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Abnormal Blood Cells : (Sickle Cell (genetics (haemoglobin S, point…
Abnormal Blood Cells :
Sickle Cell
genetics
haemoglobin S
point mutation in beta globin chain
autosomal recessive
pathogenesis
polymerisation of Hb
RBC sickles
RBC gets stuck
acute complications
painful crises
sickle chest syndrome
looks like pneumonia on CXR
CVA in childhood
chronic complications
renal impairment
pulmonary HTN
avascular necrosis
management
transfusion
stem cells transplant
chemotherapy
Thalassaemia
diminished synthesis of one or more globin chains
reduction in Hb
intermedia
anaemia
major
transfusion dependent
iron overload
HF
pancreatic failure
pituitary dysfunction
diabetes
beta
fail to feed
listless crying
pale
Hb 40-70g/l
ferrtin normal
DEXA scans
alpha
inherit pair from mum and dad
symptomatic at 2-3/4
membranopathies
autosomal dominant
neonatal jaundice
gallstones
examples
spherocytosis
elliptocytosis
treatments
folic acid
splenectomy
enzymopathies
enzyme pathways fuel the cell
deficiencies may shorten RBC life by oxidative damage
example: G6PD crises
haemolysis
jaundice
anaemia
brought on by green beans and quinines
but common in malarial areas
Polycythaemia
primary cause
polycythaemia rubra vera
secondary causes
low FiO2
too much EPO/androgen
smoking
lung disease
presentation
red face
itching
thrombosis
splenomegaly
management
aspirin
bone marrow suppressive drugs
venesection