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Child health > Haematology 3 > Flashcards

Haematology 3 Flashcards

1
Q

Classify thrombocytopenia by severity

A

Severe - (platelets <20 x 109/L) - risk of spontaneous bleeding

Moderate thrombocytopenia (20-50 x 109/L) -at risk of bleeding during operation or trauma

Mild (50-150) - low risk of bleeding, unless major operation or severe trauma

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2
Q

Symptoms of thrombocytopenia

A

Bruising
Petechiae
Purpura
Mucosal bleeding

Epistaxis
Bleeding from gums when brushing teeth.

Less common:
severe Gi haemorrhage
Haematuria
Intracranial bleeding

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3
Q

What is the significance of purpura?

A 
May signify thrombocytopenia
or
platelet dysfunction
or 
vascular disorders (eg vasculitis)
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4
Q

Which conditions cause thrombocytopenia via platelet destruction?

A

Immune: ITP
SLE

Haemolytic uraemic syndrome
Thrombotic thrombocytopenic purpura
DIC
Congenital heart disease

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5
Q

Presentation of immune thrombocytopenia (ITP)

A

Between 2-10 yrs

Onset often 1-2 weeks after viral infection

Petechiae
Purpura
Superficial bruising

Can cause epistaxis and mucosal bleeding

Profuse bleeding uncommon
Very rarely intracranial bleeding

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6
Q

How is Immune thrombocytopaenic purpura diagnosed?

A

Diagnosis of exclusion

Pay careful attention to the history, clinical features and blood film (eg. congenital causes in younger children)
eg. ALL, SLE

Bone marrow examination if treatment with steroids (could mask ALL)

If the only abnormality in the blood is low platelet, no need to examine bone marrow (unless steroids)

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7
Q

Which factor is affected in haemophilia

A

FVIII

IX:C in haemophilia B

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8
Q

Know the severities of Haemophilia

A

Graded depending on factor VIII:C

Mild - 5-40% - bleed after surgery

Moderate - 1-5% - bleed after minor trauma

Severe - <1% - spontaneous joint/muscle bleeds

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9
Q

Presentation of haemophilia

A

Severe - recurrent spontaneous bleeding into joints and muscles - can lead to crippling arthritis if not properly treated

Towards end of first year of life: when crawling starts

40% present in neonatal period: intracranial haemorrhage, bleeding post-circumcision or prolonged oozing from feel stick site

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10
Q

Clinical features of DIC

A

Acute or chronic

Bruising
Purpura
Haemorrhage

Microvascular thrombosis - purpura fulminans may occur

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11
Q

Acute management of bleeding disorders

A

Most can be managed at home
Treatment only given if evidence of major bleeding, or persistent minor bleeding such as excessive epistaxis or menstrual bleeding (otherwise not, even if platelets <10x10^9/L)

Options (all have significant side effects)
Oral prednisolone
IV anti-D
IV immunoglobulin

Platelet transfusion is reserved for life-threatening haemorrhage - buys a few hours

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12
Q

Advice to children with bleeding disorders

A

Avoid trauma as far as possible

Avoid contact sports while platelet count very low

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13
Q

Management of chronic immune thrombocytopaenic purpura

A

20% of children have it chronically (= low platelets after 6 months)

Supportive
Drug treatment only if persistent bleeding that affects daily life:
Rituximab (monoclonal antibody against B lymphocytes)

Splenectomy can be effective - reserved for children who fail drug therapy (increase in risk of infection and needs lifelong antibiotic prophylaxis)

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14
Q

What is von Willebrand’s disease

A

Deficiency of von Willebrand factor (responsible for platelet adhesion and carries FVIII)

Many subtypes
Type 1 is commonest and is fairly mild:
Diagnosed in puberty/adulthood

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15
Q

Clinical features of von Willebrand disease

A

Bruising
Prolonged bleeding after surgery
Mucosal bleeding

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16
Q

Treatment of von Willebrand disease

A

DDAVP causes secretion of FVIII and vWF

If patient <1 year - it can cause hyponatraemia and seizures if fluid intake not adequate

AVOID:
aspirin
IM injections
NSAIDs

17
Q

What are the main acquired conditions of coagulation

A

Haemorrhagic disease of the newborn (Vit K deficiency)

Liver disease

ITP
DIC
Inadequate intake, malabsorption of Vit K

18
Q

Which bleeding disorders present in neonates

A

20% of haemophilia

19
Q

Which bleeding disorders present in toddlers

A

Haemophilias may present when walking

20
Q

Which bleeding disorders present in adolescents

A

vWD with menorrhagia and FHx

21
Q

What does a FHx of all boys with bleeding suggest?

A

Haemophilia

22
Q

What does a bleeding disorder with mucous membrane and skin involvement suggest?

A

platelet disorder

vWD

23
Q

What does a bleeding into muscles/joints suggest

A

haemophilia

24
Q

Characteristics of Henoch-schonlein purpura (non-thrombocytopaenic)

A

Lesions confined to buttocks, extensor surfaces of legs and arms

Swollen painful knees and ankles

Abdominal pain

Haematuria

Haematuria

25
Q

The genetics of haemophilia

A

A (commoner) and B have X-linked recessive inheritance

A - FVIII deficiency
B - FIX deficiency

2/3 have a FHx
1/3 is sporadic

26
Q

Clinical features of haemophilia

A

Recurrent spontaneous bleeding into joints and muscles - can lead to crippling arthritis

Presentation around 1 year age (crawling)

Suspect NAI if no FHx

40% present as neonate - intracranial haemorrhage, bleeding post circumcision, oozing from heel stick/venepuncture site

27
Q

Acute management of haemophilia

A

Prompt iv infusion of recombinant FVIII/FIX for haemophilia A/B

Raising it to 30% of normal is usually sufficient to treat minor bleeds

For major surgery or life-threatening bleeds, it needs to be 100%. Maintenance at 30-50% is required for up to 2 weeks to prevent secondary haemorrhage - needs regular infusion of factor concentrate (FVIII - 8-12hrs; FIX - 12-24hrs)
Monitor plasma levels

28
Q

What should be avoided in all patients with haemophilia?

A

Intramuscular injections
Aspirin
NSAIDs

29
Q

Home treatment in haemophilia

A

To avoid delay in treatment! Risk of permanent damage

Parents are taught to give therapy at home when child is 2-3yrs of age

Many children can administer their own treatment from 7-8 yrs

Psychosocial support
Self-help groups
Specialised physiotherapy to preserve muscle strength

30
Q

Prophylaxis in severe haemophilia

A

Prophylactic FVIII given in all children with severe haemophilia A to reduce risk of chronic joint damage

Begins at age 2-3yrs (2-3 times per week)

If peripheral venous access poor, central venous device (eg. portacath)

Prophylaxis has been shown to better joint function in adult life

Similarly, in severe haemophilia B, prophylactic FIX is given

31
Q

What can be used in mild haemophilia instead of blood products?

A

Desmopressin (DDAVP)

Allows mild haemophilia A to be managed without the use of blood products

Given by infusion - stimulaters endogenous release of FVIII:C and vWF

Adequate levels for minor surgery and dental extraction

DDAVP is INEFFECTIVE in haemophilia B

Child health (51 decks)

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