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Sarah Paeds > Haematology > Flashcards

Haematology Flashcards

1
Q

Anaemia in newborn

A

<140g/L

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2
Q

Anaemia 1-12 months

A

<100g/L

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3
Q

Anaemia 1-12 years

A

<110g/L

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4
Q

Hb types at 4-8 weeks gestation

A

Hb Gower 1, 2

Hb Portland

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5
Q

Where does foetal haematopoeisis occur?

A

Liver

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6
Q

Sites of post natal haematopoeisis

A

Bone marrow

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7
Q

Globin chains in HbF

A

2 alpha 2 gamma

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8
Q

Globin chains in HbA

A

2 alpha 2 beta

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9
Q

Globin chains in HbA2

A

2 alpha 2 delta

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10
Q

What is an increased proportion of HbF after one year indicative of?

A

Some inherited disorder of Hb production

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11
Q

Three broad causes of anaemia

A

Haemolysis (membrane, enzyme, autoimmune)
Reduced red cell production (reduced erythropoeisis, red cell aplasia)
Blood loss

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12
Q

Two causes of red cell aplasia

A

Parvovirus B19

Diamong-Blackfan anaemia

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13
Q

In whom does parvovirus B19 cause red cell aplasia?

A

Those with inherited haemolytic anaemias

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14
Q

Blood features of red cell aplasia

A

Low reticulocyte count, normal BR

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15
Q

Features of Diamond-Blackfan anaemia

A

Presents 2-3 months
Profound anaemia
Short stature
Abnormal thumbs

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16
Q

IDA biochem

A

Low Hb
Low MCV
Hypochromic
Low serum ferritin

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17
Q

IDA Mx

A

Explore underlying cause

Dietary advice - iron rich foods: dark green leafy veg, iron-fortified bread, meat, apricots, prunes. Dietician r/f

Oral iron supps
- Ferrous sulphate 200mg BD/TDS
(Continued for 3 months after resolution to replenish stores)

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18
Q

What should be checked in patients with IDA not responding to treatment

A

Malabsorption e.g. coeliac

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19
Q

How long after resolution of IDA should oral iron be continued for?

A

3 months - replenish stores

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20
Q

How quickly should Hb increase on iron therapy

A

1g/dL / week

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21
Q

Adverse effects of iron treatment

A

Constipation / diarrhoea
Faecal impaction
GI irritation
Nausea

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22
Q

Four categories of haemolytic anaemias

A

Membrane (sphero/elliptocytosis)
Enzyme (G6PD)
Haemoglobinopathies (SCD/b-thal)
Autoimmune

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23
Q

Symptoms haemolytic anaemias

A

Anaemia
Raised BR (UC)
Hepatosplenomegaly (extramedullary haematopoeisisi)

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24
Q

Biochem haemolytic anaemias

A 
Low Hb 
High retics 
Raised BR (UC)
Abnormal cells on film 
Increased red cell precursors 
\+ DAT if autoimmune
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25
Q

When does haemolysis become an anaemia?

A

When BM erythropoeisis cannot compensate for the haemolysis

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26
Q

Where does extravascular haemolysis take place?

A

Liver, spleen

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27
Q

Two examples membrane disorders leading to haemolysis

A

Hereditary spherocytosis

Hereditary elliptocytosis

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28
Q

Presentation of hereditary spherocytosis

A

Anaemia
Jaundice
Mild splenomegaly
Gallstones

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29
Q

Investigations for hereditary spherocytosis

A

Film - spherocytes
Osmotic fragility test
DAT -ve

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30
Q

Mx hereditary spherocytosis

A

Neonates: supportive, folic acid. Phototherapy if significant jaundice

Non-neonate: 
Supportive care +/- transfusion.
Folic acid 
Splenectomy + vaccine (encapsulated)
Cholecystectomy
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31
Q

Mode of inheritance hereditary spherocytosis

A

AD

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32
Q

What is the defect in hereditary spherocytosis

A

Spectrin gene - membrane molecule

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33
Q

What type of bacteria ae vaccinated against if splenectomy?

A

Encapsulated

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34
Q

Example of enzyme disorder causing haemolysis

A

G6PD

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35
Q

Which kind of haemolysis does G6PD result in?

A

Intravascular

36
Q

Populations with high prevalence G6PD

A

Central Africa, Med, Middle East

37
Q

Inheritance pattern G6PD

A

X linked

38
Q

Presentation of G6PD

A

Neonatal jaundice <3 days

Jaundice, pallor, malaise, dark urine (Hb)

39
Q

Triggers for acute haemolysis in G6PD

A

Infection
Drugs (e.g. quinines and quinolones)
Fava beans
Naphthalene

40
Q

Ix G6PD

A

Measure G6PD levels during STEADY STATE (levels can rise during acute haemolysis)

41
Q

Mx G6PD

A

Advise parents to look out for acute haemolysis (pallor, jaundice, dark urine)

Avoid triggers

Supportive care + folic acid in acute haemolysis
Blood transfusion
Renal support

42
Q

When do clinical manifestations of disorders of the B globin chain occur? Why?

A

Approx 6 months - HbF being replaced by HbA

43
Q

What is the mutation in sickle cell?

A

Chr 6 B globin gene
Mutation substituting glutamine for valine
= dehydration, polymerisation and sickling

44
Q

What is the prognosis for patients with sickle cell?

A

40% die before 50 years

45
Q

Common populations with sickle cell

A

Africa

Caribbean

46
Q

Prophylactic management SCD

A

Immunisations against encapsulated bacteria
Penicillin OD
Folic acid
Avoid triggers

47
Q

Acute crises management SCD

A 
IV analgesia 
O2 
Warming 
IV abx 
Hydration 
Exchange transfusion (acute chest, priapism, stroke)
48
Q

Chronic management SCD

A

Hydroxycarbamide for children with recurrent crises/admission
(Monitor for WCC suppression)

Consider BM transplant

49
Q

which drugs induce HbF production?

A

Hydroxycarbamide

50
Q

What should be monitored for patients on hydroxycarbamide?

A

White cell counts (causes suppression)

51
Q

Inheritance pattern of sickle cell

A

AR

52
Q

Beta thal populations

A

Indian sub continent
Med
Middle east

53
Q

Two types beta thal

A

Major
Trait

(+intermedia)

54
Q

Beta thal presentation

A

Approx 3-6 months (when HbF being replaced by HbA)

Profound anaemia
Jaundice
FTT/growth failure

Hepatosplenomegaly
Extramedullary haematopoeisis

55
Q

Mx beta thal

A 
Blood transfusions (aim to keep above 100g/L)
Iron chelation - desferrioxamine

Bone marrow transplant - curative, but only performed with HLA identical sibling

56
Q

When is a bone marrow transplant appropriate in beta thal?

A

If HLA identical sibling

57
Q

Complications of constant blood transfusions in beta thal

A

Iron overload - cardiac failure, cirrhosis, infertility, growth failure

58
Q

Treatment for iron overload

A

Iron chelation - desferrioxamine

59
Q

Test for antibody mediated haemolytic anaemias

A

DAT/Coombs

60
Q

Inheritance pattern haemophilia

A

XR

61
Q

Comonest haemophilia

A

A (factor 8)

62
Q

Three grades of haemophilia

A

Mild
Mod
Severe

63
Q

Features of haemophilia

A

Spontaneous, recurrent bleeding into joints/muscles

–> joint damage

64
Q

When does haemophilia typically present?

A

40% neonates - intracranial haemorrhage, prolonged bleeding from venepuncture, etc.

When babies start to crawl/walk

65
Q

Acute mx of bleeding in haemophilia

A

Recombinant factor 8/9
Antifibrinolytics (TXA)
PROMPT IV INFUSION

Analgesia

Ortho r/v

66
Q

Prophylactic management haemophilia

A

Prophylactic factor 8 in severe haemophilia A to reduce the risk of joint damage

67
Q

How is the risk of chronic joint damage in haemophilia reduced?

A

Prophylactic recombinant factor (8)

68
Q

What should be avoided in patients with haemophilia

A

IM injection
Aspirin
NSAIDs

69
Q

Mx mild haemophilia

A

DDAVP - stimulates factor 8 and vWF

70
Q

Which drug stimulates factor 8 and vWF?

A

DDAVP desmopressin

71
Q

Features of VWD

A

Mucosal bleeding - epistaxis, menorrhagia
Bruising
Prolonged, excessive bleeding

72
Q

Mx VWD (mild/severe)

A

Mild: DDAVP (stimlulated F8 and vWF production) - caution <1 years as can cause hyponatraemia –> seizures

Severe: F8 concentrate

73
Q

What should be avoided in VWD

A

IM injections
Aspirin
NSAIDs

74
Q

What should be avoided in bleeding disorders?

A

IM injections
Aspirin
NSAIDs

75
Q

What caution should be taken prescribing DDAVP for <1 year old

A

Hyponatraemia –> seizures

76
Q

Commonest cause thrombocytopenia in childhood

A

ITP

77
Q

Pathophysiology of ITP

A

Destruction of platelets by circulating anti-platelet IgG antibodies

78
Q

What might be seen in the bone marrow of a child with ITP

A

Increased megakaryocytes

79
Q

Age of presentation ITP

A

2-10years (1-2 weeks after viral infection)

80
Q

Presentation of ITP

A

Petechiae
Bruising
Purpura

81
Q

ITP diagnosis

A

Diagnosis of exclusion

Leukaemia
Aplastic anaemia
SLE

No abnormality in blood other than a low platelet count

82
Q

Mx ITP

A

80% of children - acute, benign, self limiting in 6-8 weeks (home management)

Treatment indicated if evidence of major bleeding:

  • IVIG
  • Corticosteroid
  • Platelet transfusion
  • Antifibrinolytics (TXA)
83
Q

When is active treatment indicated in ITP

A

Major bleeding

- otherwise 80% resolve spontaneously at home

84
Q

Petechiae/purpura DDx

A 
HSP
ITP
Leukaemia 
Sepsis (DIC)
NAI
85
Q

DIC mx

A

TREAT UNDERLYING CAUSE (e.g. sepsis)
Supportive care

Replace platelets, cryo, FFP
Protein C concentrates

Sarah Paeds (20 decks)

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