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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

Haemoglobin types by age

A

Newborn: HbF predominant, with HbA and HbA2
Children >1y: HbA prominent with HbA2 (2%)
High Hbf within first year means some children are asymptomatic of disease .e.g. sickle cells

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2
Q

Haematological values at birth and infancy

A 
Hb at birth 140-215g/L
Hb 2m: 100g/L
Preterm 4-8w: 65-90g/L
Blood volume term infant 80ml/Kg
Preterm blood volume 100ml/Kg
Folic acid, iron and vit B12 stores depleted by 2-4m in preterm infants 
WCC neonates: 10-25 x 10^9/L
Platelet count at birth 150-400 x 10^9/L
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3
Q

Definition of anaemia

A

Neonate: Hb<140g/L
1m-12m: Hb<100g/L
1y-12y: Hb <110g/L

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4
Q

Mechanisms of anaemia

A 
Reduced red cell production
-Red Cell aplasia
-Ineffective erythropoiesis
Increased red cell destruction
-Red cell membrane disorder
-Red cell enzyme disorder
-Haemaglobinopathies
-Immune
Blood loss (uncommon in children)
-Feto-maternal bleeding
-Chronic GI blood loss
-Inherited bleeding disorder
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5
Q

Causes Red cell Aplasia

A 
Parvovirus B19 infection (only in children with Dx haemolytic anaemia)
Diamond Backfan Anaemia
Transient eryhtoblastopenia of childhood
Fanconi anaemia
Aplastic anaemia
Leukaemia
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6
Q

Causes Ineffective erythropoiesis

A 
Iron deficiency
Folic acid deficiency
Chronic renal failure
Chronic inflammation
Myelodysplasia
Lead poisoning
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7
Q

Causes increase haemolysis

A

RBC membrane disorder: Hereditory spherocytosis
RBC enzyme deficiency: G6PD deficiency
Haemoglobinopathy: thalassaemia, sickle cell
Immune: haemolytic disease of the newborn, autoimmune haemolytic anaemia

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8
Q

Diagnosis Ineffective erythropoeisis

A

Normal reticulocyte count
Abnormal MCV
-Low in iron deficiency
-Raised in folic acid deficiency

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9
Q

Causes iron deficiency

A

Inadequate iron intake (common in infants)
Malabsoprtion
Blood loss

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10
Q

Iron intake requirements

A

1y: 8mg/day
Adult female: 15mg/day
Adult male: 9mg/day

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11
Q

Iron sources in childhood

A 
Breast milk (low content highly available)
Infant formula (supplemented iron)
Cows milk (high content poor absorption)
Dietary: solids introduced at weaning
-cereals supplemented (poor absorption)
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12
Q

Diagnostic features on blood film

A

Spherocytes: hereditory spherocytosis
Sickle cells/target cells: sickle cell disease
Hypochromic/microcytic red cells: thalassaemia, iron deficiency, anaemia of chronic disease

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13
Q

Hb HPLC diagnostic features

A

Sickle cell: HbS and no HbA
Beta thalassaemia major: only HbF present
Beta thalassaemia trait: Increase HbA2
Alpha thalassaemia trait: HPLC normal

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14
Q

Inhibition of iron absorption

A

Tanin in tea

Phytates in high fibre food

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15
Q

Clinical features iron deficiency anaemia

A

Typically asymptomatic until 60g/L
Tire easily
Feed slowly
Pale- conjunctivae, tongue, palmar creases
Pica: inappropriate eating non-food materials
Behaviour and intellectual decline

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16
Q

Diagnosis iron deficiency

A

Microcytic hypochromic anaemia
-low MCV, low MCH
Low serum ferritin

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17
Q

Mx Iron deficiency

A 
Dietary advice
Supplementation with oral iron
-Syntron (sodium iron edetate)
-Nifarex (polysccharide iron complex)
-Continues until Hb normal + 3m
Normal Hb rise with supplements ~ 10g/L/week
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18
Q

Diagnosis of Red cell aplasia

A 
Low reticulocyte count
Low Hb
Normal bilirubin
Negative Coombs test
Absent red cell precursors on bone marrow
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19
Q

Features Diamond-Backfan anaemia

A 
Rare
Family history (20%)
Present at 2-3m or birth
Symptoms of anaemia
Associated with congenital abnormality .e.g. short stature, abnormal thumbs
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20
Q

Mx Diamond Backfan anaemia

A

Oral steroids
Monthly rbc transfusions (steroid unresponsive)
Stem cell transplantation

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21
Q

Features transient erythroblastopenia of childhood

A

Similar features to Diamond Backfan anaemia
Always recovers
Usually within several weeks

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22
Q

Features of increased haemolysis

A 
Anaemia
Hepatomegaly
Splenomegaly
Increased unconjugate bilirubin
Excess urinary urobilinogen
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23
Q

Diagnosis haemolysis

A 
Raised reticulocyte count
Polychromasia on blood film (lilac colour on stained film)
unconjugated bilirubinaemia
Increased urinary urobilinogen
Abnormal rbc appearance on film
Positive direct antiglobulin test
increased rbc precursors in bone marrow
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24
Q

Features hereditory spherocytosis

A

Jaundice
Anaemia
Mild-moderate splenomegaly
Aplstic crisis: transient 2-4w with parvovirus B19
Gallstones: associated with increased bilirubin

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25
Q

Diagnosis hereditory spherocytosis

A

Blood film usually diagnostic
Specific tests: dye binding assay, osmotic fragility
dDx spherocytes on film: autoimmune haemolytic anaemia (excluded by antibody test)

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26
Q

Genetics hereditory spherocytosis

A

Mutation in rbc membrane proteins
-spectrin, ankyrin, band 3
Loss of membrane on passage through spleen
Reduction in SA:V
Spheroidal cells destroyed in microvasculature of spleen

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27
Q

Mx hereditory spherocytosis

A

Oral folic acid (raised requirement)
Splenectomy (poor growth or symptomatic anaemia)
-HiB, Men C and Strep immunisations
Lifelong oral daily penicillin prophylaxis
Cholecystectomy if symptomatic gallstones

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28
Q

Mx Aplastic crisis

A

1-2 blood transfusions over 3-4w period

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29
Q

Features G6PD

A 
Neonatal jaundice (within 3d)
Acute haemolysis
-infection
-medications
-fava/braod beans
-naphthalene in mothballs
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30
Q

Drugs to avoid in G6PD

A

Antimalarias .e.g. primaquine, quinine, chloroquine
Anitbiotics .e.g. sulphonamides, quinolones, nitrofurantoin
Analgaesics .e.g. aspirin

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31
Q

Pathology G6PD

A

Rate limiting enzyme in pentose phophate pathway
Essential for preventing oxidative damage
RBC susceptible to oxidant-induced hymolysis
X linked

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32
Q

Presentation of haemolysis in G6PD

A 
Fever
Malaise
Abdo pain
Passage of dark urine (Hb and urobilinogen)
Rapid Hb drop (<50g/L within 24-48h)
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33
Q

Diagnosis of G6PD

A

G6PD activity in RBS
May be misleadingly elevated during haemolytic crisis due to reticulocyte conc.
Repeat assay required after haemolytic episode

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34
Q

Mx G6PD

A

Safety net parents re recognition of haemolysis
Avoidance of precipitants
Transfusion rarely required

35
Q

Features of sickle cell

A

Anaemia
Infection (associated with hyposplenism): Hib, penomoccoci, salmonella osteomyelitis
Painful crisis: vaso-occlusive, typically limbs and spine
-precipitated by excercise, stress, infection, cold, dehydration, hypoxia
Acute anaemia
Priapism
Splenomegaly

36
Q

Sickle cell painful crisis types

A

Hand-foot syndrome: dactylitis
Acute chest syndrome
Avascular necrosis of femoral head

37
Q

Long term Cx sickle cell

A 
Short stature
Delayed puberty
Stroke and cognitive problems
Adenotonsillar hypertrophy (sleep apnoea)
Cardiac enlargement
Heart failure
Renal dysfunction
Pigment gallstones (increased bile production)
Leg ulcers
Psychosocial problems
38
Q

Features sequestration crises

A

Sudden splenic or hepatic enlargement
Abdominal pain
Circulatory collapse
Accumulated sickle cells in spleen

39
Q

Types of sickle cell disease

A

Sickle cell anaemia: homozygous HbS
HbSc disease: no HbA and no normal beta globulin
Sickle beta thalassaemia: no HbA or beta globulin
Sickle cell carriers: 40% HbS haemoglobin

40
Q

Pathogenesis sickle cell

A

HbS polymerises with rbc forming rigid tubularspiral bodies
Deformation of rbc into sickle shape
Reduced cell lifespan
trapped in microvasculature causing occusion and ischaemia

41
Q

Mx Sickle cell

A 
Infection prophylaxis
-immunisation
-daily oral penicillin
Daily folic acid
Reduce precipitates of crisis
Hdroxycaramide (increased HbF production)
Bone marrow transplant
42
Q

Mx acute sickle cell crisis

A 
Oral or IV analgaesia
Good hydration
Abx if infection
Oxygen if reduced sats
Exchange transfusion for acute chest syndrome, stroke, and priapism
43
Q

Prognosis sickle cell

A

Premature death ~ 40y

Mortality rate in children 3% due to infection

44
Q

Features Haemoglobin SC disease

A

Nearly normal Hb
Fewer painful crises
Proliferative Retinopathy in adolescence (periodically checked)
Osteonecrosis of hips and shoulders

45
Q

Sickle cell trait

A

Assyptomatic

Risk with general anaesthetic (sickling possible in low oxygen tension)

46
Q

Types beta thalassaemia

A

Major: HbA cannot be produced
Intermedia: small amount of HbA and large amount of HbF
Trait: assymptomatic

47
Q

Features beta thalassaemia

A 
Severe anaemia
Jaundice
Faltering growth/growth failure
Extramedullary haemoppoiesis (without transfusion)
-hepatosplenomegaly
-bone marrow expansion
-maxiallry overgrowth and skull bossing
48
Q

Mx Beta thalassaemia

A

Transfusion dependant by 3-6m
-lifelong monthly transfusion
Iron cehlation .e.g. subcut desferrioxamine, oral deferasirox from 2-3y
Bone marrow transplantation

49
Q

Complications regular transfusions

A 
Iron deposition
-cardiomyopathy
-liver cirrosis
-diabetes
-impaired growth and sexual maturation
-hyperpigmentation of skin
Antibody formation
Infection
Venous access difficulty requiring portacath
50
Q

Alpha thalassaemia types

A

Major (Hb barts hydrops fetalis): midtrimester fetal hydrops fatal in utero/hours postnatally
-possibility of monthly intrauterine tranfusion
HbH disease: 3 genes effected, mild/moderate anaemia
Trait: assymptomatic

51
Q

Causes of anaemia in the newborn

A

Congenital B19 parovirus infection
Congenital red cell aplasia
Haemolytic anaemias
Blood loss

52
Q

Causes of anaemia of prematuriry

A

Inadequate erythropoeitin production
Reduced rbc lifespan
Frequent blood sampling
Iron/folic acid deficiency (2-3m)

53
Q

Causes blood loss in newborn

A

Feto-maternal haemorrhage
Twin-twin transfusion
Blood loss during delivery .e.g. abruption

54
Q

Features Fanconi anaemia

A

Aplastic anaemia
AR mutation in FANC gene
Associated congenital abnormality .e.g. short stature, abnormal radii and thumbs, renal malformation, micropthalmia, pigmented skin lesions
Bone marrow failure apparent 5-6y

55
Q

Ix Fanconi Anaemia

A

Normal blood count

Chromosomal breakage of peripheral blood lymphocytes

56
Q

Cx Fanconi anaemia

A

Bone marrow failure

Transformation to acute leukaemia

57
Q

Features Schwachman Diamond Syndrome

A 
AR SBDS gene mutation
Bone marrow failure
Pancreatic exocrine failure
Skeletal abnormality
Isolate neutropenia
Mild pancytopenia
Risk of transformation to acute leukaemia
58
Q

Screening tests in coagulopathy

A

FBC and blood film
Prothrombin time (Factors II, V, VII, X)
APTT (Factors II, V, VIII, IX, X, XI, XII)
50:50 mix with plasma in prolonged APTT and PT distinguishes factor deficiency or inhibitor prescence
Thrombin time (fibrinogen)
Quantative fibrinogen assay
D-dimers (fibrinogen degradation products)
Biochemical screen, renal and liver function tests

59
Q

Features haemophilia

A

Recurrent spontaneous bleeding into joints and muscles
Present when starting to walk or crawl
Neonatal intracranial haemorrhage
Bleeding postcircumscision
Prolonged oozing from heel stick and venepuncture sites

60
Q

Severity and factor VIII

A

<1% - spontaneous
1-5%- bleeds on minor trauma
>5-40% - bleeds after surgery

61
Q

Mx Haemophilia

A 
Factor VIII haemophilia A 
-Desmopressin A in mild haemophilia A
Factor IX haemophilia B
Circulating level to 30%
Major surgery/big bleeds: 
-100% circulating level 
-maintenance 30-50% for 2w 
Avoid: IM injections, aspirin and NSAIDs
62
Q

Cx treatments for haemophilia

A

Inhibitors: reduce or inhibit effect of treatment, may be amenable to immune tolerance induction
Transfusion transmitted infections
Vascular access difficulty

63
Q

Features von Willebrand disease

A

Bruising
Excessive prolonged bleeding following surgery
Mucosal bleeding .e.g. epistaxis, menorrhagia

64
Q

Mx von Willbrand disease

A

Desmopressin in type 1
-caution in <1y associated hyponatraemia and seizures
Plasma derived FVIII

65
Q

Acquired disorders of coagulation

A

Haemorrhagic disease of the newborn (Vit K deficiency)
Liver disease
ITP
DIC

66
Q

Causes of vitamin K deficiency

A

Inadequate intake
Malabsorption .e.g. coeliac, cystic fibrosis, obstructive jaundic
Antagonists .e.g. warfarin

67
Q

Platelet counts and bleeding

A

Platelet count <150 x 10^9/L
Severe (spontaneous bleeding) <20
Moderate (bleeding in trauma or surgery) 20-50
Mild (major op or severe trauma ) 50-150

68
Q

Presentation thrombocytopenia

A 
Mild:
-Bruising
-Petechiae
-Purpura
-Mucosal bleeding
Severe:
-GI haemorrgae
-Haematuria
-Intracranial bleeding
69
Q

Causes of purpura

A

Increased platelet destruction .e.g. SLE, ITP, HUS, DIC
Impaired platelet production .e.g. Fanconi anaemia, Wiskott-Aldrich, aplastic anaemia, marrow infiltration
Platelet dysfunction .e.g. uraemia
Vascular disoders .e.g. scurvy, meningitis, vasculitis

70
Q

Features ITP

A 
Antiplatlet IgG autoantibodies
Megakaryocyte increase in bone marrow
2-10y
1-2w post viral infection
Petechiae
Purpura
Superficial brusing
epistaxis
71
Q

Mx ITP

A 
Self limiting within 6-8w
Often no therapy required
Treat if major bleeding
-oral prednisolone
-IV anti-D
-IV immunoglobulin
Transfusion in life threatening haemorrhage
Avoid trauma and contact sports
72
Q

Features chronic ITP

A

Platelet count low 6m after diagnosis (20%)

73
Q

Mx Chronic ITP

A 
Mainly supportive
Rituximab: B lymphocyte monoclonal antibody
Thrombopoietic growth factors
Splenectomy (if drug failure)
SLE screening
74
Q

Features DIC

A

Due to severe sepsis or shock and circulatory collapse
Bruising
Purpura
Haemorrhage

75
Q

Ix DIC

A 
Thrombocytopneia
Low fibrinogen
Prolonged PT and APTT
Raise fibrinogen degradation productions and D-dimers
Microangiopathic haemolytic anaemia
Reduction in protein C,S and antithombin
76
Q

Mx DIC

A

Underlying cause
FFP, cryoprecipitate and platelets
Antithrombin and protein C concentrates

77
Q

Pathology DIC

A

Coagulation pathway activation leading to diffuse fibrin deposition
Consumption of coagulation factors and platelets
Initiated through tissue factor pathways

78
Q

Acquired disorders thrombosis

A 
Catheter related thrombosis
DIC
Hypernatraemia
Polycythaemia
Malignancy
SLE
Persistent antiphospholipid antibody syndrome
79
Q

Congenital prothrombotic disorders

A 
Protein C deficiency
Protein S deficiency
Antithrombin deficiency
Factor V leiden (resistant to protein C degradation)
Prothrombin gene G20210A mutation
80
Q

Indication for thrombosis screening

A

Unanticipated or extensive venous thrombosis
Ischaemic skin lesions
Neonatal purpura fulminans (or +ve fHx of)

81
Q

Screening thrombophilia

A 
Protein S Assay
Protein C Assay
Antithrombin Assay
PCR factor V leiden
Prothrombin gene mutation PCR
82
Q

Features HSP purpura

A

lesiosn confined to buttocks, legs, arms
Swollen painful knees and ankles
Abdominal paid
Haematuria

83
Q

Features septic purpura

A

Meningococcal or viral
Fever
Speticaemia
+ve glass test

84
Q

Features leukaemia thrombocytopenia

A 
Malaise
infection
pallor
hepatosplenomegaly
lymphadenopathy
low Hb blasts on film

Paediatrics (22 decks)

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