Haematology Flashcards
(69 cards)
1
Q
What are the three sizes of RBC in anaemia
A
Microcytic
Normocytic
Macrocytic
2
Q
What are the two colours of RBC
A
Hypochromic - pale
Normochromic
3
Q
What are reticulocytes
A
Immature RBC
4
Q
What does low reticulocytes mean
A
Low - lack of production
High - hemolysis/ blood loss
5
Q
What are most Microchromic anaemias
A
Hypochromic
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
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
8
Q
What are examples of Macrocytic Anaemia
A
Vitamin B12 deficiency Folate Deficiency Hypothyroidism Alcohol Access Chronic Liver disease
9
Q
What are the two types of Anaemia
A
Underproduction:
- Microcytic
- Normocytic
- Macrocytic
Normal Production:
- Haemolysis
10
Q
What are causes of severe anaemia at birth
A
Haemolytic disease of the newborn
Bleeding: umbilical cord, internal haemorrhage
11
Q
What is severe anaemia in the newborn called due to rhesus haemolytic disease
A
Erythroblastosis Fetalis
12
Q
How does erythroblastosis fetalis occur
A
- Rh negative mothers previously sensitised to Rh+ve blood from previous pregnancy
- Production of Rhesus Antibodies
- These Antibodies cross the placenta
- These cause haemolysis of fetal RBC
13
Q
What are the signs and symptoms of erythroblastosis fetalis
A
Hepatospelomegaly
Severe Anaemia
Jaundice
Oedmatous Fetus
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
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
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
17
Q
What is the role of breast milk for RBC
A
Erythopotein in Breast milk can help stimulate hematopoiesis
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
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
20
Q
How does iron deficiency anaemia present
A
Pallor Irritability Anorexia Tachycardia Cardiac Dilation Murmur
21
Q
What is the treatment for iron deficient anaemia
A
Oral Iron (Ferritin) Therapy Common cause of failure non-compliance
22
Q
What is Haemolysis
A
Increased RBC turnover/ destruction
shortened RBC lifespan e.g fragile abnormal ones
23
Q
What are intra corpuscular causes of increased RBC destruction
A
- Haemoglobin
- Enzyme
- Membrane
24
Q
What are extra corpuscular causes of increased RBC destruction
A
Autoimmune
Spleen hypertrophy/ Spleenomegaly
Plasma Factors
Fragmentation
25
What is a complication of long term haemolysis and/or transfusions
Iron Overload - affects all organs e.g Liver faliure, Heart Failure
26
How do you manage iron overload
Ferritin monitoring + imaging
| Iron Chelation
27
What are two examples of haemoglobinopathies
Sickle cell disease
| Thalassaemia
28
How are haemoglobinopathies diagnosed
- HPLC - High Performance Liquid Chromatography
| - Hb Electrophoresis
29
What is Sickle Cell Disease
AR disorder - causing production of abnormal beta globulin chains - HbS rather than HbA
Common in African Origin
30
What is the Pathogenesis of Sickle Cell
HbS polymerises when deoxygenated causing RBCs to deform - producing sickle cells
Sickle Cells - Fragile and Haemolsye, they also occlude small vessels
31
How do you screen for Sickle Cell in neonates
Hb Electrophoresis
32
What is the presentation of Sickle Cell
- 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
What will be found on Ix of Sickle Cell
- HPLC/Hb electrophoresis - HbS - no presence of HbA
- Anaemia
- Film - Sickle Cells
- Raised Reticulocytes
- Raised WCC
34
How can Sickle Cell be cured
Stem Cell Transplant
35
What should Sickle Cell patients with a fever do
Seek Help!!
Blood Cultures, CXR
IV fluids, Antipyretics
Hospitalise for any pneumonia
36
How do you manage pain crisis in sickle cell
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
What is Acute Chest Syndome SS commonly caused by how does it present and how is it managed
Infarction or Infection
- Tachypnoea, Chest pain, Fever, Cough, Wheeze
- Admit - IV fluids, blood transfusion, O2 and Abx
38
What is an Aplastic Crisis caused by in SS
Paravirus B19
39
How do you treat SS disease
* Hydroxycarbamide
* Transfusion – Prevent strokes for those at highest risk
* Stem cell transplants – In severe cases e.g strokes, severe crisis
40
What is Thalassaemia
Genetic Disorder resulting in faulty Hb chain synthesis
41
What is Beta Thalassaemia
Autosomal Recessive - Beta Haemoglobin chains are coded for by two alleles
Thalassaemia Minor - one defective allele (carrier)
Thalassaemia Major - two defective alleles
42
How does Beta Thalassaemia present
Minor: unremarkable symptoms, may be mildly anaemic
Major:
- Severe haemolytic anaemia
- Growth retardation
- Hepatosplenomegaly
- Skeletal Deformities e.g skull bossing
43
How do you manage Beta Thalassaemia
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
How do Haemolytic Anaemias present
* Hydrops fetalis
* Neonatal hyperbilirubinaemia
* Neonatal ascites
* Anaemia/failure to thrive
* Splenomegaly
* Cholecystitis/gall stones
* Hyperbilirubinaemia
* Leg ulcers
* Aplastic crisis
* Thromboembolism
45
What is Alpha Thalassaemia
Mainly caused by deletion of 4 genes
- All 4 - death in utero
- 3 - moderate anaemia and haemolysis
- 2 - asymptomatic - carrier
- 1 - normal
46
What is Glucose 6 Phosphate Dehydrogenase Deficiency
X linked chief RBC enzyme defect
| mainly effects males
47
How does G6PD present
• 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
What will see on RBC film of G6PD sporadic haemolysis
Bite and Blister Cells
49
What is the commonest type of hereditary haemolysis in europeans
Hereditary Spherocytosis - Sphere shaped RBC
50
What is the commonest type of Immunological Thrombocytopenia
Idiopathic/Immune Thrombocytopenic Purpura - immune destruction of platelets
Can be acute or chronic - usually recovers over weeks or months
51
When does acute ITP usually occur
In children following a viral infection
52
How does ITP usually present
```
Rarely Dangorous, but looks dramatic:
Petechiae (tiny purple/brown spots on skin)
Mucosal Bleeding
Easy bruising
Chronic: GI bleeding, nose bleeding
```
53
How is ITP treated
• Treatment rarely indicated except TXA
| – Occasionally needs treatment with steroids, IV Immunoglobulin or Splenectomy
54
What are Coagulopathies
Disorders of the clotting cascade
55
What are two bleeding disorders
Hemophillia
| Von Willebands Disease
56
What is Von Willebands Disease
Deficiency of vWF (von Willeband Factor) leading to: -
- platelet dysfunction (platelets cant stick together)
- Factor VIII Deficiency
57
How does VW Disease present
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
How is VW investigated
• 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
What is the management of VW
For bleeds/surgery you can give:
– Tranexamic acid
– Desmopressin - can increase vWF and factor VIII
– Severe: Factor VIII/VWF plasma concentrates
60
What is Haemophillia A and who does it affect
Deficiency of Factor VIII
| X linked recessive - affecting males
61
What is the presentation of Haemophillia A and Haemophillia B
```
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
What will be found on investigation of Haemophillia A
Prolonged APTT and low plasma level of Factor VIII
63
How is Haemophillia A managed
IV infusion of recombinant factor VIII concentrate
64
What is Haemophillia B
Deficiency of Factor IX
| X linked recessive affecting males
65
What will be found on investigation of Haemophillia B
Raised APTT and low plasma level of Factor IX
66
How is Haemophillia B managed
IV infusion of recombinant Factor IX concentrate
67
What is Diamond Blackfan Anaemia and how does it present
```
physical anomalies- at least 50%
• Cranio-facial
• thumb 10-20%
• Deafness
• Musculoskeletal
• Renal
• Cardiac
• Growth retardation
```
68
What are some causes of Hypercoagulation
```
antithrombin,
protein C,
protein S,
FVL,
PT mutation,
APS
```
69
How do you diagnose thalassaemia
Blood Sample: microcytic hypochromic anaemia
Hb Electrophoresis: reduced/absent HbA , elevated HbA2 and increased levels of HbF
DNA analysis
