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PATHOLOGY > Paediatric haematology > Flashcards

Paediatric haematology Flashcards

1
Q

Differences in blood results in children than adults

A

Children:
* higher lymphocyte
* Lower Hb
* Lower MCV

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2
Q

why is deficiency more common in children - and what deficiencies

A

rapid growth

iron deficency common on childhood during growth spurt, esp if suboptimal diet

folic acid - increased needs when growing

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3
Q

how does response to disease differ in children vs adults

A

children more likely get lypmocytosis rather than neutrophilia post-infection

reactive lymphocytes (large, basophilic cytoplasm) are common because of the frequent encounters with new microbial antigens

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3
Q

how does response to disease differ in children vs adults

A

children more likely get lypmocytosis rather than neutrophilia post-infection

reactive lymphocytes (large, basophilic cytoplasm) are common because of the frequent encounters with new microbial antigens

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4
Q

How does the fact that children are growing alter illness

A

growth retardation can occur
either from:
1. general effects of severe illness and rx
2. adverse effects of specifici methods of rx - spinal irradiation (treat CNS leukaemia) - not used now

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5
Q

effect of disease and treatment on development

A

can lead to failure to experience puberty -> growth retardation and failure to develop 2ndary sex characteristics

In B thal major - treat with transfusion – likely get iron overload – deposition in liver, heart and other organs

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6
Q

difference in blood count in neonate VS adult

A

all higher in neonate (WBV, neutrophil, lymphocyte, Hb, MCV)

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7
Q

red cells in neonate vs adult

A

neonate - more HbF (so disorder of B globin genes are less likely to manifest until 3mo)

enzyme levels in red cells differ - glucose-6-phosphate dehydrogenase (GSPD) conc is higehr

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8
Q

In utero event in twins that can have haematological effect

A

Twin-twin transfusion syndrome

Identical twins – shared placental circ – one transfuse to other – one polycythaemic and one anaemic

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9
Q

what can cause polycythaemia in fetus

A

twin-twin transfusion
intrauterine hypoxia - fetus increase epo synthesis
placental insufficiency -> hypoxia

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10
Q

causes of anaemia in fetus

A

Twin-twin transfusion

fetal-maternal transfusion (normal for a small amount to go to maternal circ, if a lot = fetal anaemia)

parvovirus infection

haemorrhage from cord/placenta

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11
Q

what can impact the intrauterine fetus

A

irradiation

substance pass from mum to baby circ (drugs, chemicals, abs)

anticoagulants -> haemorrhage, fetal deformity

ab against fetal ag (from dad) -> destroy red cells, white cells or plts

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12
Q

eg of fetus harmed by substance absorbed from breast milk

A

if lactating woman eats fava beans and baby G6PD deficient - may suffer haemolysis

drugs

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13
Q

How can the intrauterine environment effect later childhood (leukaemia)

A

first mutation that -> leukaemia occurs in utero

pre-leukaemic cells carrying mutation spread from one twin to the other

congenital leukaemia is common in Down syndrome
* This specific type of neonatal leukaemia (also sometimes called transient abnormal myelopoiesis or TAM) differs greatly from leukaemia in older infants or children because remits spontaneously
* but 25%o develop one that doesnt remit spont at 2yrs
* myeloid leukaemia - involves megakaryocyte lineage

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14
Q

Definition of thalassamia

A

Condition from reduced rate of synthesis of Hb

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15
Q

definition of haemoglobinopathy

A

Sometimes just means variant Hb ike structurally abnormal molecule

sometimes incorporates thalassaemias

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16
Q

The globin gene

A

On chr 11 and chr 16
LCRB - locus control region beta
LCRA - Locus control region alpha

If lose function of controlling region – lose function of all the genes

on Chr 11 have - embryonic gene, fetal genes, pseudobeta gene, delta (part of HbA2) then B

on chr 16 have - 2 a genes, more pseudogenes and fetal gene (zeta)

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17
Q

What are the normal Hb genes in

A

In adults fetal is only in 1-2%

A2 doesn’t have high levels until 1yr

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18
Q

graph of normal Hb

A
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19
Q

definition of sickle cell disease

A

homozygous and compound heterozygous states that are associated with the pathological effects of sickling
includes
* sickle cell anaemia
* compound heterogenous states - including SC and S/beta thalassaemia

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20
Q

Pathophysiology of SCD

A

RBC normal discoid shape
Exposed to hypoxia
HbS polymerise
cell sickle

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21
Q

Vascular obstruction in sickle cell anaemia

A

normally - red cells elongate to pass through capillary
Sickle – become rigid – adherent – obstruct post capillary venule -> obstruction
-> retrograde capillary obstruction occurs

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22
Q

Blood film for sickle cell anaemia

A
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23
Q

chr for BsBthal

A

can be B0 - where no B chain at all = only S

or B+ where some HbA but less

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24
Q

chr for BBs

A

sickle cell trait

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25
Q

Chr for BsBs

A

sickle cell anaemia

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26
Q

chr for BsBc

A
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27
Q

Genetics for sickle cell

A
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28
Q

finish this

A
29
Q

why does sickle cell anaemia not present at birth

A

become manifest as gamma chain production and haemoglobin F synthesis decrease and betaS and haemoglobin S production increase

However - normally dx at birth - Guthrie spot

30
Q

summarise guthrie test

A

Heal prick test
Drop of blood in the 4 circles – send to lab
Dilute Hb and test it

31
Q

advantage of dx haemoglobinopathies and thalassaemias in neonates

A

complications can be prevented/anticipated

32
Q

why does sickle cell anaemia in infant and child differ from in adult

A

distribution of red bone marrow (susceptible to infarction) differs—the hand/foot syndrome (infarction of small bones)
ie more in limbs and fingers in children

Vasoocclusion - stroke more in 1st 10yrs (cerebral vessels smaller in child)

infant still has spleen -> splenic sequestration can occur

33
Q

Summarise splenic sequestration

A

Rbc sickle and stuck in spleen -> spleen large ->acute anaemia and shock -> death

*Doesn’t happen in adult – because recurrent infarction lead to fibrsosi so cant expand and fill with red cells *

34
Q

risk from hyposplenism

A

spleen is important for filtering out bacteria and parasites

35
Q

sickle cell anaemia and pneumococcus/parvovirus

A

infant has immature immune system - not developed immunity to these

take regular penicillin (prophylactic) and peumococcal vaccination

cant vaccinate against parvovirus

first exposure to parvovirus B19 -> pure red cell aplasia
if pt acutely anaemia and low reticulocyte - make dx of red cell aplasia -> transfuse them

36
Q

Why does folic acid matter more in a child with sickle cell disease than in a normal child or an adult?

A

Hyperplastic erythropoiesis requires folic acid

Growth spurts require folic acid

Red cell life span is shorter so anaemia can rapidly worsen - become acutely anaemic more radidly

37
Q

How do we manage sickle cell disease in children

A

Accurate diagnosis
Educate parents
* Detect splenic sequestration by palpate abdo
* Need to know if become listless and may be anaemic

Vaccinate
Prescribe folic acid and penicillin

38
Q

when does b thalassaemia present

A

3-6mo
When F synth is decreasing and A is taking over

39
Q

complictauons of beta thalassaemia homozygosity

A

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

40
Q

Chr for b thal trait

A
41
Q

chr for b thal homozygosity

A
42
Q

Inheritance of B thal

A

with every preg when both trait chance is 1 in 4

43
Q

clinical effects of poorly treated thalassaemia

A

anaemia -> HF, growth retardation

erythropoietic drive -> bone expansion, hepatomegaly, splenomegaly
*Anaemia stim epo production – extramedullary haemopoesis *

Iron overload -> HF, gonadal failure

44
Q

How do you prevent iron overload when have regular transfusions

A

iron chelating agent

45
Q

How do you mx a child with B thalassaemia major

A

Accurate diagnosis and family counselling
Blood transfusion
Monitoring for narrowing of cerebral vessels
Once iron overload starts to occur, chelation therapy (desferioxamine, deferiprone)
Consideration of the child as an individual and as part of a family

46
Q

summarise congenital haemalytic anaemias

A

most are inherited - not all

transplacental passage of ab -> haemolytic disease of newborn
* ABO/Rh

47
Q

What can inherited haemalytic anaemias be due to

A

defect in:
* red cell membrane
* Hb molecule (sickle)
* Red cell enzymes - glycolytic pathway that provides energy for cells (pyruvate kinase deficiency)
* red cell enzyme - pentose shunt (protection against oxidation damage) (G6PD deficiency)

48
Q

principles of diagnosis of haemolytic anaemia in children

A

Is there anaemia?

Is there evidence of increased red cell breakdown, e.g. jaundice (kernicteris), splenomegaly, increased unconjugated bilirubin?

Is there evidence of increased red cell production, e.g. increased reticulocyte count, bone expansion?

Are there abnormal red cells?

49
Q

red cell membrane defects -> haemolytic anaemia

A

hereditory spherocytosis
hereditory elliptocytosis

50
Q
A

Spherocytosis

51
Q
A

elliptocytosis

52
Q
A

sickle cell

53
Q

advice for G6PD

A

Infections may precipate haemolysis
Drugs – need to not buy OTC drugs that include them
Naphthalene
Fava beans – oxidant and should be avoided. Falaffal may include beans

54
Q

Two important acquired haemolytic anaemias in children

A

autoimmune haemolytic anaemia
* Spherocytosis
* Positive direct antiglobulin test (Coombs’ test)

haemolytic uraemic syndrome
* haemolysis
* uraemia

55
Q

Pathophysiology of haemolytic anaemia in children

A

microangiopathic haemolytic anaemia

red cells damaged in capillaries -> fragmented

Small angular fragments and microspherocytes are formed
Collectively - Schistocytes – cleaved/fragmented cells

56
Q

film for haemolytic uraemic syndrome

A

usually follows E coli infection

57
Q

what are the inherited defects of coagulation

A

Haemophilia A
haemophilia B
Von Willebrand disease

58
Q

presentation of haemophilia

A

Bleeding following circumcision
Haemarthroses when starting to walk
Bruises
Post-traumatic bleeding

59
Q

ddx for inherited defects of coagulation

A

Inherited thrombocytopenia or platelet functional defect

Acquired defects of coagulation, e.g. autoimmune thrombocytopenic purpura (‘ITP’), acute leukaemia

Non-accidental injury

Henoch‒Schönlein purpura

60
Q

Ix for inherited defect of coagulation

A

History of child
* umbilical cord bleeding or bleeding with Guthrie spot?
* haematoma formation after vaccinations?
* bleeding after circumcision?

Family history (X-linked recessive or not)
Coagulation screen
Platelet count
Assays of specific coagulation factors

61
Q

Mx of inherited defect of coagulation

A

Accurate diagnosis
Counselling of family
Treatment of bleeding episodes
Use of prophylactic coagulation factors
Consideration of the child as an individual and as a family member (home treatment, self-treatment, schooling)

62
Q

presentation of von willebrand disease

A

Mucosal bleeding
Bruises
Post-traumatic bleeding

63
Q

ix for von willebrand disease

A

Family history (mainly autosomal dominant)
Coagulation screen
Factor VIII assay
? Bleeding time (maybe not) - prolonged
Platelet aggregation studies

64
Q

Rx of von willebrand disease

A

Lower purity factor VIII concentrates
*So get vw factor – not just factor 8 *

65
Q

presentation of immune thrombocytopenic purpura

A

Petechiae – pin point haemorrhages
Bruises
Blood blisters in mouth

66
Q

ddx of autoimmune thrombocytopenia

A

Henoch‒Schönlein purpura
Non-accidental injury
Coagulation factor defect
Inherited thrombocytopenia
Acute leukaemia

67
Q

Ix for autoimmune thrombocytopenic purpura

A

History
Blood count and film
Bone marrow aspirate (only if there is some very good reason)

68
Q

Rx of autoimmune thrombocytopenic purpura

A

Observation
Corticosteroids
High dose intravenous immunoglobulin
Intravenous anti-Rh D (if Rh-positive) – can ameliorate the condition

69
Q

summarise acute leukaemia in children

A

mainly acute lymphoblastic leukaemia

can get acute myeloid leukaemia (more common than ALL <1yr)

commonest childhood malignancy

PATHOLOGY (94 decks)

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