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Sarah Paeds > Haematology > Flashcards

Haematology Flashcards

(85 cards)

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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
When does haemolysis become an anaemia?
When BM erythropoeisis cannot compensate for the haemolysis
26
Where does extravascular haemolysis take place?
Liver, spleen
27
Two examples membrane disorders leading to haemolysis
Hereditary spherocytosis | Hereditary elliptocytosis
28
Presentation of hereditary spherocytosis
Anaemia Jaundice Mild splenomegaly Gallstones
29
Investigations for hereditary spherocytosis
Film - spherocytes Osmotic fragility test DAT -ve
30
Mx hereditary spherocytosis
Neonates: supportive, folic acid. Phototherapy if significant jaundice ``` Non-neonate: Supportive care +/- transfusion. Folic acid Splenectomy + vaccine (encapsulated) Cholecystectomy ```
31
Mode of inheritance hereditary spherocytosis
AD
32
What is the defect in hereditary spherocytosis
Spectrin gene - membrane molecule
33
What type of bacteria ae vaccinated against if splenectomy?
Encapsulated
34
Example of enzyme disorder causing haemolysis
G6PD
35
Which kind of haemolysis does G6PD result in?
Intravascular
36
Populations with high prevalence G6PD
Central Africa, Med, Middle East
37
Inheritance pattern G6PD
X linked
38
Presentation of G6PD
Neonatal jaundice <3 days Jaundice, pallor, malaise, dark urine (Hb)
39
Triggers for acute haemolysis in G6PD
Infection Drugs (e.g. quinines and quinolones) Fava beans Naphthalene
40
Ix G6PD
Measure G6PD levels during STEADY STATE (levels can rise during acute haemolysis)
41
Mx G6PD
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
When do clinical manifestations of disorders of the B globin chain occur? Why?
Approx 6 months - HbF being replaced by HbA
43
What is the mutation in sickle cell?
Chr 6 B globin gene Mutation substituting glutamine for valine = dehydration, polymerisation and sickling
44
What is the prognosis for patients with sickle cell?
40% die before 50 years
45
Common populations with sickle cell
Africa | Caribbean
46
Prophylactic management SCD
Immunisations against encapsulated bacteria Penicillin OD Folic acid Avoid triggers
47
Acute crises management SCD
``` IV analgesia O2 Warming IV abx Hydration Exchange transfusion (acute chest, priapism, stroke) ```
48
Chronic management SCD
Hydroxycarbamide for children with recurrent crises/admission (Monitor for WCC suppression) Consider BM transplant
49
which drugs induce HbF production?
Hydroxycarbamide
50
What should be monitored for patients on hydroxycarbamide?
White cell counts (causes suppression)
51
Inheritance pattern of sickle cell
AR
52
Beta thal populations
Indian sub continent Med Middle east
53
Two types beta thal
Major Trait (+intermedia)
54
Beta thal presentation
Approx 3-6 months (when HbF being replaced by HbA) Profound anaemia Jaundice FTT/growth failure Hepatosplenomegaly Extramedullary haematopoeisis
55
Mx beta thal
``` Blood transfusions (aim to keep above 100g/L) Iron chelation - desferrioxamine ``` Bone marrow transplant - curative, but only performed with HLA identical sibling
56
When is a bone marrow transplant appropriate in beta thal?
If HLA identical sibling
57
Complications of constant blood transfusions in beta thal
Iron overload - cardiac failure, cirrhosis, infertility, growth failure
58
Treatment for iron overload
Iron chelation - desferrioxamine
59
Test for antibody mediated haemolytic anaemias
DAT/Coombs
60
Inheritance pattern haemophilia
XR
61
Comonest haemophilia
A (factor 8)
62
Three grades of haemophilia
Mild Mod Severe
63
Features of haemophilia
Spontaneous, recurrent bleeding into joints/muscles | --> joint damage
64
When does haemophilia typically present?
40% neonates - intracranial haemorrhage, prolonged bleeding from venepuncture, etc. When babies start to crawl/walk
65
Acute mx of bleeding in haemophilia
Recombinant factor 8/9 Antifibrinolytics (TXA) PROMPT IV INFUSION Analgesia Ortho r/v
66
Prophylactic management haemophilia
Prophylactic factor 8 in severe haemophilia A to reduce the risk of joint damage
67
How is the risk of chronic joint damage in haemophilia reduced?
Prophylactic recombinant factor (8)
68
What should be avoided in patients with haemophilia
IM injection Aspirin NSAIDs
69
Mx mild haemophilia
DDAVP - stimulates factor 8 and vWF
70
Which drug stimulates factor 8 and vWF?
DDAVP desmopressin
71
Features of VWD
Mucosal bleeding - epistaxis, menorrhagia Bruising Prolonged, excessive bleeding
72
Mx VWD (mild/severe)
Mild: DDAVP (stimlulated F8 and vWF production) - caution <1 years as can cause hyponatraemia --> seizures Severe: F8 concentrate
73
What should be avoided in VWD
IM injections Aspirin NSAIDs
74
What should be avoided in bleeding disorders?
IM injections Aspirin NSAIDs
75
What caution should be taken prescribing DDAVP for <1 year old
Hyponatraemia --> seizures
76
Commonest cause thrombocytopenia in childhood
ITP
77
Pathophysiology of ITP
Destruction of platelets by circulating anti-platelet IgG antibodies
78
What might be seen in the bone marrow of a child with ITP
Increased megakaryocytes
79
Age of presentation ITP
2-10years (1-2 weeks after viral infection)
80
Presentation of ITP
Petechiae Bruising Purpura
81
ITP diagnosis
Diagnosis of exclusion Leukaemia Aplastic anaemia SLE No abnormality in blood other than a low platelet count
82
Mx ITP
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
When is active treatment indicated in ITP
Major bleeding | - otherwise 80% resolve spontaneously at home
84
Petechiae/purpura DDx
``` HSP ITP Leukaemia Sepsis (DIC) NAI ```
85
DIC mx
TREAT UNDERLYING CAUSE (e.g. sepsis) Supportive care Replace platelets, cryo, FFP Protein C concentrates

Sarah Paeds (20 decks)

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