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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

What are the risk factors for iron deficiency anaemia in children?

A 
Preterm
Low birth weight
Multiple pregnancy
Exclusive breastfeeding>6m
Female
Adolescent
Poverty
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2
Q

What are the causes of iron deficiency anaemia?

A 
Malabsorption: IBD, Coeliac
Malnutrition
Bleeding
Drugs: NSAIDs, Steroids
Inc demand: Growth spurt
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3
Q

What are the signs & symptoms of iron deficiency anaemia?

A 
Asymptomatic
Mood, cognitive, psychomotor changes
Fatigue
SOB
Pica
Pallor (conjunctival)
Angular Stomatitis
Koilonychia
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4
Q

How is iron deficiency anaemia investigated?

A

Bloods: Hb, MCV, MCHC, Clotting, Ferritin, B12 & Folate, CRP

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5
Q

How is iron deficiency anaemia treated?

A

1.5-2mg/kg Ferrous Sulphate daily
Response within 5-10days
Continue for 3m after Hb has normalised

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6
Q

What is Henoch-Schonlein purpura?

A

IgA mediated small vessel vasculitis

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7
Q

What are the signs & symptoms of Henoch-Schonlein purpura?

A

Usually after URTI
TRIAD: Arthritis (large joints), Abdo pain, palpable papular purpuric rash over legs/buttocks

GI: Melena, haematemesis, intussusception, appendicitis, perforation
Renal: Glomerulonephritis, IgA nephritis, proteinuria

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8
Q

What are the investigations for Henoch-Schonlein purpura?

A

Bloods: FBC, U&E
Urinalysis: Haematuria
Renal biopsy if signs

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9
Q

What is the management of Henoch-Schonlein purpura?

A

Most benign & resolve within 6weeks
NSAIDs: Arthritis
Steroids: Arthritis & GI

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10
Q

What is haemolytic disease?

A

Mother is rhesus -ve
Baby rhesus +ve
Mother previously sensitised
Foetal blood crossing placenta causes immune response
Maternal IgG attacks foetal RBC cells causing haemolysis

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11
Q

How does haemolytic disease present?

A

Antenatal: Anaemia on doppler, hydros fetalis
Postnatal: Hydrops fetalis, early jaundice, kernicterus (neuro damage), coagulopathy, hypotonia, hepatosplenomegaly, blueberry muffin
Anaemia

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12
Q

How is haemolytic disease investigated?

A 
Maternal: Rh -ve, inc anti-Rh titre
Cord: Dec Hb & platelets, inc reticulocytes
coomb's test
 neonatal blood fr croup & titre
SBR: 4hrly, glucose, Hb
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13
Q

How is haemolytic disease treated?

A 
Supervision +/Transfusion
After birth check cord bloods
Phototherapy
Supportive therapy
Oral folic acid: 250mcg/kg/day 6mnths
Perform audiology test

Prophylaxis: Anti-D IgG.

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14
Q

What is haemophilia?

A 
Congenital bleeding disorder 
Due to defective production of coagulation factors
Sex-linked recessive inheritance
Types A & B
Type A: Factor 8
Type B: Christmas disease, factor 9
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15
Q

What are the signs & symptoms of haemophilia?

A

A & B indistinguishable
Rarely presents in neonates
Mild: Bleed after trauma/ surgery
Mod: Persistent bleeding after venepuncture, epistaxis, haematuria
Severe: Neonatal bleeding, IC haemorrhage, sport bleeding into joints & muscles
Easy bruising
Haematuria
Joints: Localised tenderness, warmth, swelling, limited RoM, degeneration

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16
Q

How is haemophilia investigated?

A

Bloods: INR(n), APTT(i), factor 8(d), bleeding time & von Willebrand (n)
CT head: IC bleed
USS/ joint x-ray

Type B: x6 less common than type A

17
Q

How is haemophilia managed?

A 
Major bleed: Factor 8/9
Emergency: FFP, Tranexamic acid
Analgesia: NOT NSAIDs (dec platelets)
Minor bleed: Pressure
Mouth bleed: Oral Tranexamic acid 
AVOID: IM injections inc Vit K at birth
Other: Rest, ice, limb splinting, physio

PROPHYLAXIS: IV con injections 3/week in severe disease

18
Q

What are the complications of haemophilia?

A

Chronic arthropathy
Blood bourne virus transmission
Factor 8/9 inhibitor development

19
Q

What is the pathophysiology of sickle cell anaemia?

A

Autosomal recessive in homogenous sickle haemoglobin
Glutamine becomes Valine on beta global chain = HbS
Less soluble, high viscosity
Polymerises when deoxygenated
Can’t flow through small vessels (hypoxia & ischaemia)

20
Q

What are the features of sickle cell anaemia?

A

Infancy: Dactylitis, pallor, infections, hepatosplenomegaly
Young children: Infection, vaso-occlusion, stroke, UA obstruction
Older children: Infection, vaso-occlusion

21
Q

What are the complications of sickle cell anaemia?

A

Vaso-occlusion crisis: Excrutiating pain in bones/joints, hands/feet (dactylitis), precipitated by infections, cold, hypoxia
Mx: Analgesia, warmth, rehydration
Acute chest: SOB, chest pain, cough, yellow sputum
Sequestration: Sickled blood in spleen/lung/liver, can be fatal
Avascular necrosis: Hip
Renal impairment: Hyposthenuria
Priapism: Painful erectile dysfunction
Retinopathy: Small vessel occlusion
Crisis:
Stroke: 5-10yr, asymptomatic infarcts

22
Q

How is sickle cell anaemia investigated?

A 
Clinical suspicion
Blood film
Haematology: Hb 5-9
Hb electrophoresis: Sickled cells
Prophylaxis: Routine screening & heel prick test
23
Q

How is sickle cell anaemia managed?

A 
Hydration 150% normal
Analgesia: Paracetamol, NSAIDs, Opiates
Abx: If pyrexial- Cefotaxime. Add Clarithromycin if respiratory signs
Oral folate
Oxygen: Sats >95%
Vaccinations
Blood products: Crises
BM transplant
24
Q

What is thalassaemia?

A

Types: α-thalassaemia, β-thalassaemia major & minor
Autosomal Recessive
Decreased/absence of synthesis of 1 or 2 (α or β) chains that form adult human haemoglobin (HbA, α2/β2)
Results in reduced Hb in RBCs and anaemia.
HbA, the most common form of adult Hb, has 2 α & 2 β chains.
Fetal (HbF) has 2 α & 2 γ components (predominant type of Hb before birth).

25
Q

Describe the different forms of α-thalassaemia

A

Genotype -α/–: Anaemic, very low MCV & MCH; splenomegaly, variable bone changes.
Major/Hb Bart’s: Genotype –/–, No α chains produced. Incompatible with life- Fetalis Hydrops
The more α chains the fewer symptoms

26
Q

Describe the different forms of β-thalassaemia

A

Trait: Clinically asymptomatic
Minor: Anaemia, very low MCV & MCH; splenomegaly, variable bone changes, variable transfusion dependency.
Major: S.haemolytic anaemia, very low MCV & MCH; hepatosplenomegaly, chronic transfusion dependency.

27
Q

How soon do symptoms develop after birth with the different types of thalassaemia?

A

α: At birth

β: Several months after birth- 5yo: When γ chain production ceases and the β chains fail to form in adequate numbers.

28
Q

In what type of thalassaemia can Heinz bodies be seen?

A

α thalassaemia

29
Q

What may be the symptoms seen in β-thalassaemia major?

A 
Failure to thrive
Vomiting feeds
Sleepiness
Irritability
Stunted growth
30
Q

What complications can be seen in TREATED thalassaemia?

A

Growth restriction

Iron overload: Endocrinopathy with diabetes, thyroid, adrenal and pituitary disorders.

31
Q

What are the signs of severe thalassaemia?

A

Hepatosplenomegaly.
Bony deformities (frontal bossing, prominent facial bones, and dental malocclusion).
Marked pallor and slight to moderate jaundice.
Exercise intolerance, murmur/HF secondary to s. anaemia.

32
Q

What are the investigations done for someone suspected of having thalassaemia?

A 
FBC: Microcytic, hypochromic anaemia
↑ WCC
↑ Iron & Ferritin
↓ MCV & MCH
Haemoglobin electrophoresis- DIAGNOSIS
Skeletal survey 
ECG
HLA typing: If BM transplant required
Vision, hearing, U&E: Check iron levels 
PCR
33
Q

How is thalassaemia managed?

A

Education & support
Genetic counselling offered
Avoid iron rich food inc tea & coffee
Vitamins: E, C, Folic acid
Desferrioxamine: Aid iron excretion
Intermedia: Close monitoring, occasional blood transfusion
Major: Regular hypertransfusion (maintain Hb >9.5 g/dL)
Iron chelation to prevent overload syndrome
Care by MDT
Consider splenectomy if hypersplenism

34
Q

What are the complications associated with thalassaemia?

A 
Iron overload
Endocrine dysfunction: hypogonadotrophic hypogonadism, short stature, acquired hypothyroidism, hypoparathyroidism, DM
High output HF
Osteoporosis
Transfusions: BBV (Hep B & C)
Hepatocellular carcinoma
Gout

Paediatrics (26 decks)

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