Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Y5 Path: Haematology > Paediatric Haematology > Flashcards

Paediatric Haematology Flashcards

1
Q

How does sickle cell disease differ in neonates/children compared to adults?

A
  1. Children have red bone marrow in hands/feet - these are additionally susceptible to infarction by sickle cells
    • Hence infants can get Hand/foot syndrome - infarction of meta-tarsals in hand/feet
    • Occurs in first 2 years of life
  2. Infants have functioning spleen, so can get SPLENIC SEQUESTRATION (first 3yrs of life)
    • Severe anaemia, shock, death
    • Older age -> recurrent infarction of spleen -> small fibrotic spleen -> HYPOSPLENISM
  3. Infants have immature immune system - susceptible to PNEUMOCOCCUS and PARVOVIRUS infection
    • ​​1st exposure to these lead to pure red cell aplasia
    • This is problematic in sickle cell children because sickle cells have 10 day lifespan, whereas normal RBCs are 120 days. Hence you get acute anaemia.
  4. Infants are growing rapidly - Greater need for FOLIC ACID
    • ​Hence on diagnosis infants are started on folic acid for life
    • 3 reasons:
      • Hyperplastic erythropoeisis requires folic acid
      • Growth spurts require folic acid
      • Red cell life-span is shorter so can get anaemic very quickly if folic acid deficient
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Management principles of sickle cell in infants/child?

A
  1. Accurate diagnosis
  2. Educate parents
  3. Vaccinate
  4. Folic acid + Penicillin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Complications of sickle cell more common in children than adults:

A
  • Splenic sequestration
  • Red cell aplasia (Pneumococcus, parvovirus)
  • Hand-foot syndrome
  • Stroke
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Siblings with SCA present simultaneously with severe anaemia and low reticulocyte count - likely diagnosis?

A

Parvovirus B19 first exposure -> Red cell aplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

6yo Afro-Caribbean boy presents with chest + abdo pain.

Hb 63, MCV 85, blood film shows sickle cells.

Likely diagnosis?

  1. Sickle cell trait
  2. Sickle cell anaemia
  3. Sickle cell/beta thal
A

Sickle cell anaemia

Not sickle cell trait because you have sickle cells in blood film

Not b-thal because you would have microcytosis - MCV 85 is normal for his age (slightly lower range in children)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

When does beta thalassaemia first present?

A

3-6 months of life

This is when Haemoglobin A production takes over Haemoglobin F in newborn.

Beta globin chain = affects Haemoglobin A production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Types of B-thal

A

B-thal TRAIT = one B-thal gene

B-thal MAJOR = two B-thal genes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Clinical features of poorly treated B-thal major

A
  • Anaemia -> Heart failure, growth retardation
  • Erythropoietic drive -> bone expansion, hepatosplenomegaly
  • Iron overload -> Heart failure, gonadal failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Management of B-thal?

A
  1. Accurate diagnosis + Family counselling
  2. Blood transfusion
  3. Iron chelation therapy (desferioxamine, deferiprone)
  4. Consideration of child as an individual and part of a family
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Types of inherited haemolytic anaemias in children

A
  • Red cell membrane defects
    • Hereditary spherocytosis
    • Hereditary elliptocytosis
  • Haemoglobin defects
    • Sickle cell anaemia
  • Glycolytic pathway defect (pathway that provides energy to red cells)
    • Pyruvate kinase deficiency
  • Pentose shunt defect
    • G6PD deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

7yo Afro-caribbean boy presents with abdo pain + urinary Sx, was given an anti-emetic by GP

3 days later he presents with jaundice and brought to hospital

WCC 10.9, Hb 58, MCV 100, Pt 275

Likely diagnosis?

  1. Hep A
  2. Hep B
  3. Hereditary spherocytosis
  4. Sickle cell anaemia
  5. G6PD deficiency
A

G6PD deficiency

On blood film you would see irregularly contracted red cells - spleen takes a ‘bite’ out of them.

MCV on upper end of normal due to reticulocytosis as a result of haemolysis

Unlikely to be hereditary spherocytosis or SCA because they would have presented earlier in life

G6PD def - haemolysis can be triggered by INFECTION or oxidant DRUGS - in this case a UTI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What advice do you give to a mother who has G6PD def.?

A

These things can trigger haemolysis so be aware:

  1. Infections
  2. Drugs
  3. Napthalene
  4. Fava beans
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Epidemiology of G6PD def.?

A

Prevalent in northern europe/africa - thought to protect against malaria

Caucasians unlikely to have it

X-linked - so males

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Examples of acquired haemolytic anaemias?

A
  • Autoimmune haemolytic anaemia
    • Malignant - Lymphoma
    • Non-malignant - SLE
  • HUS (Haemolytic uraemic syndrome)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Examples of common inherited coagulation disorders in children?

A
  • Haemophilia A & B
  • Von Willebrand disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How might haemophilia A/B present?

A

These would only be in BOYS because factor 8/9 are X-linked

  • Bleeding after circumcision
  • Haemoarthroses when starting to walk
  • Bruises
  • Post-traumatic bleeding
17
Q

Differentials for haemophilia A/B?

A
  • Inherited platelet disorder (thrombocytopenia)
  • Acquired coagulation defects
    • ITP
    • Acute leukemia
  • Non-accidental injury (safeguarding)
  • Henoch-Schonlein Purpura
    • Bleeding into shins
    • Coagulation + platelets NORMAL
18
Q

Investigations for inherited coagulation defects in children?

A
  • History
    • Bleeding from umbilical cord or from Guthrie spot
    • Haematoma after vaccinations/vitamin K injections
    • Bleeding after circumcision?
  • FMH (X-linked?)
  • Coagulation screen
    • Haemophilia A/B = Abnormal APTT (Intrinsic pathway - F8/9)
  • Platelet count
  • Assays of specific coagulation factors
    • F8/9 for Haemophilia A/B
19
Q

How might Von Willebrand disease present?

A
  • Mucosal bleeding
  • Bruises
  • Post-traumatic bleeding
20
Q

Differentials for VWD?

A

Haemophilia A, because F8 levels are reduced in both

21
Q

Investigations/Diagnosis for VWD?

A
  • Autosomal dominant so FMH
  • Coagulation screen
    • Prolonged APTT
  • Factor 8 assay
  • Platelet aggregation studies
22
Q

Treatment of VWD?

A

Low purity F8 concentrates

23
Q

1yo boy presents with joint bleeding

Hb, WCC, Pt normal

Prolonged APTT, normal PT

Bleeding time normal

Likely diagnosis?

  1. Haemophilia A
  2. Haemophilia B
  3. VWD
  4. TTP
  5. Boy took mum’s warfarin tablets
A

Haemophilia A

Could be either A or B, but A is 5x more common, hence more likely diagnosis.

VWD unlikely as bleeding time is normal

TTP would not give you a coagulation abnormality

Warfarin would prolong PT, not APTT

24
Q

How would ITP present?

A
  • Petechiae
  • Bruises
  • Blood blisters in mouth
25
Q

Investigations for ITP?

A
  • History of febrile illness week or so before
  • Blood count + film
26
Q

Management of ITP?

A
  • Observation
  • Corticosteroids
  • High dose IVIG
  • IV anti-D (if Rh +ve)

Y5 Path: Haematology (3 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions