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PAEDIATRICS > HAEMATOLOGY PAEDS > Flashcards

HAEMATOLOGY PAEDS Flashcards

1
Q

Difference between adult haemoglobin and fetal haemoglobin

A

HbF has 2 alpha and 2 gamma subunits
HbA has 2 alpha and 2 beta subunits

Affinity to oxygen: HbF > HbA (demonstrated by oxygen dissociation curve)

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2
Q

Sickle cell disease and HbF

A

Genetic abnormality in sickle cell = the beta subunit
There is no beta subunit in HbF

Hydroxycarbamide - can be used to increase production of fetal haemoglobin in patients with sickle cell anaemia. Protective against sickle cell crises and acute chest syndrome

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3
Q

Causes of anaemia in infancy

A

Physiologic anaemia of infancy (most common)

Other:
Anaemia of prematurity 
Blood loss 
Haemolysis 
Twin-twin transfusion (blood unequally distributed between twins that share placenta)
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4
Q

Causes of haemolysis in a neonate

A

Haemolytic disease of the newborn (ABO/rhesus incompatibility)
Hereditary sperocytosis
G6PD deficiency

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5
Q

Causes of Microcytic anaemia

A 
(TAILS) 
Thalassaemia 
Anaemia of chronic disease 
Iron deficiency anaemia 
Lead poisoning 
Siberoblastic anaemia
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6
Q

Causes of Macrocytic anaemia

A

Megalobastic
- B12/folate deficiency

Normoblastic

  • alcohol
  • reticulocytosis
  • hypothyroidism
  • liver disease
  • drugs e.g. Azathioprine
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7
Q

Causes of normocytic anaemia

A 
(3 As, 2 Hs) 
Anaemia of chronic disease 
Acute blood loss 
Aplastic anaemia 
Haemolytic anaemia 
Hypothyroidism
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8
Q

Generic Symptoms of Anaemia

A 
Tiredness 
SoB
Headaches
DIziness 
Palpitations 
Worsening of other conditions
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9
Q

Generic signs of anaemia

A

Pale skin
Conjunctival pallor
Tachycardia
Raised RR

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10
Q

Specific signs/Sx of iron deficiency anaemia

A 
Pica - dietary craving for abnormal things 
Hair loss / brittle hair and nails 
Koilonychia (spoon shaped nails) 
Angular cheilitis 
Atrophic glossitis (smooth tongue)
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11
Q

Initial investigations for anaemia

A 
FBC (for Hb and MCV) 
Blood film 
Reticulocyte count 
Ferritin 
B12 and folate 
Bilirubin 
Direct Coombs test 
Haemoglobin electrophoresis
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12
Q

What does high reticulocytes suggest

A

Active production to replace rbc - suggests anaemia due to blood loss or haemolysis

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13
Q

Types of leukaemia that affect children

A

ALL - acute lymphoblastic leukaemia
AML - acute myeloid leukaemia
CML - chronic myeloid leukaemia

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14
Q

Conditions that have a higher risk of leukaemia

A

Down’s syndrome
Kleinfelter syndrome
Noonan syndrome
Fanconi’s anaemia

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15
Q

Risk factor for leukaemia

A

Radiation during pregnancy

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16
Q

Leukaemia presentation

A 
fatigue, pallor
Persistent fever
Failure to thrive 
WEight loss 
Night sweats 
Petechiae and abnormal bruising 
Unexplained bleeding 
Abdo pain 
Generalised lymphadenopathy 
Unexplained or persistent bone/joint pain 
Hepatosplenomegaly
17
Q

What is Idiopathic thrombocytopenic purpura

A

Type II hypersensitivity reaction
Antibodies produced that target platelets
Spontaneously or triggered by infection

18
Q

Idiopathic thrombocytopenic purpura PRESENTATION

A

Bleeding
Bruising
Petechiae / purpuric rash

Usually presents in children under 10 years. Often Hx of recent viral illness.

19
Q

Types of non blanching lesions

A

Petechiae - pin prick spots (<1mm)
Purpura - larger (3-10mm)
Ecchymoses - large area of blood collection under the skin (>10mm)

20
Q

Idiopathic thrombocytopenic purpura - MANAGEMENT

A

Urgent full blood count (platelets should be the only abnormality)
Other causes for low platelet excluded - e.g. heparin induced thrombocytopenia, leukaemia
Severity/management depends on how low platelet count is
Usually no treatment is required and patients monitored until platelets return to normal
70% of patients will remit spontaneously

Treatment (if pt actively bleeding or there is severe thrombocytopenia platelets <10)

  • prednisolone
  • IV immunoglobulins
  • blood transfusion
  • platelet transfusion (only works temporarily)
21
Q

Idiopathic thrombocytopenic purpura - complications

A

Chronic ITP
Anaemia
Intracranial and subarachnoid haemorrhage
GI bleeding

22
Q

Idiopathic thrombocytopenic purpura - ADVICE

A

Avoid contact sports
Avoid IM injections and procedures such as LP
Avoid NSAIDs, aspirin, blood thinning meds
Advice on managing nose bleeds
Seek help after any injury that may have caused internal bleeding

23
Q

Sickle cell genetics

A

Autosomal recessive
Abnormal gene for beta-globin on chromosome 11
One copy —> sickle cell trait (usually asymptomatic)
2 copies —> sickle cell disease

24
Q

Sickle cell relation to malaria

A

Having sickle cell trait (1 copy of faulty gene) reduces severity of malaria infection
As a result, areas with malaria - 1 faulty gene is a selective advantage
So there is higher prevalence in these areas (Africa, India, Middle East, Caribbean)

25
Q

Sickle cell COMPLICATIONS

A 
Anaemia 
Increased risk of infection 
Stroke 
Avascular necrosis in large joints such as the hip 
Pulmonary hypertension 
Painful and persistent penile erections (priapism) 
CKD
Sickle cell crises 
Acute chest syndrome
26
Q

Sickle cell GENERAL MANAGEMENT

A

Avoid dehydration/other triggers of crises
Ensure vaccines up to date
Antibiotic prophylaxis (usually penicillin V)
Hydroxycarbamide - used to stimulate production of HbF
Blood transfusion for severe anaemia
Bone marrow transplant

27
Q

Management for sickle cell crises

A

No specific treatment - manage patients supportively
Low threshold for admission to hospital
Treat any infection
Keep warm
Keep well hydrated (IV fluids if needed)
Simple analgesia (NSAIDs avoided if renal impairment)
Penile aspiration for priapism

28
Q

Types of sickle cell crises

A

VASO-OCCLUSIVE - aka “painful crisis” caused by sickle cells blocking capillaries causing distal ischaemia. Associated with dehydration and raised haematocrit. Symptoms: fever and symptoms triggering infection. Can cause priapism = urological emergency

SPLENIC SEQUESTRATION - blocking of blood flow within spleen —> enlarged painful spleen. Pooling of blood in the spleen can lead to severe anaemia and circulatory collapse (hypovolaemic shock). EMERGENCY. Management = supportive. Splenectomy prevents these crises and used in the case of recurrent crises. Recurrent crises can lead to splenic infarcts, resulting in susceptibility to infections.

APLASTIC CRISIS - temporary loss of creation of new blood cells. Most commonly triggered by infection by parovirus B19. Leads to significant anaemia. Usually resolves spontaneously - blood transfusion may be required.

ACUTE CHEST SYNDROME - diagnosis requires 1) fever or respiratory symptoms, with 2) new infiltrates seen on chest X-ray. Can be due to infection (e.g. pneumonia) or non-infective (e.g. pulmonary vaso-occlusion)

29
Q

Management of ACUTE CHEST SYNDROME

A

Medical emergency !! High mortality
Prompt supportive management and treat underlying cause
Antibiotics or antivirals for infections
Blood transfusions for anaemia
Incentive spirometry
Artificial ventilation with NIV or intubation

30
Q

Thalassaemia signs and symptoms

A 
Microcytic anaemia 
Fatigue 
Pallor 
Jaundice 
Gallstones
Splenomegaly 
Poor growth and development 
Pronounced forehead and malar eminences
31
Q

Complications of iron overload in Thalassaemia

A 
Fatigue
Liver cirrhosis 
Infertility 
Impotence 
Heart failure 
Arthritis 
Diabetes 
Osteoporosis and joint pain
32
Q

Management options for Thalassaemia

A

Blood transfusions
Iron chelation
Splenectomy
Bone marrow transplant

33
Q

Types of Thalassaemia

A

Alpha Thalassaemia

Beta Thalassaemia

  • minor
  • intermedia
  • major
34
Q

Hereditary spherocytosis

A

RBC sphere shaped
Most common inherited haemolytic anaemia in Northern Europeans
Autosomal dominant

35
Q

Hereditary spherocytosis presentation

A 
Jaundice 
Anaemia 
Gallstones 
Splenomegaly 
Episodes of haemolytic crises
36
Q

Hereditary spherocytosis: common cause of APLASTIC CRISIS

A

Parvovirus

PAEDIATRICS (18 decks)

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