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Pathology > Ha - Paeds Haem > Flashcards

Ha - Paeds Haem Flashcards

1
Q

how to response to infection differ in adults and kids

A

kids = lymphocytosis
adults = neutrophilia

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2
Q

how does neonate blood count differ from children

A

neonate has higher haem F, higher Hb, lymphocyte count, neutrophil count

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3
Q

why doesn’t beta thalassaemia major affect babies

A

they have more ham F not haem B so doesnt develop until switched to haem B

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4
Q

3 causes of polycythaemia in foetus / neonate

A

twin to twin transfusion
IU hypoxia
placental insufficiency

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5
Q

what is bad about polycythaemia

A

get hyperviscosity

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6
Q

4 causes of foetal / neonatal anaemia

A

twin to twin transfusion
foetal to maternal transfusion
parvovirus infection
haemorrhage from cord / placenta

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7
Q

when does the first leukaemia genetic hit occur

A

in utero

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8
Q

who gets congenital leukaemia (aka transient abnormal myelopoiesis)

A

downs syndrome

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9
Q

how is congential leukaemia different from normal leukaemia

A

it spontaneously remits

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10
Q

what cell type is involved in congential leukaemia

A

myeloid - megakaryocytes

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11
Q

newborn babies vs adults have got what different in their blood film ?

A

a higher Hb

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12
Q

what is thalassaemia

A

reduced rate of synthesis of one type of haemaglobin

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13
Q

what is haemaglobinopathy

A

synthesis of a structurally abnormal molecule of haemaglobin
or
can include thalassaemias too

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14
Q

why do defects in alpha / beta haemaglobinopathies occur at different ages?

A

alpha synthesis starts in utero so will show up then
beta synthesis starts after birth so newborn babies wont have it

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15
Q

what chromosome has beta hb encoded

A

11

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16
Q

what chromosome has alpha hb encoded

A

16

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17
Q

how does sickle cell cause blockage of BVs

A

regular haemaglobin is donut shaped
if it has the sickle cell gene, it will turn into sickle shape when exposed to HYPOXIA
these aggregate and block BVs

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18
Q

is sickle cell trait part of sickle cell disease

A

no - it causes subclinical sx if any

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19
Q

if both parents have sickle trait, what % of children will have:
- sickle trait
- sickle cell
- normality

A

50% trait
25% sickle cell
25% normal

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20
Q

how is sickle cell diagnosed at birth

A

guthrie spot

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21
Q

why do kids get sickle crises in hand / feet but adults dont

A

kids have extension of red marrow further into limbs - these are very vascular areas so more likely to get sickle occlusion –> leads to hand-foot syndrome where thrombosis in periperhies, which you don’t get in adults

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22
Q

how can sickle cell manifest differently in kids and adults

A

hand-foot syndrome in kids - longer red marrow
splenic sequestration - spleen is still functioning, so can get sickle cells sequestering
stroke - thinner BVs in kids

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23
Q

why doesn’t splenic sequestration happen in older children / adults

A

splenic infarction occurs so much that the spleen has become small and fibrotic

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24
Q

what risk increases as splenic sequestration risk decreases

A

hyposplenism - bacterial infections etc

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25
Q

what 2 infections are particularly bad in sickle babies

A

pneumococcal
parvovirus

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26
Q

why does folic acid matter more in a child with sickle cell than in a normal child / adult

A

hyperplastic erythropoiesis and growth spurts requires folic acid

27
Q

what is the lifespan of a sickle red cell

A

20 days

28
Q

how do you manage sickle cell and complications in infant / child

A

accurate diagnosis
educate parents - sickle cell crisis signs
vaccinate - pneumococcus
prescribe folic acid / penicillin

29
Q

is stroke more common in sickle kids or adults

A

kids - smaller BVs in brain

30
Q

siblings with sickle cell anaemia present together with severe anaemia, low reticulocyte count. Dx?

A

parvovirus b19 infection

31
Q

6 year old afro caribean boy with chest / abdo pain. hb 63, mcv 85 and blood film shows sickle cells. is this trait/anaemia/sickle with beta thalassaemia ?

A

sickle cell anaemia

32
Q

what blood film feature is seen in sickle cell with beta thalassaemia

A

microcytosis

33
Q

what is beta thalassaemia

A

condition resulting from reduced synthesis of beta globin chain and therefore haemaglobin A

34
Q

when does beta thalassaemia first present

A

3-6 months

35
Q

what is prognosis of beta thalassaemia heterozygosity/trait

A

harmless but genetically important

36
Q

what is prognosis of beta thalassaemia homozygosity

A

severe anaemia, that in the absence of transfusion, is fatal in first few years of life

37
Q

what is the name for the intermediate forms of beta thalassaemia

A

beta thalassaemia intermedia

38
Q

clinical effects of poorly treated thalassaemia

A

anaemia - HF, growth retardation
erythropoietic drive - bone expansion, hepatomegaly, splenomegaly
iron overload - HF, gonadal failure

39
Q

Mx of infant / child with beta thalassaemia major

A

accurate diagnosis
family counselling
blood transfusion
chelation therapy once iron overload occurs

40
Q

name 2 chelation therapies

A

desferioxamine
deferiprone

41
Q

are all congenital haemolytic anaemias inherited? why?

A

no - can get transplacental passage of ABs causing haemolytic disease of newborn

42
Q

what causes transplacental haemolytic anaemia

A

ABO or RH D ABs mismatch

43
Q

what causes inhertied haemolytic anaemias in children

A

defects in:
red cell membrane (spherocytosis/elliptocytosis)
Hb molecule (sickle cell)
red cell enzymes - glycolytic pathway (pyruvate kinase def) / pentose shunt (G6PD def)

44
Q

signs of haemolytic anaemia in newborn

A

jaundice
splenomegaly
increased unconjugated BR

45
Q

why is high BR an issue in babies

A

can lead to conikterose

46
Q

name 5 inherited haemolytic anaemias in children

A

hereditary spherocytosis
hereditary elliptocytosis
sickle cell
pyruvate kinase deficiency
G6PD deficiency

47
Q

what should be avoided in G6PD deficiency

A

moth balls - naphthalene
fava beans (broad beans)
some drugs
infection

48
Q

2 important acquired haemolytic anaemias in kids

A

autoimmune haemolytic anaemia
haemolytic uraemic syndrome

49
Q

characteristics of AI HA

A

spherocytosis
positive coombs test

50
Q

characteristics of hamolytic uraemic syndrome

A

haemolysis
uraemia (kidney impairment)
small angular fragments and microspherocytes –> schistocytes

51
Q

what precipitates haemolytic uraemic syndrome in kids

A

a specific e coli

52
Q

presentation of haemophilia in kids

A

boys
bleeding following minor ops eg circumcision
haemarthroses when starting to walk
bruises

53
Q

Mx of haemophilia in kids

A

accurate Dx
counselling of family
Tx of bleeding episodes
use of prophylactic coagulation
involve school / family

54
Q

mucosal bleeding, bruises and post traumatic bleeding. Dx?

A

von willebrand disease

55
Q

Tx of von willebrand factor

A

lower purity factor Viii concentrates

56
Q

1 year old boy presents with joint bleeding
hb, wcc, platelet normal
aptt long
pt, bleeding time normal
Dx?

A

haemophilia A

57
Q

which is more likely of the haemophilias

A

A

58
Q

petechiae, bruises and blood blisters in mouth. dx?

A

ITP

59
Q

Dx of ITP

A

history
blood count and film
aspirate if needed

60
Q

Mx of ITP

A

observation
steroids
high dose IV IG
IV anti Rh D (if positive)

61
Q

what leukaemia is more common in kids

A

ALL

62
Q

what leukaemia is more common in infants under 1

A

AML

63
Q

Mx of hyposplenism

A

vaccinations
prophylactic penicillin
advice to parents re other risks - malaria, dog bites

Pathology (87 decks)

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