa

Question 81

red cell fragments

Answer 81

mechanical damage

Question 82

what decreases oxidative stress

Answer 82

glutathione

Question 83

what is a megaloblast

Answer 83

abn large rbc precurser with an immature nucleus

Question 84

what are the possible causes of a hypochromic microcytic anaemia?

Answer 84

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

Question 85

what are the causes of autoimmune haemolysis

Answer 85

cold - infections / lymphoproliferative disorders warm - autoimmune disorders

Question 86

how many beta genes are there

Answer 86

one per chromosome hence 2 in total

Question 87

what types of haemoglobin are affected in beta thalassaemia?

Answer 87

HbA

Question 88

what are the membrane causes of haemolysis

Answer 88

liver disease vit e deficiency

Question 89

what is the defect in macrocytic anaemia

Answer 89

nuclear defects with either a defect in cell divison or maturation

Question 90

main causes of dic

Answer 90

sepsis obs emergency malignancy massive trauma large blood loss

Question 91

reverse heparin with

Answer 91

protamine sulphate

Question 92

what is the defect in hypochromic microcytic anaemia

Answer 92

cytoplasmic defect

Question 93

causes of non megaloblastic anaemia

Answer 93

liver disease hypothyroid alcohol marrow failure

Question 94

what is hereditary spherocytosis?

Answer 94

increase in cell lysis which increases bilirubin and decreased hb

Question 95

diagnose sickle cell

Answer 95

chromatography or gel electrophoresis

Question 96

what substances help platelets aggregate

Answer 96

thrombin/ thromboxane a2 and adp

Question 97

what are the products of intravascular haemolysi s

Answer 97

haemoglobinaemia methaemalbininaemia heamglobinuria haemosidinuria

Question 98

what are the two classes of sickle cell?

Answer 98

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

Question 99

how many alpha genes are there

Answer 99

2 per chromosome hence 4 in total

Question 100

does a reticulocyte have a nucleus?

Answer 100

no just remnants of rna

Question 101

what are the main types of single factor clotting deficiency

Answer 101

a - 8 b - 9

Question 102

what is methb?

Answer 102

fe3+ - has no oxygen carrying capacity