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Medicine > Haematology > Flashcards

Haematology Flashcards

1
Q

whats anaemia

A

low Hb

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2
Q

casues of microcytic aneima

A

TAILS
thalassemia
anaemia of chronic disease
iron deficiency
lead poisoning
sideroblastic anemia

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3
Q

causes of normocytic anaemia

A

3A, 2H
anemia of chronic disease
acute blood loss
aplastic anemia
hypothyroidism
haemolytic anemia

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4
Q

casues of macrocytic anaemi

A

megabloblastic anemaia - result of impairede DNA synthesis preventing cells from dividing normally:
B12 deficiency
folate deficiency

normoblastic macrocytic aneami:
alcohol
liver disease
hypothyroidism
reticulocytosis
drugs- azathioprine

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5
Q

symtpoms anameia

A

sob
dizzy
headaches
palpitations
tiredness
worsening of other conditions- angina, periperal vascualr disease, heart failure

pica- odd cravings- iron deficicnecy
hair loss- iron deficiency

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6
Q

signs of anemia

A

pale skin
conjunctival pallor
tachycardia
high rr

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7
Q

koilonychia

A

iron deficiency anaemia

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8
Q

anglular chelitis

A

iorn defciency anaemia

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9
Q

atrophic glossitis

A

iron deficinecyanamie=- smooth tongue

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10
Q

brittle hair and nails

A

iron deficiency anemia

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11
Q

jaundice in what casue anemia

A

haemolyitc anaemia

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12
Q

bone deformities can be seen in what cuase of anemia

A

thalassaemia

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13
Q

oedema, ht, excoritations on skin what cause of anemia

A

CKD

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14
Q

investigfations inital for anemia

A

Hb
Folate
B12
ferritin
blood film
MCV

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15
Q

further investigation for anemia

A

OGD, colonoscopy for unexplained iron deficiency aneia - 2ww
bone marrow biopsy in unclear casue

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16
Q

where and how is iron absrobed - whats needed

A

absorbed in jejunum and duodenum
need stomach acid to to keep iron in its soluble form Fe2+
low stomahc acid meas iron changes to insoluble form Fe3+- ferric

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17
Q

what medications can affect iron absorbtio n

A

meds that reduce stomach acid- PPI
need acid to keep iron in soluble form

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18
Q

causes of iron deficiency anaemia

A

poor iron absrobtion - coeliac, crohns
blood loss- cancer gi tract, oeseophagitits, gastirits
dietry insufficency- most common in children
increase requirements during pregnacy

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19
Q

casues of blood loss casuing iron deficiency anemia

A

most common cause- gastirtits and oesophagitis in adults
gi tract cancer
mentrauting wmen- esp menorhagia - heavy periods
IBD

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20
Q

what form is iron travelling in blood

A

Fe3+ ferric irons bind to trasnferrin

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21
Q

whats total iron binding capacity

A

total space oon transferring moleucle for iron to bind

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22
Q

trasnferrin saturation

A

good indicaiton of how much iron in body
propportion of transferring moleclres bound to iron- directly related to amount of trasnferrin in blood

trasnferrin saturation = serum iron/ TIBC

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23
Q

ferritin is what
what affect levels

A

ferritin is form iron is stored in cells

inflammation (infection/cancer) can casue ferritin to be release fro cells
- if high doesnt mean iron overload could just be due to inflammation
can still be iron deficinet if high ferritin levles as check to see if they have an infection

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24
Q

serum iron
how useful

A

varies throughout day
higher in morning and after iron meal

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25
Q

iron deficiency anemia bloods

Hb
MCV
serum ferritin
serum iron
TIBC
trasnferrin saturation
trasnferrin

A

low Hb
small MCV
low serum ferritin
low serum iron
high total iron binding capacity (lots spaces free cus not much iron binding)
low trasnferrin saturation
high transferrin (trying to produce it to make sure picing up every bit of iron possible)

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26
Q

managment of iron deficiency

A

if new iron def and adult without clear casue - OGD and co,onoscopy

treat underlying casue and correct anemia:
blood transfusion= immediately corercts anemia (low Hb) but doesnt correct the iron def

iron infucsion= immedialty corrects iron def
avoid in spesis as iron feeds bacteria
samll risk anaphylaxis

oral iron = slowly corrects iron defi = but unsuitable if malabsirbtion is cause
ferrous sulphate
se= constipation, black stool
expect rise of 10 g/l per week of Hb

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27
Q

casues of B12 deficiency

A

insuficent dietry intake
gastric malabsrobtion:
total/sub gastrectomy
pernicious anemia
congential IF deficnecy

intstinal malabsrobtion:
ileal resection
crohs of ileum
congeital b12 malabsibrtion
bacterial colonisation of Small intestine
stagnant bowel loop syndrome

long term use of drugs that decrease gasric acid- gastric acid needed to relase b12 bound to protiens in food

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28
Q

pathophysiology of pernicious anemia

A

autoimmune condition
casue of b12 deficiency
paritetal cells of stomach produce intrinsic factor (protein)
IF essential for absrobtion of B12 in the ileum
antibodies form agaisnt IF/ parietal cells - lack IF- prevents absrobtion b12- deficient

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29
Q

signs and sympotms b12 deficiency
(same as sign and symptoms of pernicious anemia)

A

neuro!!!
:
visual changes
mood / cognitive changes- depression, dementia, confusion, delerium
peripheral neuropathy with numbness/ paraesthesia
loss of vibration sense/ proprioception

fatigue
pallor
lemon tinge
faintness
headaches
sob
palpitations
glossitis
ulceration
macrocytic megaloblastic - but could be normocytic if also got iron deficiency

may have other sings of other uatoimmune condutions - T1DM, hypoaldosteronism- addisons-, hashimotits, vitiligo

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30
Q

differentials for
visual changes
mood / cognitive changes- depression, dementia, confusion, delerium
peripheral neuropathy with numbness/ paraesthesia
loss of vibration sense/ proprioception

fatigue
pallor
lemon tinge
faintness
headaches
sob
palpitations
glossitis
ulceration

A

B12 deficiency
folate deficiency- dont have the neruo symtpoms
neuro complication- subacute combined degenration of spinal cord may occur despite normal b12

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31
Q

investigfations for pernicious anemia

A

intrinsic factor antibodies- first line
may have icnreased MCV- can be normal if also got iron deficiency = dimorphic blood film
gastric parietal cell antibodies - less helpful
oval macrocytes, hypersegemtned neutrophils and circulating macroblasts on blood film
may have other autoimmune conditiosn- T1DM, hypoaldosteronism- addisons- vitiligo, hashimotis
B12

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32
Q

What autoimmune conditions can be seen in patients with pernicious anemia

A

hashimotos
t1dm
adidsions- hypoaldosteronism
vitiligo

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33
Q

manamgent of b12 deficiny

A

can give oral hydroxocobalmin (cyanocobalmin used to be) if the cause is low in diet

pernicious anemia:
IM injection of hydroxycobalamin 1mg 3 times a week for 2 weeks then 1mg every 3 months

if neruo symptoms:
IM injection of hydroxycobalamin 1mg alternate days until symtpoms improve then every 2 months

if folate is also low TREAT B12 DEFICIENCY FIRST
if treat folic acid def when got b12 deficiency too this can lead to subacite combined degenration of spinal cord- parasesthesia, vison changes, loss vibration/propriocpetion etc and can eventually lead to paraplegia

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34
Q

treat pernicious anemia pt with neuro symtpoms

A

1mg IM hydroxycobalamin alternate days until symtpoms improve and no further imporvemnt then every 2 months 1mg IM

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35
Q

patient has b12 deficinecy and folate deficnecy
how manage

A

treat b12 deficinecy first as if treat folice acid and got b12 deficiency can casue subacute combined degenration of spinal cord

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36
Q

blood cells develop whre

A

bone marrow

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37
Q

what are pluripotent haemoatopoietic stemm cells

A

undifferentiaed cells with potential to trasnapform into variety blood cells

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38
Q

pluripotent haemoatopoietic stem cells become what

A

myeloid stem cells
or
lymphoid stem cells
or
dentritic cells via stages

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39
Q

stages of cells of red blood cells

A

pluripotent haemoatopoietic stemm cells
–>
myeloid stem cells
–>
reticulocytes- immature rbc
–>
rbc - survive 3 months

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40
Q

platelts are made form what

A

megakaryocytes
survive 10 days

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41
Q

white blood cells what stages of cells come frm

A

pluripotent haemoatopoietic stemm cells
–>
myeloid stem cells
–>
promyelocytes
–>
mono cytes become macrophages
neutrophils
eiosinophils
mast cells
basophils

for b and t cells= lymphocytes diff route:
pluripotent haemoatopoietic stemm cells
–>
lymphoid stem cells
–>
b cells or t cells
–>
plasma cells and memory cells or CD4/CD8/killer cells

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42
Q

where do t cells mature

A

thymus

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43
Q

where do b cells mature

A

bone marrow

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44
Q

b and t cells made from what

A

lymphoid stem cells

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45
Q

other wbc not lymohoctes made form

A

myeloid stem cells

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46
Q

anisocytosis on blood film

A

variation size rbc
myelodysplasic syndrome
some forms anaemia

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47
Q

target cells on blood film

A

iron deficincy
posy splenectomy

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48
Q

heinz bodies on blood film

A

dna left in- spleen normallly removes
post splenectomy
severe anemia- where rbc generated v quickly

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49
Q

reticulocytes on blood film

A

increase when have rapid turnover of rbc
hameolytic anemi
= represent the bone marrow is active in replacing lost cells

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50
Q

schistocytes

A

fragments of rbc
haemolytic anemia
HUS
DIC- disseminated intravascualr coagulation
thrombotic thrombocyti[enic purpura
metallic heart valves

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51
Q

sideroblasts

A

immature rbc with blob iron in
bone marri unable to incorporate iron into the hb
myelodysplastic syndorme

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52
Q

smudge cells

A

ruptured wbc
chornic lymphocytic leukaemia

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53
Q

spherocytes

A

autoimmune haeolytic anemia
hereditary spherocytosis

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54
Q

whats serum

A

clotting facotrs removed
glucose in
electroyltes in
protiens in- hormones and immunoglobulins

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55
Q

inherited cuases of haemolytic anaemia

A

hereditary spherocytosis
hereditary elliptocystosis
thalassaemia
sickle cell anemia
GP6D deficiency

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56
Q

acquuired cuase sof haemolytic anemia

A

alloimmmune hameolytic anaemia
autoimmune haemoltic anemia - trasnfusion rxn/haemoltic in newborn
paroxysmal nocturna; haemaglobinuria
microangiopathic haemolytic anemia
prostehtic heart valve related hameolysis

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57
Q

features of haemolytic anemia

A

jaundice
anemia
splenomegaly- filled wth destoryed rbc

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58
Q

investigations for hameolyic anemia

A

blood film- schistocytes
FBC- normocytic anemia
direct coombes test- postive in autoimmune haemolytic anemia

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59
Q

jaundice
splenomegaly
gallstones
anamie - normocytic

A

hereditary spherocytosis
hereditary elliptocytosis
GP6D deficincy

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60
Q

herediary spherocytosis
s and s
dx
rx

A

most common inhertied haemolytic anaemia
autosomal dominant
sphere shaped rbc - fragile

s and s
jaundice
gallstones
anamei a
splenomegaly
asplastic crisis in prescence of the parovirus

dx
hx and clinical
spherocytes on blood film
rasied MCHC
Raised reticulocytes due to rapid turnover

rx
folate +splenectomy
+/ cholesectomy if gallstones issue

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61
Q

hereditary elliptocytosis

A

similar to sphereocytosis
anaemia
autosomal dominant
ellipse shaped rbc - fragile

s and s
jaundice
gallstones
anamei a
splenomegaly

dx
hx and clinical
eelliptocytes on blood film
Raised reticulocytes due to rapid turnover

rx
folate +splenectomy
+/ cholesectomy if gallstones issue

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62
Q

GP6D deficiency
what it is
s and s
dx
triggers

A

defect in GP6D enxyme in rbc
medditaranean and african pts seen more
x linked recessive
crises thats triggered by infection/medication/ fava beans- broad beans

s and s
jaunice
splenomegaly
gallstones
anemia
heinzbodies on blood film

dx
GP6D enzyme assay

triggers:
meds- quinine- antimalarial, ciprofloxacin
sulphylureas,
sufalazine
sulphonamide drugs
flava beans
infection

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63
Q

pt jaundie
becomes anemia

this is after eating broad beans/ developing an infection/ treated with anitmalariasl

A

GP6D deficiency

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64
Q

autoimmune haemoltic anemia

A

anitbodies agasint pts rbc
warm types = most common
haemolysis at normal/above normal tem
idiopathic

cold type:
cold aggulutin disease
lower temp antibodies agasint rbc attacht and clump= agglutination
this casues destruction by immunesystem of the clumps
casues of cold:
seocndary to other condition:
lymphoma
leukaeia
SLE
EBV
HIV
CMV
mycoplasmsa

treat:
blood trasnfusions
prednisolone
rituximab- monocolonal ab agasitn b cells
splenectomy

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65
Q

infections/ conditions that can casue autoimmune haremolytic anameia cold type

A

leukaemia
lymphoma
SLE
EBV
HIV
CMV
mycoplasma

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66
Q

paroxysmal nocturnal haemolglobinura

A

rare
genetic mutation aquired in life
mutation agasint haematpoteitc stem cells in bone marriw
get loss of protiens on rbc surdace that inhibits the complement system= activation of complement cascade on sruface of rbc = estruction rbc

red urine in morning with hb and haemosiderin in
anemia
predisposed to thrombus- dvt, pe, hepatic vein thrombosis
smooth muscle dystonia - oesopahgeal dysmotility, Erectile dysfucntion

treat
eculizumab- monocolonal antibody agasunt compleent
or
bone marriw trasnplant- cure

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67
Q

alloimmune haemolytic anemia

A

due to eother
foreign rbc circulating causises an immune rxn= transfusion tcn - antibodies form agaisnt it

or
forgin anttibody ciruclating that acts agasint the rbc- antibody from mum through placmneta = haemolytic disease of new born

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68
Q

anemia after blood trasnfusion

A

alloimmune haemolytic anemia

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69
Q

microangiopathic haemolytics anemia

A

small bv structure abnomalitiy casuing hamolysis of blood as it goes through
usualy secondary to underlying ocndition:
HUS
DIC
TTP
SLE
cancer

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70
Q

red urine in morning
hard to swallow
dvt

A

paraoxysal nocturnal haemolgobinuria
loos protien on surface rbc that inhibts complement cascade so have complement cascade on surface of rbc so get destruction rbc and thrombus

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71
Q

thalassaemia

A

genetic defect of the protein chains that make up hb
2 alpha and 2 beta
autosomal reccessive
varies on type and mutation
rbc more fragile and break down easier

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72
Q

presentation thalassaemia

A

microcytic anemia
fatigue
jaundice
pallor
gallstones
pronounced forehead and malar eminences and suseptible to fractures= the bone marrow expands to produce extra rbc to compensate for chornic anemaia
splenomegaly= spleen colelcts all thr destoryed cells
can get iron overload

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73
Q

ivestgations for thalassamei

A

FBC- microcytic anemia
Hb electrophoreisis
DNA testing - genetic abnormality

regant women are offered screen test at boooking

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74
Q

what can happen to patients iron levels in thalassaemi

A

can ge tiron overload
due to creation of faulty rbc, recurrent transufsions and increased absorbtio of iron in response to the anamei

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75
Q

how do you manage iron overload in pt with thallasmeia

A

mornitr serum ferritin
limit trasnufsions and give iron chelation

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76
Q

iron overload in thalassaemia can casue what

A

similar to haemochormatosis symtmptons
liver cirrhosis
fatigue
infertility and impotence
heart fialure
diabetes
arthritits
OA and joint pain

77
Q

types of thalassaemia

A

alpha thalassaemia- defect in alpha chain - gene coding on chromosome 16
beta thalassaemia- defect in beta chain - gene coding on chromosome 11

three types of beta thallasameia
thalassaemia minor = 1 abnormal gene and 1 normal gene
thalassaemia intermedia = 2 abnomal generes: either 2 defective genes or 1 defective gene and 1 deletion gene
thalassaemia major = homozygous deletiion genes

78
Q

alpha thalassaemia
what it is and rx

A

defect in alpha globin chain
chromosome 16 - gene that codes this protien
Rx=
monitor FBC
monitor for complcations
blood transfusion
splenctomy maybe
bone marrow transplant can be curative

79
Q

beta thalassaemia minor
what it is and rx

A

beta chain abnrmaility
chromosome 11
one abnormal gene and one normal gene
mild micorcytic anameia
monitor only = carrier

80
Q

beta thalassaemia intermedia

A

beta chain abnomality
chromosome 11
2 abnormal copies of beta globin chain:
one defective and one deletion gene
or
2 defective genes - still have some fucntion in defective one

have more significant microcytic anemia than minor
monitor and occasional blood trasnfusions
if need more transufiosn then consider iron chelation tp prevent iron overload

81
Q

beta thalassaemia major
what i t s
causes
rx

A

beta chain defect
chromosome 11
homozygous for deletion genes
= no fucntioning beta globin genes

causes:
severe microcytic anemia
failure to thrive often in early childhood
splenomegaly
bone deformities

rx:
regular blood trasnfusions + iron chelation + splenectomy
bone marrow transplant can be curative

82
Q

sickle cell anemia

A

genetic condition causing sickle shaped rbc - crecent
fragile and easily destroyed rbc => haemolytic anemia
autosomal recceive
abnomral gene for beta globin on chr 11
sickle cell disease- two abnormal copes

83
Q

sickle cell trait

A

one abnormal gene = reduces severity of malaria = sickle cell disease seen more in areas of africa, carribean, mid east and india
usually asymptomatic

84
Q

diangosis of sickcle cell disease

A

pregnacy if at risk of being carrier are offered screening
at heel prick screening at 5 days old

85
Q

complcaitions of sickle cells disease

A

aniemai
increase risk of infection
aplastic anemia
stroke
avascualr necrosis of large joints- hip
pulmoanry ht
priapism = painful and persitant penile erection
CKD
sickecell crisis
acute chest syndrome

86
Q

general mamangment of sicke cell disease

A

avoid dehydration and other triggers of crisis
vaccine sup to date
prophylactic abx= peniccilin V= phenoxymethypenicillin
hydroxycarbamid = stimulates fetal Hb - which doesnt lead to sickeling andis protective agasitn sickle cell crisis and acute chest syndrome
blood trasnfusision for severe anameia
bone marrow trasnplant can be curative

87
Q

whats aplastic crisis
what it is
what casues iit
rx

A

can occur in sickle cell disease
can also occur in herediatry spherocytosis

tmeporary loss of creation of new blood cells
trigger is commonly paravirus B19

get significant anemai

rx= supportive with blood transfusions and usuallt resovles spontaneously within a week

88
Q

sickle cell crisis

A

umbrella term for spectrum of acute crises related to condition
can be mild to life threatening

89
Q

casues of sickle cell crisis and managment

A

casues: can be spontaenous
can be triggered:
infeciton, dehydration, cold, significant life event

rx:
supportive
admit to hosp with low suspcion
treat any ifnection
keep warm
well hydrated- iv fluids
simple analgesia- avoid nsaids if renal impairemnt
penile aspiration if priapism

90
Q

vaso oclusive crisis

A

also called painful risis

sicke shaped rbc get stuck and clogg the cappialiress=> distal sichemia

associated with dehydration and high haematocrit

91
Q

symtpms of vaso oclusive crisis

A

pain
fever
symtpoms of the infection that triggered it
can cause priapism in penis by trapping blood in penis= painful and persitant erection = treat by apsirating blood from penis
- in sickle cell anemia

92
Q

splenic sequestrian crisis

A

rbc blocking flow in spleen - due tp sickle cell anameina
acutly enlarged and painful spleen
get pooling of blood in the spleen so get severe anemia and circulatory collaspe- hypovolamiec shock

93
Q

treat splenic sequestrian crisis

A

emergency
supportw with blood transfusions and fluids
if have recurence then splenectomy can prevent
if get recurrence of crisis this can lead to spleninc infarction and thereform more suseptibel to infection

94
Q

acute chest symdrome

A

in sickle cell aniemai
have fever / resp symotkmas WITH new infilatrates in CXR

95
Q

casues of acute chest syndrome

A

sickle cell anemia and then either:
infection= pnumonia/ broncholitis
or non infective:
pulmonary vaso oclusion/ fat emboli

96
Q

treamtent of acute chest syndrome

A

med emergency
supportive and treat underlying cause
antibiotics/antivirals if infection
blood transfusion to trat the anamei a
incetive spirometry
artifical ventilaiton may be needed

97
Q

what antibiotics use forn prophylaxis of infection in sickle cell anemia

A

penicilllin V= phenoxymethypenicllin

98
Q

lymphoma

A

group of cancers tat affect the lymphocytes (B and T cells) inside the lymphatic system

canceorus cells prolgierate within the lymph nodes and casue lymphadenopahty

99
Q

hodgkins lymphoma risk facotrs

A

HIV
EBV
fam history
autoimmue conditions- RA, sarcoidosis

1 in 5 lymohomas are hodgkins
bimodal age distribution 20 s and 75

100
Q

presentation of all lymphomas

A

LYMPHADENOPATHY !
neck/ axilla/ inguinal
non tender, rubbery feel
can et pain in node affected when drink alcohol

B symtpms = systemic symtpoms of lymohona
fever
weight loss
night sweats

other:
itching
sob
cough
abdo pain
recurrent infections
fatigue

101
Q

investigaions for lymphomas

A

LDH= raised in hodgkins- can be rasied in other cancers and non caneorus disease
biopsy= key diangostic- often only way differentiate between non and hodgkins => reed sterberg cell in hodkings lymphoma
CT,MRI PET= staging and diagnosis

102
Q

key diangosit for lymphoma

A

biospy

103
Q

reed sternberg cell foun on biopsy

A

hodgkins lymphpma

104
Q

what use for staaging of lymphpmas

A

for non and hodkins use ann arbor staging= whetehr nodes affected are above +/ below diaphragm

1= one region
2- more than one region bu same side diaphgram
3= affected nodes above and below diagphram
4- widespread with non lyphatic organ involvement ef. liver/ lungs

105
Q

types on non hodgkins lyphoma

A

burkitt lymphoma
MALT lymphoma
diffuse large B cell lymphoma

106
Q

burkitt lymphoma is assocaited with what

A

HIV
EBV
malaria

107
Q

MALT lymphona is what, where usually and assicated with what

A

mucosa assocaited lymphoid tissue
usally around the stomach
associated with H.pylori

108
Q

diffuse large B cell lymohpma presents as what

A

rapidly growing painless mass in pts over 65

109
Q

rapidly growing painless mass in pts over 65

A

diffuse large b cell lymphoma

110
Q

h.pylori infection
enlarge lymphnoeds

A

MALT lymphoma

111
Q

risk factors for non hodgkins lymphoma

A

HIV
EBV
H pylori- malt lymphoma
hep B/C
exporusre to pesticies and trichloroethylene (used in industrial process)

112
Q

treatment for hodkins lymphoma

A

chemo - risk of leukaemia and infertility
radiotherapy- risk of cancer, damage tissies, hypothryodisim
aim is to cure but can get relaspe ot other heametological cancer

113
Q

treamtnent for non hodgkins lymphoma

A

depends on type
watch and wait
radioatheyrpy
chemo
monocolonal antibodies eg rituximab
stem cell trasnplantation

114
Q

leukaemia

A

cancer og a particular of stem cells in the bone marrow
unregulated production of certain types of blood cells

115
Q

patholphisioly leaukamei

A

cacner of cells in bone marrow
genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal wbc==> due to this get supression of other cell lines (focus on this one) and so casues underproduction of other cell types= pancytopenia=
dec rbc =anemia
dev wbc= leukopenaia
dec platelets= thrombocytopenia

116
Q

types of leukamiae

A

acute myeloid leukameia
acute lymphoblastic leukaemia
chronic myelroid leukamia
chronic lymphocytic leukameia

are others

117
Q

ages get the diff leuakmiea

A

ALL CeLLmates have CoMmon AMbitions
acute lymphoblastic leukaemia = under 5 and over 45
over 55= chronic lymphocytic leaukaemia
over 65= chronic myeloid leukaemia
over 75= acute myeloid leukaemia

118
Q

presentation of leukamiae

A

fatigue
serious infections- abnomalabc dont work agaisnt infections
abnomral bleeding
PETECHIAE and abnormal brusing
easy bruising, bleeding from gums= thrombocytopenia
pallor- anemai
failure to thirve in children
fever
weight loss
lymphadenopathy and hepatosplenomegaly due to wbc buidling up in them

119
Q

differentials of petechiae

A

non blacnhing rash - asmall dots
leuakaemia
menigiococcal septicaemia
vascultits
henoch - schonlein purpura
idiop[athic thrombocytopenia purpura
non accidental injury -ABUSE- dont forget

120
Q

diagnosis for leukaemia tests

A

FBC- intial
within 48hrs if suspect leukamia
go hosp imeidate if child/young adult with petechiae/hepatosplenomegaly
blood film= abnormal cells and inclusions
rasied LDH
bone marrow biopsy- definitive = aspiration/ trephine - solid core- takes longer/ biopsy from iliac crest
CXR- infection/ mediastinal lymphadenopathy
lumbar puncture if suspect cns involemet
CT/MRI/PET staging and asses for lymphoma or other tumours

121
Q

Von Willebrand disease

A

deficiency/defective/abscnee of von willebrand facotr- glycoprotein used in clotting

most common inherited casue of abnormal bleeding- haemophilia
many diff genetic casues - most autosomal dominant
type 3 most sever

122
Q

presentation von willebrand disease

A

unusually easy bleeding
prolonged/ heavy bleeding :

bleeding of gum when brusing teeth
menorhagia- heavy periods
nose bleeds- epistaxis
heavy bleeding during surgery
fam hx of heavy bleeding/ vw disease

123
Q

pt has lots nose bleeds and bleeding of gums
ask about periods and they have v heavy periods too
fam hx of ehavy bleeding

A

vON wILLEBRAND DISEASE
- most common inherited casue pf abnormal bleeding- haemophilia

124
Q

diagnosis for VW disease

A

clinical- hx, fam hx
bleeding assesment tools
lab stuff

125
Q

managemnt of patient with von willerand disease

A

non on day to day

treat either inrepsonse to major bleed/tauma - stopping bleed
or treat to prep for operations to prevent bleeding:
desmopressin = stimulate release of vwf
VWF infused
factor VIII infused with plasma derived VWF

if having heavy periods:
tranexamic acid- antifibrinolytic agent
mefanamic acid
norethisterone
COCP
mirena coil
hysterectomy if severe

126
Q

myeloma

A

cancer of plasma cells - B cells that produc eantibodies

cancer in a specific plasma cell reuslts in large quantities of a single type of antibody produced
multiple myleoma= myeloma affectcs multiple areas of body

127
Q

monocoloncal gammopathy of undetermined signifiance- MGUS

A

excess of a single type of antibody/comppent of antibody without the other features of myeloma/cancer
may progress to myeloma so monitor it

128
Q

smouldering myeloma

A

progression of MGUS
with higher levels of antibody/componenets = premalignant

eg. waldenstroms macroglobulinaemia = type of excessive IgM

129
Q

most common Immunoglobulin (antibody) thats abundant i myeloma

A

most common one that is produced lots is IgG

130
Q

paraprotein

A

nonocolonal paraprotein= the single type of abnormal protein produced in myeloma

131
Q

features of myeloma

A

CRAB - most important features to remeber
Calcium- high
Renal failure
Anemia- normocytic, normochromic
Bone lesions and bone pain

pathologcial fracutures
leukopenia
thrombocytopenia
neutropenia

hypervisocitiy
myeloma renal disease
myeloma bone disease
anemia

therefore have:
due to hyper visocity:
easy brusiing and bleeding
reduces or loss of sight due to vascular disease in eye
purple discolouration extrmeities
heart. failure

due to anemia:
neutropenia
thrombocytopenia
leukopenia

132
Q

why does hypervisocity occur in myeloma

A

have increase proteins- immunoglobulins so increase viscoity of blood
this casues
easy brusing
easy bleeding
purple discolouration of extrmeitites
reduced or loss of sight due to vascualr disease
heart failure

133
Q

why do you get anemia, leukopenia, neutropenia and thrombocytopenia in myeloma

A

canceorus cells infiltrate bone marrow
causes supresion of development of other blood cells
:
anemia- reduce rbc
thrombocytopenia- reduced platelts
leukopenia- reduce wbc
neutropenia- reduce neutrophils

:
lethargy
pallor
infections
poor clotting so bruise lots and blled lots

134
Q

why d you get myeloma bone disease

A

have increasesd osteoclast acitivty and decreased osteoblast acitivty due to cytokine produced by the plasma cells and stromal cells that are in contact with the plasama clls

casues increase ca to be reabsorbed
occurs in skull, long bones, ribs, spine most
casues pain
get patchy areas so get osteolytic lesions and pathological fractures

135
Q

why do you get myeoma renal diseae

A

casues of renal failure:
the immunoglobulins block the flow in the tubules
the increase ca impairs renal functioning
dehydration
meds to treat eg. bisphosponates bad tp kidneys

136
Q

plasmacytomas in myeloma

A

they can deveop= tumours formed by cancerous plasma cells in the bones and replace normal tissue

137
Q

investigations for susepcted myloma

A

if got FBC- low WBC
or
high ca
or
high ESR
or
icnrease plasma viscocity then do inital investigfations of BLIP:
bence jones protein = urine elctrophoreiss
Serum free Light chain asssay
serum Immunoglobulins
Serum protein electropheresis

diagnositc:
bone marrow biopsy
also do imaging- whole body MRI - first line
whole body ct- seocond line
skeletal surveyr- whole body XRRAY- 3rd line

138
Q

initial investigfations myeloma

A

BLIP
bence jones protein = urine elctrophoreiss
Serum free Light chain asssay
serum Immunoglobulins
Serum protein electropheresis

139
Q

diagnostic investigations myloma

A

bone marrow biospy

iaging - whole body MRI

140
Q

what see on xray in pt with myeloma

A

ouched out lesions
lytic lesions
pepperpot skull- due to lots of punched out lesions scattered in skull

141
Q

managment of meyloma

A

control it
relapsing and remiitting
first lien:
chemo + bortezomid + dexamethasone +/ thalidomide

can do stemm cell trasnplant part of clinical trial
VTE prophlaxiss - aspirin or LMWH- chemo regimes on can increase risk clots eg. thalidomide

treat the bone disease:
bisphosphonates
radiotherpy of bone to reeduce bone [pain
surgery to stbalise/ treat freactures
cement augmentation

142
Q

complication of myeloma

A

anemia
hypercalcemia
hyperviscotiy
renal fialure
pain
infection
peripheral neuropahty
spinal cord compressio

143
Q

pt has spinal cord comrepssion
AKI/ HYPERCALCEMIA

A

admit to hosp immediatelt
myeloma complciations

144
Q

bence jone protein found in urine electrophoreisis

A

myleoma

145
Q

myelodysplastic syndrome

A

casued by the myleoid bone marrow cells not maturing properly- therefore not producing healthy blood cells

= low levels of blood components that originate from mlyeloid cell line:
anemia - low rbc
neutropenia - low neutrophils
thrombocytopenia - low platlets

risk of transforming into acute myeloid leukaemia

146
Q

risk facotrs for myelodysplastic syndorme

A

over 60s
had chemo/radiotherpay

147
Q

presentation of myelodysplastic syndrome

A

anemia- pallor, SOB, fatigue
thrombocytopenia - purpura/ bleeding
neutropenia- frequent/ severe infecitons

148
Q

diangosis of myelodysplastic syndrome

A

FBC abnormal - inital- low rbc, low platlekts, low neutrophils
blood film- blasts

diagnositc:
bone marrow aspiration and biospy

149
Q

managment of myelodysplasti syndrome

A

depends on symtpoms and risk of progression and prognosis:
watch and wait
blood transfusion if severe anemia
chemo
stem cell transplantation

150
Q

myeloproliferative disorders

A

type of bone marrow cancer
occurs due to uncontrolled proliferation of a single type of stem cell

151
Q

primary myelofibrosis
due to what stem cell proliferation

A

haematopoetic stem cell

152
Q

essentail thrombocythaemia
due to what stem cell proliferation

A

megakaryocyte

153
Q

polycythameia vera
due to what stem cell proliferation

A

eryhthroid cels- expansion of red cell mass

154
Q

what leuakemia can myeloproliferative disorders progress to

A

they cance rof bone marrow them selves
but can progress to acute myeloid leukaemia

155
Q

what myelofibrosis due to and what is it

A

due to priamry myelofibrosis or can be seconday due to essentail thrombocythaemia and polycythameia vera

prolfieration of the cell line leads to bone marrow fibrosis

bone marrow gets replaced with scar tissue and so cant produce blood cells
this is due to cytokines released form the prolfierating cell line eg. fibroblast growth facotr

leads to anemia and leukopenia

but the liver and spleen take over of haemotpoeisi= extramedullary haematopoesis leading to splenomegaly and hepatomegaly

if occurs around the spine can get spinal cord comrpessions

156
Q

presentation of myeloproliferative disorders

A

usually intially asymptomatc

systemic symtpoms:
weight loss
fever
fatigue
night sweats

can then have symptoms due to complctions:
anemia - in ET and primary myelofiboriss
splenomegaly = abdo pain (due to the spleen trying to make blood cells now)
portal hypertension- ascites, varices and abdo pain
low platelets- in polycythamiea vera and primary myelofibrosis = bleeding and petechiae
thrombosis = in polycythamia and thrombocytothemia
increase red blood cells in polycythamiea causing thombus and red face
decreased wbc= infections

157
Q

polycythameia vera signs and symtpoms

A

fatigue
pruitus
symptomatic splenomegaly
increase risk of thrombus formation
can progress to myelofibrosis and acute myeloid leukaemia

158
Q

what will be deranged in polycythamia vera in FBC

A

high Hb = over 185 in men and over 165 in women

159
Q

what will be deranged in essentail thrombocythaemia in FBC

A

rasied platelets = over 600 x10 9

160
Q

what will be deraged in meylofibrosis
on FBC

A

can be due to promary meylofibrosis or secondary to polycythameia vera and essential thrombocytothameia

varies:
aneima
leukocytosis/leukopenia ( if spleen and liver cant compensate then low)
thrombocytosis/ thrombocytpenia

161
Q

blood film show for myelofibrosis

A

tear drop rbc
aniscotysosis- vary sixe rbc
blasts- immature rbc and wbc

162
Q

FBC in myeloproliferative disordera

A

PV= rasied RBC
essential thrombocytothameia = rasied platelets - over 600
meylofibrosis- varies= anemia, leukpenia/ leukocytosis, thrombocytpenina/ thrombocytosis

163
Q

tear drop shaoed rbc

A

myelofibrosis

164
Q

what genes are associated with myeloproliferative disorders

A

JAK2
MPL
CALR

165
Q

JAK2
MPL
CALR

these genes associated with:

A

myeloprolgoerative disorders
JAK2 ca treat eith jak2 inhibtir- ruxoliitinib

166
Q

treatment for primary myelofibrosis

A

mild= monitor
allogenic stem cell trasnplantation - can cure but has risks
chemo- not currative on own
supoort for anemia, splenomegaly, portal ht

167
Q

treatment for polycythamia vera

A

first line= venesection - get Hb and hametocrit to normal
aspirin- redice risk of thrombus formation
chemo- control it

168
Q

treamemnt for essentail thrombocytothamia

A

aspirin- reduce risk of thrombus formation
chemo- control it

169
Q

haemophilia types and what is it

A

inheirted bleeding disorder
haemophilia A= deficinecy in facotr VIII
haemophilia B (christmas disease) = defieicnecy in factor IX

170
Q

haemophilia is what type of genetic condition

A

x linked receissve!!!
= mostly males affected

171
Q

presentation of haemophilia

A

bleeding excessively in repsonse to minor trauma
haemoarthrosis!! (bleeding into joint) and bleeding into muscles = if not treated can lead to joint damage and deformity
often presents in neonates- haemoatoma, intracranial haemorrhage and cord bleeding
risk of spontaenous haemorrhage without trauma!!

bleed in other places:
GI tract
urinary tract - haematuria
gums
retroperioneal space
intracrnaila
folowing procdures

172
Q

diangosis of haemophilia

A

bleeding scores
fam hx
coagualtion factor assay
genetic testing

173
Q

manamgemnt of hameophilai

A

IV infusion of affected clotting facotr = VIII , IX
either give as prophylaxis or in repsonse to bleeding

complication though is can form antibodies to the clotting facotr and then treatment becomes ineffecive

in repsonse to acute episode of bleeding or to prevent excessive bleeidnging in surgery:
desmopressin- stimulate VWF release
infusion of afected clotting facotr 8/9
antifibrinolytic= tranexamic acid
(v similar rx to vw disease)

174
Q

what drugs not to give pts with haemophilia

A

avoid nsaids
avoid anticoagulants

175
Q

casues of thrombocytopenia

A

problems with production:
myelodysplastic syndrome
sepsis
B12 / folic acid deficiency
leukaemia
liver failure= rediced production of thrombopietin from liver

problems with destruction:
heparin induced thrombocytopenia
immune thromboctypenic purpura
thrombolytic thrombocytopenic purpura
alchol
haemolytic uraemic syndrome

176
Q

presentation thrombocytopenia

A

low plaetels
mild = non
under 50 x10 9:
easy / spontaneous bruising
prolonged bleeding time
nose bleeds
heavy periods
easy brusing
blood in urine/ stool

under 10 x10 9:
inc risk spontaenous bleeding
intracranial haemorrhage and gi bleed concerning features

177
Q

differntials of abnormal / prolonged bleeding

A

too high anti coag meds
thrombocytopenia
haemophilia A and B
von willebrand disease
disseminated intravascualr coagulation

178
Q

whats immune thrombocytopenia purpura

A

also called idiopathic/ autoimmune/ primary …
antibodies are created agasint the plateltes leading to destruction of platelets

179
Q

treat immune thromboctyopenic purpura

A

prednisolone
IV immungloublins
rituximab- monocolonal antibody agasint b cells
spleectomy

monitor platlet count
educate pt on prolonged headachses/ meleane then seek help
control bp
supress periods

180
Q

thrombotic thrombocytopenic pupura

A

tiny clots form in small vessles that use up the platleetes = dec platets ad brusing under ksin

due to defeciny/abnormal ADATS13 proteitn = protein inactivcates VWF and decreases platelt adhesion to vessel walls and clot formation

got reduced funciton protein and so then have ovreactivity of VWF and formation of clots

the tiny blood clots break up rbc and so also get haemolytic anemia

casues: autoimmune = antibodies created against the protien ADATS13
or
genetic mutation in ADAT13

treatment:
plasma exhange
steroids
rituximab

181
Q

protien thats deficient/ antobodies agasitn it in thrombotic thrombocytopenic purpura

A

ADATS13

182
Q

heparin induce thrombocytopenia

A

pt on heparin but then gets clot

antibodies agasint platelts are prodiced in repsonse to exposure of heparin

antibody targets protien on the surface of plactlets PF4= anti PF4/heparin antibodies

bind to the platelets and activate the clotting mechansim = casues the platelts to get destroyed but clotting occurs so get hyperccoagulable state => thrombus formation and thrombocytopenia

183
Q

diagnosie herparin induce thrombocytopnia

A

look for heprain indudced antibodiesin blood- PF4 antibodies

184
Q

treatment of herparin indce thrombocytopenia

A

stop heparin and give diff anticoag

185
Q

budd chiari syndrome

A

thrombosis developsin the hepatic vein blocking out flow of blood

asscoaited with hypercoagualble state
casues acute hepatits

186
Q

abdo pain
hepatomegaly
ascites

A

budd chiari syndrome

187
Q

whats the triad symptoms of budd chiari syndrome

A

hepatomegaly
ascites
abdo pain

188
Q

manamget of budd chiari syndrome

A

hepatin/warfarin
look for casue of hyper coagulation
treat hepatitis

Medicine (11 decks)

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