questions Flashcards
what are immunoglobulins
produced by B-cells and plasma cells
each immunoglobulin recognises a specific antigen
can be expressed on B cell surface or released into blood stream as antibodies by plasma cells
what are immunoglobulins made up of
2 heavy chains- determines the class of antibody produced
2 light chains
together the type of heavy and light chains are called isotype of the antibody
common blood results in alcoholic liver disease
macrocytic anaemia and thrombocytopenia
first line management of patient with autoimmune haemolytic anaemia
steroids
basophilic stippling of red blood cells means
lead poising or siderblastic anaemia
presentation of siderblastic anaemia
microcytic anaemia
fatigue
pallor
SOB
where is folate absorbed
the jejunum
causes of B12 deficiency
not enough dietary intake
pernicious anaemia
gastrectomy
malabsorbtion: crohn’s
medicine: metaformin
causes of folate deficiency
diet
malabsorption: coeliac
excessive peeing: heart failure, liver damage, long term dialysis
medicine: anticonvuslants
pregnancy
malignancy
exfoliating dermatitis
what is pernicious anaemia
autoimmune condition resulting in destruction of gastric parietal cells > intrinsic factor deficiency
importance of B12 and folate
co-factors in DNA synthesis and nuclear maturation and DNA modification and gene activity
how is B12 metabolised
freed from protein complexes in food by gastric enzymes
then binds to R binder (binding protein)
released from R binder by pancreatic enzymes and then binds to intrinsic factor
intrinsic factor carries B12 to specific receptor on mucosa of ileum
B12 inters ileal cells and IF is excreted
how is folate absorbed
dietary folates converted to monoglutamate
absorbed in jejunum
stores are lower than for B12
presentation of B12/folate deficiency
anaemia
weight loss
diarrhoea
infertility
sore tongue
jaundice
development problems
why can patients with macrocytic anaemia appear jaundiced
due to ineffective erythropoiesis caused by intramedullary haemolysis
meaning a breakdown of cell within the bone marrow
B12 deficiency specific symptoms
neurological problems
- posterior/dorsal column abnormalities
- neuropathy
- dementia
- psychiatric manifestations
management of pernicious anaemia
vit B12 injections for life- hydroxocobalamin
causes of microcytic anaemia
TAILS
- thalassaemia
- anaemia of chronic disease
- iron deficiency
- lead poising
- sideroblastic
what causes anaemia of chronic disease
IL-6 is released due to chronic diseases and increases production of hepcidin > this down regulates ferroportin expression, reducing iron absorption and precipitating anaemia
what is sideroblastic anaemia
excess iron buildup in mitochondria due to failure to incorporate iron into haem
can be hereditary
most common type of Hodgkin’s lymphoma
nodular sclerosing
bleeding and bruising, petechial and purpuric rash
often with a history of viral illness
immune thrombocytopenic purpura
management of immune thrombocytopenic purpura
prednisolone
IV immunoglobulins
blood transfusion
platelet transfusions only work temporarily
management of thrombotic thrombocytopenic purpura
IV plasma exchange
splenectomy
what does cryoprecipitate contain
factor VIII, fibrinogen, von Willebrand factor and factor XIII
when is hydroxyurea used in sickle cell disease
to prevent a further crisis if the patient has had repeated attacks
diagnostic of beta thalassaemia trait
raised HbA2
presentation of beta thalassaemia major
presents aged 6-24 months (as HbF falls)
pallor
failure to thrive
hepatosplenomegaly
skeletal changes
organ damage
haemoglobin analysis of beta thalassaemia major
mainly HbF
no HbA
management of beta thal major
regular transfusion programme to maintain Hb
- risk of iron overload from transfusion
bone marrow transplant may be option
consequences of iron overload
endocrine dysfunction: impaired growth and pubertal development, diabetes, osteoporosis
cardiac disease: cardiomyopathy, arrhythmias
liver disease: cirrhosis, hepatocellular cancer
management of iron overload
250mg of iron per unit packed red cells
chronic anaemia also drives increased iron absorption
iron chelating drugs: desferrioxamine
what type of haemophilia is turners related to
haemophilia A
- because it is X linked recessive and people with turners only have one X chromosome
