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1 - Paeds AE > Haematological disorders > Flashcards

Haematological disorders Flashcards

1
Q

What is haemopoiesis?

A

The process which maintains lifelong production of haemopoietic (blood) cells

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2
Q

Where is the main site of haemopoiesis in the foetus and in postnatal life?

A

Fetal - fetal liver

Postnatal - bone marrow

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3
Q

How does HbF (fetal haemoglobin) differ from adult Hb?

A

HbF has a higher affinity for oxygen than adult Hb

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4
Q

When does HbF stop being present in the blood stream?

A

After about 1 year HbF is very low in healthy children

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5
Q

How does Hb alter throughout birth and the first stages of life?

A

Hb concentration is high at birth but falls to its lowest level at 2 months of age

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6
Q

What are the mechanisms of anaemia?

A 
Reduced red cell production (iron deficiency)
Increased red cell destruction (haemolysis)
Blood loss (relatively uncommon in children)
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7
Q

What are the main causes of reduced red cell production?

A 
Ineffective erythropoiesis (iron deficiency, folic acid deficiency, chronic inflammation (JIA), chronic renal failure)
Red cell aplasia (complete absence of red cell production)
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8
Q

What are the main causes of increased red cell destruction?

A

Red cell membrane disorders
Red cell enzyme disorders
Haemoglobinopathies
Immune

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9
Q

What are the main causes of iron deficiency in children?

A

Inadequate intake
Malabsorption
Blood loss

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10
Q

How would you diagnose iron deficiency in children?

A

Microcytic, hypochromic anaemia

Low serum ferritin

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11
Q

If the bilirubin is raised in anaemia what does that tell you about the cause?

A

It is haemolysis as bilirubin is created from the break down of red blood cells

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12
Q

What are the main diseases causing haemolytic anaemia?

A

Sickle cell
Beta thalassaemia
G6PD deficiency

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13
Q

How do you manage iron deficiency?

A

Dietary advice

Supplementary oral iron for several months, a minimum 3 months after Hb has returned to normal

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14
Q

What are the disease consequences of haemolysis?

A

Anaemia
Hepatomegaly and splenomegaly
Increased blood levels of unconjugated bilirubin
Excess urinary urobilinogen

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15
Q

What are the diagnostic clues to haemolysis?

A

Raised reticulocyte count
Unconjugated bilirubinaemia and increased urinary urobilinogen
Abnormal appearance of the red cells on a blood film
Positive direct antiglobulin test
Increased red blood cell precursors in the bone marrow

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16
Q

What is G6PD deficiency?

A

G6PD is the rate-limiting enzyme in the pentose phosphate pathway and is essential for preventing oxidative damage to red cells. Those deficient are susceptible to oxidant-induced haemolysis

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17
Q

How is G6PD deficiency inherited?

A

It is X-linked so mainly affects males

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18
Q

How does G6PD deficiency present?

A

Neonatal jaundice within first 3 days
Acute haemolysis precipitated by infection, certain drugs, fava beans and naphthalene in mothballs.
Fever, malaise and dark urine

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19
Q

How do you diagnose G6PD deficiency?

A

Measuring G6PD activity in red blood cells

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20
Q

How do you manage G6PD deficiency?

A

The parents should be given advice about the signs of acute haemolysis and provided with a list of drugs, foods and chemicals to avoid

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21
Q

What are haemoglobinopathies?

A

RBC disorders which cause haemolytic anaemia because of reduced or absent production of HbA (thalassaemia) or because of the production of abnormal Hb (sickle cell)

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22
Q

Which races is sickle cell most common in?

A

Patients whose parents are black and originate from tropical Africa or the Caribbean but it is also found in the middle east

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23
Q

How does sickle cell disease affect the cell shape?

A

HbS polymerises within RBCs forming rigid tubular spiral bodies which deform the red cells into a sickle shape.

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24
Q

What does the abnormal sickle cell shape mean practically?

A

The cells have a reduced lifespan and may be trapped in the microcirculation, resulting in blood vessel occlusion and therefore ischaemia in an organ or bone.

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25
Q

What exacerbates vaso-occlusive crises in sickle cell?

A

Low oxygen tension, dehydration, undue stress, excessive exercise and cold

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26
Q

What are the clinical features of sickle cell disease?

A 
Anaemia
Infection
Painful crises
Acute anaemia
Priapism
Splenomegaly
Long-term problems
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27
Q

What does the increased risk of infection in sickle cell disease mean practically?

A

Full immunisation is required
Daily oral penicillin throughout childhood should be given
Daily folic acid

28
Q

What is the most common painful crisis in sickle cell disease?

A

Vaso-occlusive crises

29
Q

How do vaso-occlusive crises present ?

A

Pain may affect many organs of the body with varying frequency and severity. A common mode of presentation in late infancy is the hand-foot syndrome, dactylics with swelling and pain of the fingers and/or feet from vast-occlusion

30
Q

What is the most severe painful crisis in sickle cell disease and why?

A

Acute chest syndrome, it can lead to severe hypoxia and the need for mechanical ventilation and emergency transfusion

31
Q

What are the crises that can lead to acute anaemia in sickle cell disease?

A

Haemolytic crises
Aplastic crises
Sequestration crises

32
Q

What are the long term complications of sickle cell disease?

A 
Short stature and delayed puberty
Stroke and cognitive problems
Adenotonsillar hypertrophy
Cardiac enlargement
Heart failure
Renal dysfunction
Pigment gallstones
Leg ulcers
Psychosocial problems
33
Q

How do you treat painful crises in sickle cell disease?

A

Oral or IV analgesics

Good hydration

34
Q

How can you treat the chronic complications of sickle cell?

A

Hydroxyurea

35
Q

People from which areas are at more risk of beta thalassaemia?

A

Indian subcontinent, Mediterranean and Middle East

36
Q

What are the two main types of beta thalassaemia?

A

B-thalassaemia major

B-thalassaemia intermedia

37
Q

What are the clinical features of beta thalassaemia?

A 
Severe anaemia (transfusion dependent age 3-6 months)
Failure to thrive
Extramedullary haemopoiesis (prevented by regular transfusions)
38
Q

How do you treat beta thalassaemia?

A

Lifelong monthly RBC transfusions

39
Q

What are the complications of lifelong monthly RBC transfusions?

A 
Chronic iron overload, leading to:
cardiac failure
liver cirrhosis
diabetes
infertility 
growth failure
40
Q

How are the complications of RBC transfusions treated in B-thalassaemia?

A

Additional iron chelate with SC desferrioxamine OR

oral iron chelator drug such as deferasirox

41
Q

What is the only cure for B-thalassaemia?

A

Bone marrow transplant

42
Q

How can you distinguish between anaemia and B-thalassaemia trait?

A

Measuring serum ferritin, which is low in iron deficiency but not in B-thalasseamia trait

43
Q

What is a haemolytic disease of the newborn example?

A

Rhesus disease

44
Q

What are the main causes of blood loss in neonates?

A

Feto-maternal haemorrhage
Twin-to-twin transfusion syndrome
Blood loss around the time of delivery

45
Q

What are the main causes of anaemia of prematurity?

A

Inadequate erythropoietin production
Reduced red cell lifespan
Frequent blood sampling whilst in hospital
Iron and folic acid deficiency (age 2-3 months)

46
Q

What are the five main components of normal haemostasis?

A 
Coagulation factors
Coagulation inhibitors
Fibrinolysis
Platelets
Blood vessels
47
Q

What is the endpoint of the coagulation cascade?

A

The generation of thrombin

48
Q

What is haemophilia?

A

X-linked recessive disorder

Excessive bleeding in a variety of different severities, the severity usually stays constant within the family

49
Q

How would you treat haemophilia?

A

Recombinant Factor (whichever is causing type A or B) concentrate

50
Q

What should be avoided in all haemophilia patients?

A

Intramuscular infections, aspirin and NSAIDs

51
Q

What are the two major roles of von Willebrand factor?

A

Facilitates platelet adhesion to damaged endothelium

Acts as the carrier protein for VFIII:C; protecting it from inactivation and clearance

52
Q

How is von Willebrand disease inherited?

A

Usually autosomal dominant

53
Q

What are the clinical features of von Willebrand disease?

A

Bruising
Excessive, prolonged bleeding after surgery
Mucosal bleeding such as epistaxis and menorrhagia

54
Q

How does von Willebrand disease differ from Haemophilia?

A

In contrast to Haemophilia, spontaneous soft tissue bleeding such as large haematomas and haemarthroses are uncommon

55
Q

How can von Willebrand disease be treated?

A

DDVAP

56
Q

What are the main causes of acquired disorders of coagulation?

A

Haemorrhagic disease of the newborn due to vit K deficiency
Liver disease
Immune thrombocytopenia
DIC

57
Q

What are the clinical features of thrombocytopenia?

A

Bruising
Petechiae
Purpura
Mucosal Bleeding (epistaxis, gum bleeding)

58
Q

What causes immune thrombocytopenia ?

A

Destruction of circulating platelets by anti-platelet IgG autoantibodies

59
Q

What age and after what does ITP present?

A

Ages 2-10 years, 1-2 weeks after viral infection

60
Q

What is a serious but rare complication of ITP?

A

Intracranial bleeding

61
Q

How is ITP diagnosed?

A

It is a diagnosis of exclusion

62
Q

How do you treat ITP?

A

In most cases, the disease is acute, benign and self-limiting, usually remitting spontaneously within 6-8 weeks

63
Q

What is chronic ITP?

A

Low platelet count after 6 months, treatment is usually still supportive

64
Q

What is DIC?

A

Coagulation pathway activation leading to diffuse fibrin deposition in the microvasculature and consumption of coagulation factors

65
Q

What are the predominant clinical features of DIC?

A

Bruising, purpura and haemorrhage

66
Q

What are some investigation results that may suggest DIC?

A 
Thrombocytopaenia
Prolonged prothrombin time
Prolonged APTT
Low fibrinogen
Raised fibrinogen degradation products
D-dimers
Marked reduction in the naturally occurring anticoagulants - Proteins C and S and antithrombin
67
Q

How do you treat DIC?

A

TREAT CAUSE

Supportive: FFP, cryo and platelets

1 - Paeds AE (24 decks)

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