Anaemia Flashcards
Anaemia definition
reduction of total circulating red cell mass below normal limits - reduces oxygen carrying capacity of blood
reduced oxygen carrying capacity of blood could lead to
tissue hypoxia
etiological clues to anaemia (3)
red cell size, shape, degree of haemoglobinisation
3 red cell sizes
normocytic, microcytic, macrocytic
2 degrees of haemoglobinisation
normochromic, hypochromic
microcytic and hypochromic anaemia suggest
haemoglobin synthesis disorder
macrocytic anaemia suggests
maturation in bone marrow abnormalities
leg ulcers suggest
sickle cell anaemia
bone deformities suggest
thalassaemia major
jaundice suggests
haemolytic anaemia
koilonychia (spoon-shaped nails) suggests
iron deficiency anaemia
3 features of haemolytic anaemias
premature destruction and short life span RBCs, accumulation of breakdown material, elevated erythropoietin and rate of erythropoiesis
cause of extravascular haemolytic anaemia
alterations which make RBCs less deformable
causes of intravascular haemolytic anaemia (4)
mechanical injury, complement fixation, intracellular parasites, exogenous toxic factors
3 signs of extravascular haemolytic anaemia
anaemia, splenomegaly, jaundice (bilirubin)
3 signs of intravascular haemolytic anaemia
anaemia, jaundice (bilirubin), haemoglobinaemia
process extravascular haemolytic anaemia
can’t navigate splenic pulp > red cell sequestration > phagocytosis within cords > some escapes
process intravascular haemolytic anaemia
large amount Hb free from lysed cells > bound to HAPTOglobin > rapidly cleared by phagocytes > Hb oxidised to METHEMOglobin (brown) > some passes in urine
Jaundice is from
iron build up in kidney > bilirubin
genetics of sickle cell disease
maturation in 6th codon of beta globin > replacement of glutamate residue with valine residue
in sickle cell disease, glutamate residue is replace with
valine residue
haemoglobin type of sickle cell sufferers
HbS
HbS heterozygous phenotype
sickle cell trait
sickle cell trait protects against
malaria
sickle cell trait symptoms
asymptomatic
sickle cell disease and trait most common in people of what descent
african
3 major pathological manifestations of sickle cell anaemia
chronic haemolysis, microvascular occlusions, tissue damage
4 variables affecting sickling
interaction of HbS with other types of Hb in cell, mean cell Hb concentration, transit time of red cells through microvascular beds, intracellular pH
sickle cell trait, percentage HbS and HbA
HbS = 40%, HbA = 60%
HbA interferes with
HbS polymerisation
RBCs of people with sickle cell TRAIT do not sickle unless under what conditions
hypoxic conditions
intracellular what facilitates sickling
dehydration
intracellular dehydration facilitates sickling because
increases mean cell Hb conc.
transit times slow where (3)
spleen, bone marrow, inflamed vascular beds
why might vascular beds be inflamed
increased adhesion molecules
sickle red cells express higher than normal amounts of what
adhesion molecules
decrease in pH reduced Hb affinity for what
oxygen
reduced Hb oxygen affinity increases fraction of
deoxygenated HbS
low pH increases tendency of
sickling
sickle cell disease, splenomegaly can be seen in
children
autosplenectomy seen in
adults with sickle cell disease
progressive hypoxic tissue damage leads to
autosplenectomy
how does sickling damage RBCs
HbS polymers grow > herniate membrane > influx calcium ions into RBC > efflux potassium ions and water > dehydration > increased density and rigidity