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Haematology - Coggle Diagram
Haematology
Sickle cell
Definition
Beta haemoglocin gene
Chromosome 11
Autosomal recessive
Valine for glutamate
Features
Polymerisation (worse in hypoxia)
Haemolytic anaemia
Microvascular occlusion
Thrombosis
Sickle crisis
Veno occlusive
Acute chest syndrome
Stroke
Avascular necrosis
Abdominal pain
AKI
Sequestertration
Splenic
immunisation
antibiotic prophalaxis
Aplastic
Parvovirus B19
Folate deficiency
Investigations
Blood film
Howell-Jolly bodies
Sickledex test
Electrophoresis
Indications for exchange transfusion
Acute chest syndrome
Stroke
Acute hepatic dequestration
Multiorgan failure
Haemophilias
Factor V leiden
Autosomal dominant
APC not inactivated
Thrombophilia
Antiphospholipid syndrome
Diagnostic criteria
One clinical event
Two antibody tests at least three months apart
Lupus anticoaglulant
Anticardiolipin antibodies
Management
Aspirin
PLasma exchange
Haemophilia A ( Factor VIII)
DDAVP
TXA
Clotting factor concentrate
Haemophlia B (Factor IX)
Clotting factor concentrate
vWF
Type 1 85% - mild
Type 2 15% functional defect
Type 3 severe deficincy
DDAVP 0.3mcg/kg +fVIII concentrate
DIC
Pathophysiology
intravascular activation of coagluation
excessive thrombin
Excessive thrombogenesis
Causes
Sepsis
Trauma
Obstetrics
placental abruption
Pre-eclampsia
PPH
Clinical features
Bleeding
eccymoses on skin
Mucosal eg gums, bowel
Thrombosis related
AKI
hepatic dysfunction
respiratory failure
PE
alveolar haemorrhage
ARDS
CNS dysfunction
Stroke
SAH
Diagnosis
International society on thrombosis and haeostasis (ISTH)
underlying precipitant
Platelet count
d-dimer
Fibrongen
PT prolongation
Score >5
TEG
Short R
Increased alpha
increase MA
Secondary fibrinolysis - high Ly30/Ly60
Thalassaemia
Definition
Alpha - 1 to 4 genes
Beta
minor
major
Management
Transfusion support <100g/l
infection prevention
Yersinia infection
Diarrhoea
Iron-overload