HAEM 2 Flashcards

1
Q
primary iron overload is what 
pathology 
symptoms 
in who 
genetics
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

ix for haemachromatosis
family screening
treatment

A

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%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

secondary cause of iron overload
how much iron is in a transfusion
symptoms
treatment

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

anaemia of chronic disease why

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

acute blood loss steps
what can be given to reduce blood loss in surgeries
observations for transfusion

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

why is irradiated blood components given

A

shortens shelf life by 2 weeks
kills any viable donor lymphocytes
can lead to GVHD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

CMV neg blood who is it given to

A

pregnant women, IUD op, neonates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

immediate TR haemolytic what is assoc with
symptoms
management 1-4

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

delayed haemolytic TR is what and when
symp
investigations

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

febrile non haemolytic is what
symp
treatment

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

urticarial/severe TR

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

circulatory load in transfusions

A

pulmonary oedema. RF elderly, CCF patients

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

bacterial infections

A

fever, immediate collapse, DIC, shock, chills, vom, decreased BP, achy
RC: pseudomonas
PLT: staph, strep, salmonella

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what will the blood bank send you

A

4x RC 4x FFP 1x PLT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

haemolysis extravascular

intravascular

A

released of protoporphyrins, unconjugated bilirubin - jaundice, gallstones, urobiliuria

haemoglobinaemia, methalmalbuninaemia, haemoglobinuria, haemesideruria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

ix for haemolysis

film

A
FBC. film. reticulocyte count. unconjugated bili. serum hepatoglobins. urinary uroglobins. 
membrane damage - spherocytes
mechanical damage - RC fragments
oxidative stress - Heinz bodies
HbS - sickle cell
17
Q

cause of haemolysis

A

ABO incompatibility, transfusion, sickle disease, G6PD defic, black water fever, PNH, liver disease, vit e defic

18
Q

mechanical RC destruction

A

DIC, haemolytic uraemia syndrome E coli 0157, leaking heart valve (MAHA), TTP infection - malaria, burns - microspheres

19
Q

pre destruction of RC

A

AI haemolysis, alloimmune haemolysis, mechanical RC destruction

20
Q

abnormal cell membrane

A

liver disease (zieves), via E defic, PNH, hereditary spheocytosis

21
Q

abnormal cell metabolism

abnormal Hb

A

G6PD defic

sickle cell

22
Q

warm IgG extravascular occurs when

cause

A

body temp

idiopathic, SLE, CLL, penicillins, infection

23
Q

cold IgM intravascular occurs when

cause

A

at 4 degrees

idiopathic, EBV, mycoplasma, CLL

24
Q

alloimmune haemolysis immune responsive cause

passive transfer

A

haemolytic TR, immediate IgM, delayed IgG extravasation

haemolytic disease of newborn, IgG

25
Q

myeloma is what
symptoms
ix

A

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

26
Q

criteria major for myeloma

minor

A

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

27
Q

treatment for myeloma
prevent bone disease
symptom control
what vaccinations

A

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

28
Q
MGUS symptoms 
ix
no evidence of what 
in who 
management
A

asymp

paraprotein <30, monoclonal protein in blood, bone marrow plasma cells <10%

no evidence of myeloma and organ damage

14% in >90s

follow up

29
Q
AL amyloidosis is what 
genetic 
prognosis 
symptoms 
ix
A

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

30
Q
walderstorms is what 
clonal disease of what 
secretion of what 
IgM is what 
symptoms 
treatment
A

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