haemolytic anaemia Flashcards
symptoms haemolytic anaemia
low Hb, reticulocytosis, low haptoglobin, raised LDH and bilirubin
what are reticuloytes
RBC released from bone marrow, mature after 2-3 days
bloods reticuloytes
polychromatic, blue
where does extravascular haemolysis occur
spleen, liver, lymph nodes
causes of extravascular haemolytic anaemia
haemoglobinopathy // spherocytes // haemolytic disease newborn // warum autoimmune haemolytic anaemia
findings extravascular haemolysis
bilirubin, splenomegaly, gallstones, jaundice
in intravascular haemolytic anaemia what does free Hb bind too
haptoglobin + albumin
lab findings in intravascular haemolytic anaemia
Hemoglobinemia (Hb), Methaemalbuminaemia, hemosiderinuria (fe) , haemolgoburia
urine in intravascular haemolysis
pink –> black on standing
causes intravascular haemolytic anaemia
blood transfusion // G6PD // heart valve // TTP // DIC // HUS // Paroxysmal nocturnal haemoglobinuria // cold autoimmune haemolytic anaemia
inherited causes haemolytic anaemia
spherocytosis // G6PD // haemoglobinopathy
acquired immune haemolytic anaemia
autoimmune // alloimmune eg transfusion, haemolytic newborn // drug eg methyldopa
causes non-immune haemolytic anaemia
TTP, HUS, DIC // prsothetic heart valves // paroxysmal nocturnal haemoglobinuria // malaria // dapson // Zieve syndrome
what is Zieve syndrome
alcohol binge –> haemolysis, jaundice, hyperlipideamia
how is non-autimmune vs autoimmune acquired haemolytic aneamia differentiated
coombes
inheritance hereditary spherocytosis
autosomal dominant
symptoms hereditary spherocytosis
northern european // failure to thrive // jaundice, gallstones, splenomegaly (extraV) // aplastic crisis
what precipitates aplastic crisis
parvovirus
bloods + lab findings hereditary spherocytosis
film - spherocytes // bloods = raised reticulocytes, raised MCHC, raised bilirubin, low Hb
if blood tests borderline for hereditary spherocytosis what testing is diagnostic
EMA binding test (+ cyroheamolysis test // atypical = eletophoresis)
mx hereditary spherocytosis
acute crisis = supportive +/- transfusion // long term = folate + splenectomy
inheritance G6PD def
X linked (more common in men)
consequences G6PD deficiency
reduces NADPH + glutathione so more oxidation on red blood cells
what can cause G6PD deficiency presentation
infection, fava beans, medications
