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3A > Paeds - Haematology > Flashcards

Paeds - Haematology Flashcards

1
Q

Sickle cell anaemia aetiology

A

Most common in blacks

Autosomal recessive - Chromosome 11

GLUT - VAL change in B-chain of Hb
Abnormal Hb chain - HbS
Hypoxic conditions - HbS polymerises
Becomes sickle shaped - Occludes vessels

Sickled cell life span = 30 days

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2
Q

Sickle cell triggers

A 
Deoxygenation after exercise or exertion
Dehydration 
Infection 
Cold weather 
Stress
Alcohol
Smoking
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3
Q

Sickle cell presentations

A

6-12 months

Painful/thrombotic crisis 
Acute chest syndrome 
Aplastic crisis - Parvo B19 
Sequestration crisis 
Haemolytic crisis
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4
Q

Painful/thrombotic crisis

A

Lots of Hb deoxygenated at once
- Cold, dehydration, hypoxia

Lots of sickling - Occlusion of small vessels

Severe pain 
AVN - Bone marrow 
Swollen joints 
Dactylitis 
Acute abdomen - Mesenteric ischaemia
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5
Q

Acute chest syndrome

A

Vaso-occlusive crisis in the lungs

Pain
Dyspnoea
Cough
Fever

XR - New pulmonary infiltrate

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6
Q

Aplastic crisis

A

Parvo B19 infection

Anaemia

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7
Q

Sequestration crisis

A

Splenomegaly

Hb - LOW
Reticulocytes ^

Circulatory collapse - Shock

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8
Q

Haemolytic crisis

A

Anaemia

Jaundice

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9
Q

Sickle cell - Additional S/S

A

Anaemia
Jaundice
Splenomegaly

Priapism

Infection

  • Strep pneumonia
  • HiB
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10
Q

Sickle cell investigations

A

Prenatal diagnosis - Chorionic villous sampling

Screening - Guthrie heel prick test

Hb electrophoresis

  • HbSS
  • HbF ^

FBC - Anaemia
Increased production in BM - Reticulocytes ^

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11
Q

Haemolytic anaemia diagnosis

A

RBC - LOW

Reticulocytes ^

Unconjugated bilirubinaemia
Urinary urobilinogen

Blood film - Sickled or spherocytes

Positive Coombs test!

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12
Q

Sickle cell long-term management

A

Vaccinations - Flu + Pneumococcal + Hep B

Prophylaxis - Oral penicillin

Oral folic acid

Desferroxamine - Iron chelation if repeated transfusion

Hydroxycarbamide - Increases HbF

Annual transcranial USS

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13
Q

Sickle cell painful crisis management

A 
Analgesia 
Abx 
O2 
Fluids 
Blood transfusion
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14
Q

Acute chest syndrome management

A 
Oxygen 
Chest physio 
NIV or I+V 
Antibiotics 
Transfusion
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15
Q

Priapism management

A 
Fluids
Oxygen 
Analgesia 
Gentle exercise 
Bladder emptying 
Warm bath

Aspiration and irrigation of corpora cavernosa with adrenaline

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16
Q

Sickle cell complications

A 
Chronic pain 
Pulmonary HTN - Cor pulmonale 
Stroke 
Infection 
Gallstones 
Retinopathy 
AVN - Femoral head
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17
Q

B-Thalassaemia

A

Autosomal recessive

Hypochromic
Microcytic

Major - No HbA
Minor - Low HbA

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18
Q

B-Thalassaemia electrophoresis

A

HbF ^
HbA2 ^

Beta chains - ABSENT

Major - No HbA
Minor - Low HbA

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19
Q

B-Thalassaemia presentation

A

Jaundice
Anaemia - Transfusion dependent from 3-6 months
FTT

Hepatosplenomegaly

Delayed puberty

Facial

  • Maxillary overgrowth
  • Skull bossing
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20
Q

B-Thalassaemia management

A

Regular transfusions

IRON CHELATING AGENT - DESFERRIOXAMINE

BM transplant

Genetic counselling

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21
Q

A-Thalassaemia

A

1 or 2 alpha chains affected

  • Hypochromic microcytic
  • Normal Hb

3 alpha chains affected - HbH disease
- Hypochromic microcytic
- Anaemia
Splenomegaly

4 alpha chains affected - Major

  • Death in utero - Mid-trimester
  • Hydrops fetalis

Diagnosis - Hb electrophoresis
Management - Monthly intrauterine transfusions

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22
Q

Haemophilia

A

X-linked recessive

A - Factor 8 deficiency
B - Factor 9 deficiency

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23
Q

Haemophilia presentation

A

40% present in neonatal period

  • Intracranial haemorrhage
  • Bleeding after circumcision
  • Heel-prick oozing

Bleeding into joints - Haemoarthrosis
Bleeding after dental work
Bleeding after surgery
Haematomas

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24
Q

Haemophilia management

A

A - Factor 8
B - Factor 9

FFP

Avoid NSAIDS

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25
Q

Immune thrombocytopenic purpura

Aetiology and presentation

A

Post-strep infection
Immune destruction of platelets by IgG

2-10 years

1-2 weeks post-viral infection

  • Petechiae
  • Splenomegaly
  • Mucosal bleeds - Gums and epistaxis
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26
Q

ITP management

A

Self-limiting
1-2 weeks

Avoid contact sports
Avoid NSAIDS

Chronic - Rituximab
Splenectomy

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27
Q

Von-Willebrands inheritance

A

Autosomal dominant

Most common inherited bleeding disorder

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28
Q

Von-Willebrands factor

A

Large glycoprotein
Promotes platelet adhesion to damaged endothelium
Carrier molecule for factor 8

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29
Q

VWD presentation

A

Bruising
Bleeding
Epistaxis

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30
Q

VWD types

A
  1. Partial reduction - Most common
  2. Abnormal form
  3. Total lack - AR inheritance
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31
Q

VWD investigations

A

Bleeding time ^
APTT ^
Factor 8 - LOW

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32
Q

VWD management

A

Desmopressin

Tranexamic acid if bleeding
Factor 8

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33
Q

G6PD deficiency inheritance

A

X-linked recessive

Mediterranean
Africa
Middle-East

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34
Q

G6PD deficiency pathophysiology

A

G6PD is an enzyme of the pentose phosphate pathway

Pentose phosphate pathway maintains level of NADPH

NADPH maintains levels of glutathione

Glutathione protects red cells from oxidative stress

35
Q

G6PD triggers

A

Drugs

  • Anti-malarials
  • Ciprofloxacin
  • Nitrofurantoin
  • Sulphonamides

Infection

FAVA BEANS!

36
Q

G6PD presentation

A

Neonatal jaundice

Intravascular haemolysis

  • Dark urine
  • Fever
  • Back pain
  • Malaise

Gallstones
Splenomegaly

37
Q

G6PD investigations

A

Blood film

  • Bite and blister cells
  • Hemi ghosts
  • Heinz-bodies
38
Q

Hereditary spherocytosis

A

Autosomal dominant

Haemolytic anaemia

Northern european

Mutation in gene for RBC membrane
RBC loses part of membrane
Becomes spheroidal
Destroyed in spleen

39
Q

Hereditary spherocytosis presentation

A

Chronic

Anaemia 
Neonatal jaundice 
Gallstones 
Splenomegaly 
Aplastic crisis - Parvo B19
40
Q

Hereditary spherocytosis investigations

A

Blood film - Spherocytes

Osmofragility test

41
Q

Hereditary spherocytosis management

A

Oral folic acid
Splenectomy

Transfusion

Desferrioxamine

42
Q

Petechiae DDx

A 
ALL 
HSP 
ITP 
Meningococcal septicaemia 
DIC 
SLE
43
Q

Long-term transfusion problems

A

Iron overload - GIVE DESFERRIOXAMINE

  • Cardiomegaly
  • Liver cirrhosis
  • Diabetes

Infection - Less common now due to screening
Antibody formation

44
Q

Where does cell production take place

A

Fetal - liver

Post-natal - BM

45
Q

Hb concentration

A

Highest at birth
Lowest at 2 months
HbF gradually replaced during infancy

46
Q

Anaemia in neonates

A

< 140

47
Q

Anaemia in 1-12 months

A

< 100

48
Q

Anaemia in 1-12 years

A

< 110

49
Q

Causes of neonatal anaemia

A

Decreased production
Decreased lifespan - Haemolysis
Changover to HbA

^ Plasma = Dilution

50
Q

Anaemia in newborns

A

Erythroblastosis fetalis

51
Q

Inherited aplastic anaemias

A

Fanconi anaemia

Shwachman-Diamond syndrome

52
Q

Fanconi anaemia

A

Autosomal recessive
Bone marrow failure

Congenital abnormalities

  • Short stature
  • Abnormal radii and thumbs
  • Renal malformations

Diagnosis - Increased chromosomal breakage of peripheral blood lymphocytes

Management - BM transplant

53
Q

Shwachman Diamond syndrome

A

Autosomal recessive
Bone marrow failure

Pancreatic exocrine failure
Skeletal abnormalities

Isolated neutropenia
Pancytopenia

54
Q

Iron deficiency pathophysiology

A

Ineffective erythropoiesis

  • Microcytic
  • Hypochromic
55
Q

Iron deficiency causes

A

Inadequate intake
Malabsorption
Blood loss

56
Q

Iron deficiency features

A 
Fatigue 
Slow feeding 
Signs of malabsorption 
Blood loss 
Conjunctival pallor 
Behavioural and intellectual difficulties
57
Q

Iron deficiency diagnosis

A

Blood film

  • Microcytic
  • Hypochromic

Ferritin - LOW

58
Q

Iron deficiency management

A

Dietary advice

Ferrous sulphate

59
Q

Red cell aplasia

A

Congenital red cell aplasia - Diamond Blackfan
Transient erythroblastopenia
Parvovirus B19

60
Q

Congenital red cell aplasia

A

Diamond blackfan
2-3 months anaemia

Short stature
Abnormal thumbs

Management

  • Steroids
  • Transfusion
61
Q

Transient erythroblastopenia

A

Triggered by viral infections

Recovery within several weeks

62
Q

Parvovirus B19

A

Causes red cell aplasia in children with inherited haemolytic anaemia

63
Q

Red cell aplasia diagnosis

A

Reticulocytes - LOW
Bilirubin - NORMAL

Negative coombs test

Absent red cell precursors in bone marrow

64
Q

Coombs test

A

Tests for autoimmune haemolytic anaemia

65
Q

Sickel cell long-term problems

A 
Short stature 
Delayed puberty 
Stroke 
Cognitive problems 
Adenotonsillar hypertrophy
Cardiac enlargement - HF 
Renal dysfunction
Pigmented gallstones 
Psychosocial problems
66
Q

Desmopressin

A

Stimulates the release of factor 8 and VWF

Water retention - Risk of hyponatraemia

67
Q

Vitamin K deficiency

A

Factors 2,7,9,10

Inadequate intake
Malabsorption

Antagonists - Warfarin

68
Q

Disseminated intravascular coagulation

A

Coagulation pathway activation
Fibrin deposition in microvasculature
Usage of coag factors and platelets

Usually due to severe sepsis or shock

69
Q

DIC features

A

Bruising
Purpura
Haemorrhage

70
Q

DIC diagnosis

A 
Thrombocytopenia
PT ^ 
APTT ^ 
Fibrinogen - LOW 
D-DIMER ^ 
Reduction in naturally occurring anticoagulants
71
Q

DIC management

A

Cryoprecipitate
Platelets

ABC
IV fluids

72
Q

Thrombosis in children

A

Congenital - Thrombophilia

  • Protein C deficiency
  • Protein S deficiency
  • Antithrombin deficiency
  • Factor V leiden - Resistant to degradation by protein C

Acquired

  • DIC
  • Hypernatraemia
  • Polycythaemias
  • SLE
  • Persistent antiphospholipid antibody syndrome
73
Q

ALL types

A

Common - 75%
T-cell only - 20%
B-cell only - 5%

74
Q

ALL epidemiology

A

Males

2-5 years

75
Q

ALL poor prognostic factors

A 
Male 
Philadelphia chromosome
< 2 
> 10 
WCC > 20
76
Q

ALL aetiology

A

Down syndrome

Philadelphia chromosome - 22

Ionising radiation

77
Q

ALL presentation

A
  1. Bone marrow infiltration
    - Bone pain
    - RBCs - SOB, lethargy, pallor
    - Platelets - Bruising, petechiae
  2. Hepatosplenomegaly
  3. Organ infiltration
    - Painless testicular enlargement
    - Neuro - CNS palsies and papilloedema
78
Q

ALL investigations

A

Bloods

  • RBC - LOW
  • WCC ^
  • Neutrophils - LOW

U/E

  • Urea ^
  • Creatinine ^

CXR/MRI - Mets

Bone marrow biopsy

LP - Pleocytosis

79
Q

ALL management

A

Induction

  • Vincritine
  • Dexamethasone

Consolidation
- Doxorubicin

Maintenance
- Methotrexate

80
Q

ALL complications

A

Neutropenic sepsis - Tazocin

Hyperuricaemia - Allopurinol

Secondary cancers
Stunted growth

81
Q

Hodgkin’s lymphoma

A

Reed Sternberg cells

82
Q

Hodgkin’s lymphoma presentation

A

Painless lymphadenopathy
B symptoms
Abdo mass

83
Q

Hodgkin’s lymphoma investigations

A

CT
PET
Lymph node biopsy

84
Q

Hodgkin’s lymphoma management

A

Chemotherapy

Radiotherapy

3A (31 decks)

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