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THIRD YEAR > Haem 2 > Flashcards

Haem 2 Flashcards

1
Q

haemaglobinopathies are generally what

A

autosomal recessive

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2
Q

commonest monogenic disorders

A

thallasaemias

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3
Q

increase in thallasaemia where

A

malaria endemic areas

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4
Q

where are people with thallasaemia from

A

SEA
ME
medditirean

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5
Q

alpha thal trait

what it be mistaken for

A

1 or 2 missing

iron defic but ferritin normal and RBC count is high

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6
Q

HbH disease
alpha chain production
what kind of anaemia

A

only 1 alpha chain left
<30% or normal
anaemia with low MCV and MCH

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7
Q

what are HbH bodies

A

excess beta chains that form tetramers that can’t carry oxygen
red cell inclusions (HbH bodies) can be seen with some stains

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8
Q

Hb barts hydrops fetalis is what

what forms are seen at birth

A 
minimal or no alpha chain production. HbA can't be made
Hb barts (gamma4) and hbh at birth
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9
Q

what with hydrops fetalis is seen in peripheral blood

what happens to most babies with this

A

nucleated RBC

most die in utero

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10
Q

in beta thallasamei what kind of hb is affected

A

only HbA

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11
Q

genetic for beta thallasaemias

A

point mutations

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12
Q

beta thallasmaie trait is what

what kind of anaemia

A

reduced amount in one or none in one

mild or no anaemia

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13
Q

beta thal intermediate

symp and rx

A

both low or ones got none and the other low

mod severity requiring occasional transfusion

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14
Q

beta thal major

treatment

A

none in both

life logn transfusion

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15
Q

blood film of beta thal major

A

macrocytosis
hypochromia
anispokilocytosis and target cells

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16
Q

HPLC of beta thal major

A

mainly HbF present

small amounts of HbA

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17
Q

treatment for beta thal major

A

regular transfusion to maintain hb 95-150

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18
Q

transfusion cx

A 
iron over load
hepB/C
alloautoantibodies
TRs
bacterial sepsis
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19
Q

how much iron in a unit of red cells

A

200-250mg

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20
Q

iron overload leads to what endocrine
cardiac
liver

A

endo - impaired growth, DM, osteoporosis
cardiac - cardiomyopathy, arrhythmias
liver - cirrhosis, hepatocellular cancer

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21
Q

what can be given to treat iron overload

A

desferrioxamine - chelatops bind to iron and secreted in urine/stool

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22
Q

increase in HbA2 seen on HPLC

A

beta that trait

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23
Q

can HPLC diagnose alpha thal trait

A

no normal in it

need DNA testing to confirm

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24
Q

sickle cell genetics

A

point mutation on chromosome 6 of the beta globin gene that substitutes glutamine to valine production Bs

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25
Q

HBS polymerase

A

changes shape if exposed to low levels of oxygen fro a prolonged period of time

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26
Q

sickle trait

HbS

A

one normal one betas

HbS<50%

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27
Q

what can happen in sickle trait

A

can sickle in severe hypoxia - high amplitude, under anaesthesia

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28
Q

sickle cell anaemia genetic
HbS HbA
episodes of what

A

autosomal recessive
HbS>80% no HbA
episodes of tissue infarction due to vascular occlusion

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29
Q

effects of sickle cell anaemia
chronic haemolysis
hyposplenism
sickle cell crisis

A

shortened RBC life spac
due to repeated splenic infarcts
hypoxia, dehydration, infection, cold exposure, stress/fatigue

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30
Q

treatment of sickle cell crisis

A

opiate analgesia, hydration, rest, oxygen, ABs, transfusion

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31
Q

life long treatment

hyposplenism and others

A

prophylactic penicillin. pneumococcal, meningococcal and haemophilus influenza vaccines yearly

folic acid supplementation

hydroxycarbamide - induce HbF production

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32
Q

HbS/beta thal

A

mild if b+ severe if b0

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33
Q

HbSC dx

A

milder but increased risk of thrombosis

lysine replaces glutamic acid in position 6

34
Q

activation of complement cascade in haemolytic TR

A

c3a and c5a
powerful anaphylaxotoxins, increase vascular permeability, dilate blood vessels, release serotonin and histamine

formation of MAC - rupture of transfused RBCs

35
Q

activation of coagulation in haemolytic TR

A

DIC

36
Q

activation of kinin system in haemolytic TR

A

activated by factor 7, formation of bradykinin:
arteriolar dilatation
vascular permeability - hypotension - release of catecholamines - vasoconstriction in kidneys and in other organs

37
Q

delayed TR when

A

5-10 d after

38
Q

what can be found in a delayed HTR

A

spherocytic red cells on film
increase in bilirubin and LDH
positive DAGT and/- RC alloautoantibodies
may be degree of renal failure present

39
Q

what kind of transfusions for febrile TRs happen in

A

2% of RC 20% of platelet

40
Q

symp of febrile TR

A

1-2 degrees rapid rise

41
Q

preventionof febrile TR

A

prevention - antipyretics, leucodepleted blood components

42
Q

whats found in febrile TR

A

ABs to contaminating white blood cells - release of cytokine and vasoactive substances from white cells during storage

43
Q

urticarial reactions why
symp
rx

A

mast cells - IgE to infused plasma proteins
rash and wheals within mins

slow transfusion.

44
Q

circulatory TR who what

A

elderly px with CCF

pulmonary oedema

45
Q

bacterial infection organisms in RC

platelets

A

psuodomonas, yersinina

staph, strep, serrritla, salmonella

46
Q

viral infections HIV
HBC
HCV

A

1/6mil
1/1.6mil
1/2.6mil

47
Q

transferrin >50%

A

risk of iron overload

48
Q

dx of iron overload

A

males ferritin >300
females >200
liver biopsy if uncertain or to assess organ damage

49
Q

treatment of primary haemochromatosis

A

weekly phlebotomy 450-500ml (200-250mg of iron)

get ferritin to <20 then keep it under 10ug/L

50
Q

treatment for secondary iron overload

why is venesection not an option

A

not an option in already anaemic px

deferoxamine SC or IV, deferipone PO, deferasirox PO

51
Q

what happens to anaemia in chronic disease

A

inflam macrophages iron block -> iron supply to marrow erythroblasts impaired -> hypochronic red cells

52
Q

why are red cells more prone to damage

A

biconcave shape
limited metabolic reserve
can’t generate new proteins - no nucleus

53
Q

consequences of haemolysis

A

erythroid hyperplasia

excess RC breakdown

54
Q

extravascular haemolysis what happens
what grows
release of what

A

taken up by reticuloendothelial system (spleen and liver predom)
hepatosplenomegaly
release of protoporphyrian: unconjugated bilirubin - jaundice, gall stones, urobiliuria

55
Q

intravascular what happens

ix kind of

A

RC destroyed within circulation spilling contents

haemglobinaemia
methalmalbinaemia
haemoglobinuria - pink urine turns black on standing
haemisderinuria

56
Q

increased hb and increased haemsiderin

A

intravascular

57
Q

which kind of haemolysis is most common

A

extrvacular

58
Q

what causes intravascular haemolysis

A

haemolytic TR
GP6D
black water fever
PNH/PCH

59
Q

film showing spherocytes
red cell fragments
heinz bodies
HbS

A

membrane damage
mechanical damage
oxidative stress
sickle cell

60
Q

test for auto immune haemolysis

A

direct coombs test

61
Q

AI haemolsysi types IgG

IgM

A

idiopathic. commonest. SLE, CLL, penicillins, infections

idiopathic. EBV. mycoplasma. CLL

62
Q

alloimmune haemolyiss
immune response
passive transfer

A

AB produced

haemolytic disease of the newborn IgG

63
Q

immune transfer is what
immediate
delayed

A

haemolytic TR
immediate IgM intravascular
immediate IgG extravascular

64
Q

mechanical destruction of Rbc causes

A 
DIC
haemolytic uraemia syndrome EColi 0157
leaving heart valve - MAHA
TTP infection - malaria 
burns - microspeheres
65
Q

abnormal cell membrane liver

A

liver disease - zieves syndrome

haemolysis, alcoholic liver disease, hyerlipidaemia

66
Q

other causes of abnormal cell membrane

A

vit E defic, paroxysmal nocturnal haemaglobinemia

hereditary pherocytosis

67
Q

paroxysmal nocturnal haemaglobinemia

A

hams test

urine dark only at night and morning

68
Q

abnormal cell metabolism

A

GP6D defic - genetic

dapsone - haemolytic anaemia

69
Q

myeloma direct tumour effects

A

bone lesions, increased calcium, bone pain, marrow failure

70
Q

myeloma paraprotein mediated effects

A

renal failure, immune depression, hyperviscosity, amyloid

71
Q

malignant melanoma cell shift balance of bone production

A

increase in osteoclasts

decrease in osteoblasts

72
Q

treatment of myeloma

A

corticosteroids, alkylating agent - cyclophosphamide, thalidomide, lenlamide

73
Q

what can be used to monitor myeloma

A

paraprotein

74
Q

monoclonal gamapathy of uncertain significance

A

paraprotein <30
monoclonal protein in blood
bona marrow plasma cells <10%
no evidence of myeloma and organ damage

75
Q

AL amyloidosis is what
where is mutation
poor prognosis if
symp

A

small cells plasma clone
mutation in light chain
poor prognosis esp if cardiac failure
kidneys: nephrotic syndrome, neuropathy, GI - malabsorption, heart - cardiomyopathy, liver disease

76
Q

AL amyliodosis ix

A

congo red stain
green birefringence
rectal or fat biopsy

77
Q

walderstroms macroglobinaemia what Ig

A

IgM - pentameric

78
Q

lymphaplasmacytoid neoplasm is what

A

clonal disease of cells intermediate between lymphocytes and plasma cells

79
Q

tumour effects of walderstroms

A

lymphadenopathy
splenomegaly
marrow failure

80
Q

paraprotein effects of walderstorms

A

hyper viscosity - fatigue, visual disturbances, confusion, bleeding CF
neuropathy

night sweats, weight loss

81
Q

treatment for walderstorms

A

chemo

plasmapheresis

THIRD YEAR (27 decks)

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