haemolytic anaemia Flashcards
what is haemolytic anaemia
destruction of red blood cells resulting in low haemoglobin concentration (anaemia)
inherited conditions that lead to chronic haemolytic anaemia
hereditary spherocytosis
hereditary elliptocytosis
thalassaemia
sickle cell anaemia
G6PD deficiency
features of haemolysis
anaemia
splenomegaly
jaundice
investigations in haemolytic anaemias
FBC: normocytic anaemia
blood film: schistocytes
direct coombs test
coombs positive acquired haemolytic anaemias
autoimmune: warm/cold antibody type
alloimmune: transfusion reaction, haemolytic disease newborn
drug: methyldopa, penicillin
acquired coombs negative causes of haemolytic anaemia (non-immune causes)
microangiopathic haemolytic anaemia
prosthetic heart valves
paroxysmal nocturnal haemoglobinuria
infections: malaria
drug: dapsone
zieve syndrome
what is hereditary spherocytosis
most common inherited haemolytic anaemia in north europe
autosomal dominant conditions
fragile, sphere-shaped red blood cells break down passing through the spleen
presentation of hereditary spherocytosis
anaemia
jaundice
gallstones
splenomegaly
aplastic crisis in presence of parovirus
family history
key investigations findings in hereditary spherocytosis
raised MCHC on FBC
raised reticulocyte count
spherocytes on blood film
EMA binding test used to diagnose
treatment of hereditary spherocytosis
folate supplementation
blood transfusions when required
splenectomy
what is hereditary elliptocytosis
similar to hereditary spherocytosis except red blood cells are ellipse-shaped
autosomal dominant
what is G6PD deficiency
caused by defect in the gene coding for G6PD, enzyme responsible for protecting the cells from oxidative damange
X-linked recessive
presentation of G6PD deficiency
jaundice- often neonatal
gallstone
anaemia
splenomegaly
heinz bodies
haemolytic anaemia triggered by infections, drugs or fava beans
key drugs: ciprofloxacin, sulfonylureas, sulfasalazine
when does autoimmune haemolytic anaemia occur
when antibodies are created against the patients red blood cells
can be warm or cold based on the temperature which the auto-antibodies destroy the red blood cells
more common type of autoimmune haemolytic anaemia
warm
management of autoimmune haemolytic anaemia
blood transfusions
prednisolone
rituximab
splenectomy
two scenarios where alloimmune haemolytic anaemia occurs
transfusion reactions
haemolytic disease of the newborn
presentation of paroxysmal nocturnal haemoglobinuria
red urine in the morning which contains Hb and haemosiderin
anaemia
thrombosis
smooth muscle dystonia (oesophageal spasm and erectile dysfunction)
management of paroxysmal nocturnal haemoglobinuria
eculizumab or bone marrow transplantations
when can microangiopathic haemolytic anaemia
HUS
disseminated intravascular coagulation
thrombotic thrombocytopenic purpura
SLE
cancer
