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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

What are the three sizes of RBC in anaemia

A

Microcytic
Normocytic
Macrocytic

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2
Q

What are the two colours of RBC

A

Hypochromic - pale

Normochromic

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3
Q

What are reticulocytes

A

Immature RBC

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4
Q

What does low reticulocytes mean

A

Low - lack of production

High - hemolysis/ blood loss

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5
Q

What are most Microchromic anaemias

A

Hypochromic

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6
Q

What are examples of Hypochromic Microcytic Anaemia

A
  • Iron Deficiency Anaemia
  • Thalassaemia
  • Chronic Inflammatory disease - usually normocytic but can be microcytic
  • Sideroblastic Anaemia
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7
Q

What are example of Normocytic Anaemia

A 
Chronic Inflammatory Disease - IBD, Infection 
Bone Marrow Failure/Malignancy
Prematurity   
Recent Blood Loss
Pregnancy 
Chronic Renal Disease 
HIV
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8
Q

What are examples of Macrocytic Anaemia

A 
Vitamin B12 deficiency 
Folate Deficiency 
Hypothyroidism 
Alcohol Access
Chronic Liver disease
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9
Q

What are the two types of Anaemia

A

Underproduction:

  • Microcytic
  • Normocytic
  • Macrocytic

Normal Production:
- Haemolysis

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10
Q

What are causes of severe anaemia at birth

A

Haemolytic disease of the newborn

Bleeding: umbilical cord, internal haemorrhage

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11
Q

What is severe anaemia in the newborn called due to rhesus haemolytic disease

A

Erythroblastosis Fetalis

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12
Q

How does erythroblastosis fetalis occur

A
  1. Rh negative mothers previously sensitised to Rh+ve blood from previous pregnancy
  2. Production of Rhesus Antibodies
  3. These Antibodies cross the placenta
  4. These cause haemolysis of fetal RBC
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13
Q

What are the signs and symptoms of erythroblastosis fetalis

A

Hepatospelomegaly
Severe Anaemia
Jaundice
Oedmatous Fetus

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14
Q

What is the Rx of erythroblastosis fetalis

A

Prevention of sensitisation with Rh immune globulin during and following pregnancy in Rh-ve mothers

Intra-uterine Blood Transfusion

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15
Q

What are causes of Physcological anaemia in the newborn

A

Fall in haemaglobin from birth to 2 months

  • Decreased RBC production
  • Plasma dilution from increasing blood volume
  • Shorter lifespans on neonatal RBC
  • More fragile RBC
  • Switch from HbF to HbA
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16
Q

Why does anaemia occur in prematurity

A

Low birth weight infants - poor erythropotein response (Low EPO) (promotes formation of RBC by bone marrow)

Breast milk erythopotein may not be sufficient for haematopoiesis

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17
Q

What is the role of breast milk for RBC

A

Erythopotein in Breast milk can help stimulate hematopoiesis

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18
Q

What are the signs and symptoms of anaemia and prematurity and how do you manage it

A

Poor weight gain
Pallor
Decreased activity
Tachycardia

Blood Transfusions

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19
Q

What are the main causes of iron deficiency anaemia in new borns

A 
Poor Intake 
Blood Loss e.g GI bleeding 
Malabsorption e.g Coeliac Disease
Trophics e.g Hookworm 
Increased Requirement e.g Infection, Breast feeding
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20
Q

How does iron deficiency anaemia present

A 
Pallor 
Irritability 
Anorexia 
Tachycardia 
Cardiac Dilation 
Murmur
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21
Q

What is the treatment for iron deficient anaemia

A 
Oral Iron (Ferritin) Therapy 
Common cause of failure non-compliance
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22
Q

What is Haemolysis

A

Increased RBC turnover/ destruction

shortened RBC lifespan e.g fragile abnormal ones

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23
Q

What are intra corpuscular causes of increased RBC destruction

A
  • Haemoglobin
  • Enzyme
  • Membrane
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24
Q

What are extra corpuscular causes of increased RBC destruction

A

Autoimmune
Spleen hypertrophy/ Spleenomegaly
Plasma Factors
Fragmentation

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25
Q

What is a complication of long term haemolysis and/or transfusions

A

Iron Overload - affects all organs e.g Liver faliure, Heart Failure

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26
Q

How do you manage iron overload

A

Ferritin monitoring + imaging

Iron Chelation

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27
Q

What are two examples of haemoglobinopathies

A

Sickle cell disease

Thalassaemia

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28
Q

How are haemoglobinopathies diagnosed

A
  • HPLC - High Performance Liquid Chromatography

- Hb Electrophoresis

29
Q

What is Sickle Cell Disease

A

AR disorder - causing production of abnormal beta globulin chains - HbS rather than HbA

Common in African Origin

30
Q

What is the Pathogenesis of Sickle Cell

A

HbS polymerises when deoxygenated causing RBCs to deform - producing sickle cells

Sickle Cells - Fragile and Haemolsye, they also occlude small vessels

31
Q

How do you screen for Sickle Cell in neonates

A

Hb Electrophoresis

32
Q

What is the presentation of Sickle Cell

A
  • Anaemia - Cardiomegaly, Low Pulse
  • Dactylitis - painful swelling of fingers and toes - often first symptom in babies
  • Infarction - low O2 RBC sickle causing pain crises and stroke
  • Infection - Asplenia from infarction/damage from abnormal RBC leads to increased risk of life threatening infections and sepsis - any fever is serious!!!!

Sickle Chest Syndrome - pain, fever, cough wheeze,
tachypnoea

  • Splenic Sequestration - RBC become blocked in spleen causing spleen to enlarge and become damaged

Chronic Complications: Renal impairment, Splenic Infarction, Retinal disease, Pulmonary HTN and joint damage

33
Q

What will be found on Ix of Sickle Cell

A
  • HPLC/Hb electrophoresis - HbS - no presence of HbA
  • Anaemia
  • Film - Sickle Cells
  • Raised Reticulocytes
  • Raised WCC
34
Q

How can Sickle Cell be cured

A

Stem Cell Transplant

35
Q

What should Sickle Cell patients with a fever do

A

Seek Help!!
Blood Cultures, CXR
IV fluids, Antipyretics
Hospitalise for any pneumonia

36
Q

How do you manage pain crisis in sickle cell

A

Triggered by cold, dehydration, infection

Treat mild with NSAIDs and Parecetamol

Severe: 
Fluids, 
Give O2 and Keep warm 
Blood Transfusion 
Exchange Transfusion 
give analgesics e.g. IV morphine
37
Q

What is Acute Chest Syndome SS commonly caused by how does it present and how is it managed

A

Infarction or Infection

  • Tachypnoea, Chest pain, Fever, Cough, Wheeze
  • Admit - IV fluids, blood transfusion, O2 and Abx
38
Q

What is an Aplastic Crisis caused by in SS

A

Paravirus B19

39
Q

How do you treat SS disease

A
  • Hydroxycarbamide
  • Transfusion – Prevent strokes for those at highest risk
  • Stem cell transplants – In severe cases e.g strokes, severe crisis
40
Q

What is Thalassaemia

A

Genetic Disorder resulting in faulty Hb chain synthesis

41
Q

What is Beta Thalassaemia

A

Autosomal Recessive - Beta Haemoglobin chains are coded for by two alleles

Thalassaemia Minor - one defective allele (carrier)

Thalassaemia Major - two defective alleles

42
Q

How does Beta Thalassaemia present

A

Minor: unremarkable symptoms, may be mildly anaemic

Major:

  • Severe haemolytic anaemia
  • Growth retardation
  • Hepatosplenomegaly
  • Skeletal Deformities e.g skull bossing
43
Q

How do you manage Beta Thalassaemia

A

Minor: no treatment, maybe iron or folate supplements

Major: 
• Genetic Counselling
• Regular blood transfusion
• Iron chelation for iron overload (Liver, Heart, Pancreas) 
• Bone Marrow Transplantation - cure
44
Q

How do Haemolytic Anaemias present

A
  • Hydrops fetalis
  • Neonatal hyperbilirubinaemia
  • Neonatal ascites
  • Anaemia/failure to thrive
  • Splenomegaly
  • Cholecystitis/gall stones
  • Hyperbilirubinaemia
  • Leg ulcers
  • Aplastic crisis
  • Thromboembolism
45
Q

What is Alpha Thalassaemia

A

Mainly caused by deletion of 4 genes

  • All 4 - death in utero
  • 3 - moderate anaemia and haemolysis
  • 2 - asymptomatic - carrier
  • 1 - normal
46
Q

What is Glucose 6 Phosphate Dehydrogenase Deficiency

A

X linked chief RBC enzyme defect

mainly effects males

47
Q

How does G6PD present

A

• Three main presentations
– Neonatal jaundice
– Chronic non-spherocytic haemolytic anaemia
– Intermittent episodes of intravascular haemolysis

• Sporadic haemolysis
– Typically induced by drugs, fava beans, fever, acidosis
– Intravascular haemolysis - haemoglobinuria, rigors, fever, backpain
– Treated by stopping precipitant, transfusion, renal support

48
Q

What will see on RBC film of G6PD sporadic haemolysis

A

Bite and Blister Cells

49
Q

What is the commonest type of hereditary haemolysis in europeans

A

Hereditary Spherocytosis - Sphere shaped RBC

50
Q

What is the commonest type of Immunological Thrombocytopenia

A

Idiopathic/Immune Thrombocytopenic Purpura - immune destruction of platelets

Can be acute or chronic - usually recovers over weeks or months

51
Q

When does acute ITP usually occur

A

In children following a viral infection

52
Q

How does ITP usually present

A 
Rarely Dangorous, but looks dramatic: 
Petechiae (tiny purple/brown spots on skin) 
Mucosal Bleeding
Easy bruising
Chronic: GI bleeding, nose bleeding
53
Q

How is ITP treated

A

• Treatment rarely indicated except TXA

– Occasionally needs treatment with steroids, IV Immunoglobulin or Splenectomy

54
Q

What are Coagulopathies

A

Disorders of the clotting cascade

55
Q

What are two bleeding disorders

A

Hemophillia

Von Willebands Disease

56
Q

What is Von Willebands Disease

A

Deficiency of vWF (von Willeband Factor) leading to: -

  • platelet dysfunction (platelets cant stick together)
  • Factor VIII Deficiency
57
Q

How does VW Disease present

A

Type 1 - AD - mild bleeding can be asymptomatic
Type 2 - AD - moderate bleeding e.g mucosal bleeding from nose and GI and prolonged bleeding after surgery
Type 3 - AR - Severe - Joint and muscle bleeding

58
Q

How is VW investigated

A

• History
– often mild bleeding (e.g. bruising, epistaxis, primary
menorrhagia)
• Investigation
– Clotting screen may be normal or APTT increased
– vWF and Factor VIII variably decreased

59
Q

What is the management of VW

A

For bleeds/surgery you can give:
– Tranexamic acid
– Desmopressin - can increase vWF and factor VIII
– Severe: Factor VIII/VWF plasma concentrates

60
Q

What is Haemophillia A and who does it affect

A

Deficiency of Factor VIII

X linked recessive - affecting males

61
Q

What is the presentation of Haemophillia A and Haemophillia B

A 
Level <1 - Severe disease - frequent spontaneous prolonged bleeding into muscles and joints leading to deformity and arthritis 
Neonatal
– FHx
– Cephalohaematoma/ICH
– Iatrogenic bleeding
– Umbilical cord bleeding
Early childhood
– Classically <2yrs once mobile presents with:
• Easy bruising/soft tissue haematomas 
• Mouth bleeds- often trauma related
• Muscle/joint bleeds

Level 1-5 - moderate disease - associated with severe bleeding following injury an occasional spontaneous episodes

Level >5 - mild disease - may present later with bleeding following trauma or surgery/dental extraction

62
Q

What will be found on investigation of Haemophillia A

A

Prolonged APTT and low plasma level of Factor VIII

63
Q

How is Haemophillia A managed

A

IV infusion of recombinant factor VIII concentrate

64
Q

What is Haemophillia B

A

Deficiency of Factor IX

X linked recessive affecting males

65
Q

What will be found on investigation of Haemophillia B

A

Raised APTT and low plasma level of Factor IX

66
Q

How is Haemophillia B managed

A

IV infusion of recombinant Factor IX concentrate

67
Q

What is Diamond Blackfan Anaemia and how does it present

A 
physical anomalies- at least 50%
• Cranio-facial
• thumb 10-20%
• Deafness
• Musculoskeletal
• Renal
• Cardiac
• Growth retardation
68
Q

What are some causes of Hypercoagulation

A 
antithrombin,  
protein C, 
protein S, 
FVL, 
PT mutation,
APS
69
Q

How do you diagnose thalassaemia

A

Blood Sample: microcytic hypochromic anaemia
Hb Electrophoresis: reduced/absent HbA , elevated HbA2 and increased levels of HbF
DNA analysis

Paediatrics (13 decks)

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