other Flashcards
what are insoluble protein fibrils
amyloid
most common presenting symptom of amyloidosis
weakness and dyspnoea
what amyloidosis refers to light chain and what one refers to inflammatory
Light - AL
inflammatory - AA
commonest reason doe hereditary haemochromatosis
mutation in HFE gene
mx for hereditary haemochromatosis
weekly venesection
mx for secondary iron overload
iron chelating agents eg desferrioxamine
increased destruction cause of pancytopenia
hypersplenism
causes of hypersplenism
portal hypertension, systemic disease eg RA, haematological disease eg splenic lymphoma
what pancytopenia has hypocellularity
aplastic anaemia
minimum criteria for blood transfusion donor
Hb 125 for women and 135 for men and weight 50kg
ABO gene is on chromosome
9
O blood can only receive what blood
0 blood
what is antisera
using reagents with known antibody specificity to identify antigens present on red cell
what does agglutination indicate
presence of an antibody
when do you infuse FFP
- Treatment of bleeding in patient with coagulopathy (PT ratio >1.5)
- Prior to surgery or procedure in patient with coagulopathy (PT ratio >1.5)
what is febrile non haemolytic transfusion reaction
presents with fever, rigours/chills but patient otherwise well
mx of febrile non haemolytic transfusion reaction
slow the transfusion and give paracetamol
which protein modulates transferrin uptake (iron overload)
HFE
what can lower iron levels in iron overload
desferrioaxamine
patients post splenectomy have what
Howell-jolly bodies and Pappenheimer bodies
highest risk of infection after splenectomy is when and with what bacteria
2 years after and from pneumococcal
lifelong antibiotics prophylaxis post splenectomy is what antibiotic
Phenoxymethylpenicllin also know as penicillin V
COX is necessary to produce what
thromboxane 2
what should be stopped 7 days prior to elective operations
anti platelet agents
what are ADP receptor antagonists
Clopidogrel and Prasugrel
what is a phosphodiesterase inhibitor
Dipyridamole
what drug is a GP2b/3a inhibitor
abciximab
how do you monitor unfractionated heparin
APTT
monitoring for LMWH
no usually needed as more predictable response
what can reverse heparin in severe bleeding
protamine sulpahte
what drug should be taken at the same time every day
warfarin
dabigatran MOA
direct thrombin inhibitor
moa of edoxaban, rivaroxaban, apixaban)
factor X inhibitors
haematopoiesis is the term used
for the production of blood cells
adult where is haemopoiesis restricted to a
axial skeleton
granulocytes include what
neutrophils, eosinophils and basophils
what cell are bi lobed and usually have bright red/ orange granules
eosinophils
what do basophils look like
large deep purple granules obscuring nucleus
what cell has a large single nucleus, faintly staining granules and are often vacuolated
monocyte
small with condensed nucleus and rim of cytoplasm, when activated appear large with plentiful blue cytoplasm extending round neighbouring red cells
lymphocyte
what shows lineage of progeny
bioassays
how do red cells have a limited lifespan
there is no nucleus so they can’t divide or replace damaged proteins
adult haemoglobin is
2 alpha and 2 beta chains
red cell production is regulated by what and is produced in hypoxia
erythropoietin
red cell destruction normally occurs where
Spleen (and liver)
the heme group is broken down into what
iron and bilirubin
NADPH replenishes what in the hexose monophosphate shunt
glutathione
in the hexose monophosphate shunt what is the rate limiting enzyme
Glucose -6 - phosphate dehydrogenase
most co2 is transported with
bicarbonate
what is increased in chronic anaemia
2-3 DPG
where does iron absorption mainly happen
duodenum
what decreases iron absorption
tea, calcium, phylates
what increases iron absorption
ascoribic acid and alcohol
what is the major negative regulator of iron uptake
hepcidin
what levels decrease when iron deficient
hepcidin
molecule that is spherical and stores iron
ferritin
what measures functional iron
haemoglobin conc
what also acts as an acute phase protein so goes up with infection, malignancy, liver injury etc
serum ferritin
what is secondary haemostasis
formation of fibrin clot
von willebrand factor deficiency has what inheritance
autosomal dominant
drugs that can reduce platelet function
aspirin or NSAIDs
the only really screening test for primary haemostasis
platelet count
what converts prothrombin to thrombin
V/Xa
What converts fibrinogen to fibrin
thrombin
what is a single clotting factor deficiency
haemophilia
what has an isolated prolonged APTT
haemophilia
examples of what has multiple clotting factor deficiences
DIC, liver failure (as all coagulation factors are synthesises in the liver)
prothrombin time relates to what pathway
extrinsic
what is used as a measure of overall clotting
prothrombin time
APTT measures what factors
8,9,11 and 12
most common causes of increased APTT are
haemophilia and possibly von willebrands disease
most common reasons for both prothrombin and APTT to be increase
due to multiple clotting factor deficiencies eg liver diease, anticoagulants
what breaks down fibrin
plasmin
anti- thrombin, protein C and S are
anticoagulant defences
what cell only lives for 7-8hrs
neutrophils
what are nucleated precursor cells
blasts
what are immediate red cell precursor
reticulocytes
what means you have more immature red blood cells than what considered normal
polychromasia
what is a myelocyte
nucleated precursor between neutrophils and myeloblasts
what is used for bone marrow biopsy in a young child
tibia
what marrow increases with age
yellow
are lymph nodes primary or secondary lymphoid tissues
secondary
where does B cell maturation occur
bone marrow
where does T cell maturation occur
thymus
what things stimulate a predominant T cell response
viral infections, drugs
what cell response predominates in autoimmune conditions and infections
B
what is troiseries sign
manifestation of metastasis from an abdominal malignancy
what is a sentinel lymph node
first lymph node to which cancer cells are more likely to spread
where will metastatic cells be identified first
subscapular sinus
where is the spleen located
upper left quadrant of the abdomen
there can be lack of differentiation or maturation in
malignant haemopoiesis
in what there is a proliferation of abnormal progenitors with a block in differentiation/maturation
acute leukaemia. in acute there is a block and in chronic there is no differentiation/ maturation
is normal haeompoiesis mono or poly clonal
polyclonal
myeloma is malignancy of what cell
plasma
CLL affect more what
mature B lymphocytes
leukaemia affects blood and lymphoma affects the lymph node. but what is an exception
CLL - can involve both
what leukemias are usually more aggressive
acute
immunoglobulin are produces by what cells
B and plasma
where do B cells encounter the antigen
follicle germinal centre
what is a monoclonal immunoglobulin
paraprotein
what allows for identification of an abnormal protein band
serum electrophoresis
what allows to classify the abnormal protein band
serum immunofixation
bence jones protein has immunoglobulin … chains
light
