HAEM 2 Flashcards
primary iron overload is what pathology symptoms in who genetics
hereditary haemachromatosis
long term excess iron absorption with parenchymal rather than macrophage iron loading and essentially organ damage
weakness, fatigue, joint pains, impotence, arthritis, DM, CM
middle age or later
mutation in HFE gene/other iron regulatory proteins
ix for haemachromatosis
family screening
treatment
transferrin sat >50%
ferritin in men/post menopausal women >300
>200 in women
liver biopsy if uncertain/assess organ damage
HFE genotype and ferritin and transferrin sat
weekly phlebotomy 450-500mls = 200/250mg of iron
get ferritin <20 then keep <10mcg/L or <75%
secondary cause of iron overload
how much iron is in a transfusion
symptoms
treatment
repeated cell transfusion, overactive erythro, thal, siderblastic anaemia, myelodysplasia, RC aplasia
200-250mg/unit
damage to liver, heart, endocrine organs
venesection not an option in anaemic patients
desferoxamine, deferiprine, defeasirox
anaemia of chronic disease why
inflammation of chronic disease leading to an increase hepicidin leading to internalisation of ferroportin. ferroportin transports iron into the cell out into bloodstream. decreased ferroportin - decreased access of iron to circulation
acute blood loss steps
what can be given to reduce blood loss in surgeries
observations for transfusion
arrest bleeding. gain IV access. samples for cross matching etc. restore and maintain blood volume saline, albumin, gelofusion. transfuse when Hb <70 group A Rh neg blood
Transfamic acid
obs before transfusion, 15 after start, 60 min after
why is irradiated blood components given
shortens shelf life by 2 weeks
kills any viable donor lymphocytes
can lead to GVHD
CMV neg blood who is it given to
pregnant women, IUD op, neonates
immediate TR haemolytic what is assoc with
symptoms
management 1-4
associated with naturally occurring IgM ABs of the ABO system, activation of complement cascade c3a and c5a, coagulation DIC, kinin system factor
early, can be asymptotic, increased temperature, chills, respiratory distress, decreased BP, pyrexia, rigors, tachy, tachypnoea, pallor, headache, pain, cyanosis
stop
assess patient
recheck compatibility and inspect pack
document
delayed haemolytic TR is what and when
symp
investigations
alloantibodies. 5-10d after
similar to acute. unexplained Hb as transfused RBC destroyed. jaundice. renal failure
anaemia. spehrocytes on film. increased bili and LDH. positive DAGT +/- RC alloantibodies
+/- degree of renal failure
febrile non haemolytic is what
symp
treatment
assoc with mild allergic
2% of RC transfusions 20% PLT
increased temp >38 or mild rash. increase in temp by 1-2 degrees
check compatibility, slow transfusion, close monitoring, paracetamol/piriton
urticarial/severe TR
was cells - IgE
rash wheals within mins
slow/stop depending
IV fluids. IM adrenaline 500mcg. IV chloramhenime 10mg. IV/PO steroids - take time to work
circulatory load in transfusions
pulmonary oedema. RF elderly, CCF patients
bacterial infections
fever, immediate collapse, DIC, shock, chills, vom, decreased BP, achy
RC: pseudomonas
PLT: staph, strep, salmonella
what will the blood bank send you
4x RC 4x FFP 1x PLT
haemolysis extravascular
intravascular
released of protoporphyrins, unconjugated bilirubin - jaundice, gallstones, urobiliuria
haemoglobinaemia, methalmalbuninaemia, haemoglobinuria, haemesideruria