Haemolytic anaemia Flashcards
state 5 signs of intravascular haemolysis?
haemoglobinaemia haemogloburia decreased serum haptoglobins increased urine haemosiderin methaemalbuminaemia
what is a sign of extravascular haemolysis ?
splenomegaly
state two enzyme causes of haemolysis ?
G6PD and pyruvate kinase deficiency
what is the inheritance of G6PD deficiency?
X linked
what process is G6PD used in?
pentose phosphate shunt
what are some triggers of haemolysis in G6PD deficiency ?
broad beans
infection
drugs - antimalarials, henna, sulphonamides
what 4 things are seen on a blood film for G6PD deficiency ?
blister cells
bite cells
ghost cells
Heinz bodies
what is the treatment for G6PD deficiency ?
treat underlying cause
transfusion may be needed
what is the inheritance of pyruvate kinase deficiency ?
autosomal recessive
what are the three features of pyruvate kinase deficiency ?
jaundice, splenomegaly, anaemia
what is seen on the blood film of B12/folate deficiency ?
oval macrocytes
hypersegmented PMN
what is seen on the blood film of alcohol caused microcytic anaemia ?
target cells
what is the role of B12 in the body?
DNA and myelin synthesis
what is glossitis ?
beefy red tongue seen in macrocytic anaemia
state four neuro complications of b12/folate deficiency ?
paraesthesia
peripheral neuropathy
optic atrophy
SACD
what is SACD?
subacute combined degeneration of the spinal cord
what is SACD usually caused by?
pernicious anaemia
what are the signs of SACD?
combined symmetrical dorsal column and corticospinal tract loss
- mixed UMN and LMN signs
- spastic paraparesis
- brisk knee jerks
- absent ankle jerks
- up going plantars
temp and pain intact!!
what are the two antibodies in pernicious anaemia?
intrinsic factor abs
parietal cells abs
what cancer are pernicious anaemia patients more at risk of ?
gastric adenocarcinoma
auto immune haemolytic anaemia warm is mediated by what?
IgG
auto immune haemolytic anaemia cold is mediated by what?
IgM
what are the causes of auto immune haemolytic anaemia warm ?
idiopathic
SLE
RA
Evans
what is auto immune haemolytic anaemia cold coombs test positive for?
complement only
what is PNH haemolytic anaemia ?
paroxysmal nocturnal haemoglobinuria
what causes paroxysmal nocturnal haemoglobinuria ?
absence of RBC anchor molecule GPI which decreases complement and results in intravascular haemolysis
what does paroxysmal nocturnal haemoglobinuria bloods result in ?
BMF
anaemia, thrombocytopenia, neutropenia
low complement CD55 and CD59
What is haemolytic uraemic syndrome caused by?
E coli 0157
what does haemolytic uraemic syndrome result in ?
bloody diarrhoea abdo pain microangiopathic hemolytic anemia thrombocytopenia renal failure
what cells are seen on the blood film for haemolytic uraemic syndrome and TTP?
schistocytes
what is the treatment for haemolytic uraemic syndrome?
usually resolves on its own
what acquired deficiency can cause TTP?
ADAMTS13
what does MAHA stand for?
microangiopathic hemolytic anemia
what are the signs of TTP?
microangiopathic hemolytic anemia
fever
thrombocytopenia
renal failure
what is the treatment for TTP?
plasmapheresis
immunosuppression
splenectomy
what is the commonest inherited haemolytic anaemia ?
hereditary spherocytosis
what is the inheritance of hereditary spherocytosis?
autosomal dominant
what are three features of hereditary spherocytosis?
splenomegaly
pigment gallstones
jaundice
is hereditary spherocytosis immune mediated ?
no
what are some triggers for sickle cell?
infection
cold
hypoxia
dehydration
what is the presentation of sickle cell? SICKLED
splenomegaly (which may cause sequestration crisis - fills with blood) infarction crises (pulmonary, mesenteric) kidney disease liver/lung disease erection dactylitis
- last due to thrombosis from sickled cells clumping together and blocking blood in the capillary system
what is the treatment for sickle cell?
penicillin
immunisations
folate
