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
ix for haemolysis
film
FBC. film. reticulocyte count. unconjugated bili. serum hepatoglobins. urinary uroglobins. membrane damage - spherocytes mechanical damage - RC fragments oxidative stress - Heinz bodies HbS - sickle cell
cause of haemolysis
ABO incompatibility, transfusion, sickle disease, G6PD defic, black water fever, PNH, liver disease, vit e defic
mechanical RC destruction
DIC, haemolytic uraemia syndrome E coli 0157, leaking heart valve (MAHA), TTP infection - malaria, burns - microspheres
pre destruction of RC
AI haemolysis, alloimmune haemolysis, mechanical RC destruction
abnormal cell membrane
liver disease (zieves), via E defic, PNH, hereditary spheocytosis
abnormal cell metabolism
abnormal Hb
G6PD defic
sickle cell
warm IgG extravascular occurs when
cause
body temp
idiopathic, SLE, CLL, penicillins, infection
cold IgM intravascular occurs when
cause
at 4 degrees
idiopathic, EBV, mycoplasma, CLL
alloimmune haemolysis immune responsive cause
passive transfer
haemolytic TR, immediate IgM, delayed IgG extravasation
haemolytic disease of newborn, IgG
myeloma is what
symptoms
ix
plasma cell malignancy
bone lesions, hypercalcaemia, marrow failure, renal failure, immune depression, hyper viscosity, amyloid, RF 30% have cast nephropathy. sepsis, hyperuricaemia, headaches, anaemia, bleeding, purpura, pallor
FBC. film. BM biopsy. protein electrophoresis, serum immunofixation, urine - BJ proteins, whole body MRI
use paraproteins to monitor response
criteria major for myeloma
minor
plasmacytomas on tissue biopsy, bone marrow plasmycytosis >30% plasma cells, increased M protein in blood/urine
10-30% plasma cells in bone marrow sample, minor increase in M proteins in blood/urine, osteolytic lesions, low levels of ABs not produced by cancer cells in blood
treatment for myeloma
prevent bone disease
symptom control
what vaccinations
thalidomide, bortezomib and cyclophosphamide and dexamatheasone. ixazomib and lenlinide and dexam
zoldronic acid
opiate, analgesia (avoid NSAIDs), local radio, bisphosphonates, vertebroplasty
seasonal flu vaccine
pneumococcal vaccine
MGUS symptoms ix no evidence of what in who management
asymp
paraprotein <30, monoclonal protein in blood, bone marrow plasma cells <10%
no evidence of myeloma and organ damage
14% in >90s
follow up
AL amyloidosis is what genetic prognosis symptoms ix
rare. small cell plasma clone
mutation in light chain - alt structure
poor prognosis if heart failure
kidneys nephrotic, neuropathy, malabsorption, CM, liver dx
congo red stain green birefringence. rectal or fat biopsy
walderstorms is what clonal disease of what secretion of what IgM is what symptoms treatment
macroglobinaemia IgM protein. old men. lymphoplasmacytoid neoplasm
clonal disease of cells intermediate between lymphocytes and plasma cells
secretion of IgM
pentameric
LD splenomegaly, marrow failure, hyper viscosity: fatigue, visual disturbance, confusion, bleeding. neuropathy. night sweats. weight loss. fatigue
chemo. plasmapheresis
