Haematology Flashcards
(188 cards)
1
Q
whats anaemia
A
low Hb
2
Q
casues of microcytic aneima
A
TAILS
thalassemia
anaemia of chronic disease
iron deficiency
lead poisoning
sideroblastic anemia
3
Q
causes of normocytic anaemia
A
3A, 2H
anemia of chronic disease
acute blood loss
aplastic anemia
hypothyroidism
haemolytic anemia
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
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
6
Q
signs of anemia
A
pale skin
conjunctival pallor
tachycardia
high rr
7
Q
koilonychia
A
iron deficiency anaemia
8
Q
anglular chelitis
A
iorn defciency anaemia
9
Q
atrophic glossitis
A
iron deficinecyanamie=- smooth tongue
10
Q
brittle hair and nails
A
iron deficiency anemia
11
Q
jaundice in what casue anemia
A
haemolyitc anaemia
12
Q
bone deformities can be seen in what cuase of anemia
A
thalassaemia
13
Q
oedema, ht, excoritations on skin what cause of anemia
A
CKD
14
Q
investigfations inital for anemia
A
Hb
Folate
B12
ferritin
blood film
MCV
15
Q
further investigation for anemia
A
OGD, colonoscopy for unexplained iron deficiency aneia - 2ww
bone marrow biopsy in unclear casue
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
17
Q
what medications can affect iron absorbtio n
A
meds that reduce stomach acid- PPI
need acid to keep iron in soluble form
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
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
20
Q
what form is iron travelling in blood
A
Fe3+ ferric irons bind to trasnferrin
21
Q
whats total iron binding capacity
A
total space oon transferring moleucle for iron to bind
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
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
24
Q
serum iron
how useful
A
varies throughout day
higher in morning and after iron meal
25
iron deficiency anemia bloods
Hb
MCV
serum ferritin
serum iron
TIBC
trasnferrin saturation
trasnferrin
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)
26
managment of iron deficiency
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
27
casues of B12 deficiency
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
28
pathophysiology of pernicious anemia
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
29
signs and sympotms b12 deficiency
(same as sign and symptoms of pernicious anemia)
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
30
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
B12 deficiency
folate deficiency- dont have the neruo symtpoms
neuro complication- subacute combined degenration of spinal cord may occur despite normal b12
31
investigfations for pernicious anemia
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
32
What autoimmune conditions can be seen in patients with pernicious anemia
hashimotos
t1dm
adidsions- hypoaldosteronism
vitiligo
33
manamgent of b12 deficiny
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
34
treat pernicious anemia pt with neuro symtpoms
1mg IM hydroxycobalamin alternate days until symtpoms improve and no further imporvemnt then every 2 months 1mg IM
35
patient has b12 deficinecy and folate deficnecy
how manage
treat b12 deficinecy first as if treat folice acid and got b12 deficiency can casue subacute combined degenration of spinal cord
36
blood cells develop whre
bone marrow
37
what are pluripotent haemoatopoietic stemm cells
undifferentiaed cells with potential to trasnapform into variety blood cells
38
pluripotent haemoatopoietic stem cells become what
myeloid stem cells
or
lymphoid stem cells
or
dentritic cells via stages
39
stages of cells of red blood cells
pluripotent haemoatopoietic stemm cells
-->
myeloid stem cells
-->
reticulocytes- immature rbc
-->
rbc - survive 3 months
40
platelts are made form what
megakaryocytes
survive 10 days
41
white blood cells what stages of cells come frm
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
42
where do t cells mature
thymus
43
where do b cells mature
bone marrow
44
b and t cells made from what
lymphoid stem cells
45
other wbc not lymohoctes made form
myeloid stem cells
46
anisocytosis on blood film
variation size rbc
myelodysplasic syndrome
some forms anaemia
47
target cells on blood film
iron deficincy
posy splenectomy
48
heinz bodies on blood film
dna left in- spleen normallly removes
post splenectomy
severe anemia- where rbc generated v quickly
49
reticulocytes on blood film
increase when have rapid turnover of rbc
hameolytic anemi
= represent the bone marrow is active in replacing lost cells
50
schistocytes
fragments of rbc
haemolytic anemia
HUS
DIC- disseminated intravascualr coagulation
thrombotic thrombocyti[enic purpura
metallic heart valves
51
sideroblasts
immature rbc with blob iron in
bone marri unable to incorporate iron into the hb
myelodysplastic syndorme
52
smudge cells
ruptured wbc
chornic lymphocytic leukaemia
53
spherocytes
autoimmune haeolytic anemia
hereditary spherocytosis
54
whats serum
clotting facotrs removed
glucose in
electroyltes in
protiens in- hormones and immunoglobulins
55
inherited cuases of haemolytic anaemia
hereditary spherocytosis
hereditary elliptocystosis
thalassaemia
sickle cell anemia
GP6D deficiency
56
acquuired cuase sof haemolytic anemia
alloimmmune hameolytic anaemia
autoimmune haemoltic anemia - trasnfusion rxn/haemoltic in newborn
paroxysmal nocturna; haemaglobinuria
microangiopathic haemolytic anemia
prostehtic heart valve related hameolysis
57
features of haemolytic anemia
jaundice
anemia
splenomegaly- filled wth destoryed rbc
58
investigations for hameolyic anemia
blood film- schistocytes
FBC- normocytic anemia
direct coombes test- postive in autoimmune haemolytic anemia
59
jaundice
splenomegaly
gallstones
anamie - normocytic
hereditary spherocytosis
hereditary elliptocytosis
GP6D deficincy
60
herediary spherocytosis
s and s
dx
rx
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
61
hereditary elliptocytosis
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
62
GP6D deficiency
what it is
s and s
dx
triggers
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
63
pt jaundie
becomes anemia
this is after eating broad beans/ developing an infection/ treated with anitmalariasl
GP6D deficiency
64
autoimmune haemoltic anemia
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
65
infections/ conditions that can casue autoimmune haremolytic anameia cold type
leukaemia
lymphoma
SLE
EBV
HIV
CMV
mycoplasma
66
paroxysmal nocturnal haemolglobinura
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
67
alloimmune haemolytic anemia
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
68
anemia after blood trasnfusion
alloimmune haemolytic anemia
69
microangiopathic haemolytics anemia
small bv structure abnomalitiy casuing hamolysis of blood as it goes through
usualy secondary to underlying ocndition:
HUS
DIC
TTP
SLE
cancer
70
red urine in morning
hard to swallow
dvt
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
71
thalassaemia
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
72
presentation thalassaemia
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
73
ivestgations for thalassamei
FBC- microcytic anemia
Hb electrophoreisis
DNA testing - genetic abnormality
regant women are offered screen test at boooking
74
what can happen to patients iron levels in thalassaemi
can ge tiron overload
due to creation of faulty rbc, recurrent transufsions and increased absorbtio of iron in response to the anamei
75
how do you manage iron overload in pt with thallasmeia
mornitr serum ferritin
limit trasnufsions and give iron chelation
76
iron overload in thalassaemia can casue what
similar to haemochormatosis symtmptons
liver cirrhosis
fatigue
infertility and impotence
heart fialure
diabetes
arthritits
OA and joint pain
77
types of thalassaemia
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
alpha thalassaemia
what it is and rx
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
beta thalassaemia minor
what it is and rx
beta chain abnrmaility
chromosome 11
one abnormal gene and one normal gene
mild micorcytic anameia
monitor only = carrier
80
beta thalassaemia intermedia
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
beta thalassaemia major
what i t s
causes
rx
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
sickle cell anemia
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
sickle cell trait
one abnormal gene = reduces severity of malaria = sickle cell disease seen more in areas of africa, carribean, mid east and india
usually asymptomatic
84
diangosis of sickcle cell disease
pregnacy if at risk of being carrier are offered screening
at heel prick screening at 5 days old
85
complcaitions of sickle cells disease
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
general mamangment of sicke cell disease
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
whats aplastic crisis
what it is
what casues iit
rx
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
sickle cell crisis
umbrella term for spectrum of acute crises related to condition
can be mild to life threatening
89
casues of sickle cell crisis and managment
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
vaso oclusive crisis
also called painful risis
sicke shaped rbc get stuck and clogg the cappialiress=> distal sichemia
associated with dehydration and high haematocrit
91
symtpms of vaso oclusive crisis
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
splenic sequestrian crisis
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
treat splenic sequestrian crisis
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
acute chest symdrome
in sickle cell aniemai
have fever / resp symotkmas WITH new infilatrates in CXR
95
casues of acute chest syndrome
sickle cell anemia and then either:
infection= pnumonia/ broncholitis
or non infective:
pulmonary vaso oclusion/ fat emboli
96
treamtent of acute chest syndrome
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
what antibiotics use forn prophylaxis of infection in sickle cell anemia
penicilllin V= phenoxymethypenicllin
98
lymphoma
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
hodgkins lymphoma risk facotrs
HIV
EBV
fam history
autoimmue conditions- RA, sarcoidosis
1 in 5 lymohomas are hodgkins
bimodal age distribution 20 s and 75
100
presentation of all lymphomas
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
investigaions for lymphomas
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
key diangosit for lymphoma
biospy
103
reed sternberg cell foun on biopsy
hodgkins lymphpma
104
what use for staaging of lymphpmas
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
types on non hodgkins lyphoma
burkitt lymphoma
MALT lymphoma
diffuse large B cell lymphoma
106
burkitt lymphoma is assocaited with what
HIV
EBV
malaria
107
MALT lymphona is what, where usually and assicated with what
mucosa assocaited lymphoid tissue
usally around the stomach
associated with H.pylori
108
diffuse large B cell lymohpma presents as what
rapidly growing painless mass in pts over 65
109
rapidly growing painless mass in pts over 65
diffuse large b cell lymphoma
110
h.pylori infection
enlarge lymphnoeds
MALT lymphoma
111
risk factors for non hodgkins lymphoma
HIV
EBV
H pylori- malt lymphoma
hep B/C
exporusre to pesticies and trichloroethylene (used in industrial process)
112
treatment for hodkins lymphoma
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
treamtnent for non hodgkins lymphoma
depends on type
watch and wait
radioatheyrpy
chemo
monocolonal antibodies eg rituximab
stem cell trasnplantation
114
leukaemia
cancer og a particular of stem cells in the bone marrow
unregulated production of certain types of blood cells
115
patholphisioly leaukamei
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
types of leukamiae
acute myeloid leukameia
acute lymphoblastic leukaemia
chronic myelroid leukamia
chronic lymphocytic leukameia
are others
117
ages get the diff leuakmiea
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
presentation of leukamiae
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
differentials of petechiae
non blacnhing rash - asmall dots
leuakaemia
menigiococcal septicaemia
vascultits
henoch - schonlein purpura
idiop[athic thrombocytopenia purpura
non accidental injury -ABUSE- dont forget
120
diagnosis for leukaemia tests
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
Von Willebrand disease
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
presentation von willebrand disease
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
pt has lots nose bleeds and bleeding of gums
ask about periods and they have v heavy periods too
fam hx of ehavy bleeding
vON wILLEBRAND DISEASE
- most common inherited casue pf abnormal bleeding- haemophilia
124
diagnosis for VW disease
clinical- hx, fam hx
bleeding assesment tools
lab stuff
125
managemnt of patient with von willerand disease
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
myeloma
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
monocoloncal gammopathy of undetermined signifiance- MGUS
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
smouldering myeloma
progression of MGUS
with higher levels of antibody/componenets = premalignant
eg. waldenstroms macroglobulinaemia = type of excessive IgM
129
most common Immunoglobulin (antibody) thats abundant i myeloma
most common one that is produced lots is IgG
130
paraprotein
nonocolonal paraprotein= the single type of abnormal protein produced in myeloma
131
features of myeloma
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
why does hypervisocity occur in myeloma
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
why do you get anemia, leukopenia, neutropenia and thrombocytopenia in myeloma
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
why d you get myeloma bone disease
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
why do you get myeoma renal diseae
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
plasmacytomas in myeloma
they can deveop= tumours formed by cancerous plasma cells in the bones and replace normal tissue
137
investigations for susepcted myloma
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
initial investigfations myeloma
BLIP
bence jones protein = urine elctrophoreiss
Serum free Light chain asssay
serum Immunoglobulins
Serum protein electropheresis
139
diagnostic investigations myloma
bone marrow biospy
iaging - whole body MRI
140
what see on xray in pt with myeloma
ouched out lesions
lytic lesions
pepperpot skull- due to lots of punched out lesions scattered in skull
141
managment of meyloma
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
complication of myeloma
anemia
hypercalcemia
hyperviscotiy
renal fialure
pain
infection
peripheral neuropahty
spinal cord compressio
143
pt has spinal cord comrepssion
AKI/ HYPERCALCEMIA
admit to hosp immediatelt
myeloma complciations
144
bence jone protein found in urine electrophoreisis
myleoma
145
myelodysplastic syndrome
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
risk facotrs for myelodysplastic syndorme
over 60s
had chemo/radiotherpay
147
presentation of myelodysplastic syndrome
anemia- pallor, SOB, fatigue
thrombocytopenia - purpura/ bleeding
neutropenia- frequent/ severe infecitons
148
diangosis of myelodysplastic syndrome
FBC abnormal - inital- low rbc, low platlekts, low neutrophils
blood film- blasts
diagnositc:
bone marrow aspiration and biospy
149
managment of myelodysplasti syndrome
depends on symtpoms and risk of progression and prognosis:
watch and wait
blood transfusion if severe anemia
chemo
stem cell transplantation
150
myeloproliferative disorders
type of bone marrow cancer
occurs due to uncontrolled proliferation of a single type of stem cell
151
primary myelofibrosis
due to what stem cell proliferation
haematopoetic stem cell
152
essentail thrombocythaemia
due to what stem cell proliferation
megakaryocyte
153
polycythameia vera
due to what stem cell proliferation
eryhthroid cels- expansion of red cell mass
154
what leuakemia can myeloproliferative disorders progress to
they cance rof bone marrow them selves
but can progress to acute myeloid leukaemia
155
what myelofibrosis due to and what is it
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
presentation of myeloproliferative disorders
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
polycythameia vera signs and symtpoms
fatigue
pruitus
symptomatic splenomegaly
increase risk of thrombus formation
can progress to myelofibrosis and acute myeloid leukaemia
158
what will be deranged in polycythamia vera in FBC
high Hb = over 185 in men and over 165 in women
159
what will be deranged in essentail thrombocythaemia in FBC
rasied platelets = over 600 x10 9
160
what will be deraged in meylofibrosis
on FBC
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
blood film show for myelofibrosis
tear drop rbc
aniscotysosis- vary sixe rbc
blasts- immature rbc and wbc
162
FBC in myeloproliferative disordera
PV= rasied RBC
essential thrombocytothameia = rasied platelets - over 600
meylofibrosis- varies= anemia, leukpenia/ leukocytosis, thrombocytpenina/ thrombocytosis
163
tear drop shaoed rbc
myelofibrosis
164
what genes are associated with myeloproliferative disorders
JAK2
MPL
CALR
165
JAK2
MPL
CALR
these genes associated with:
myeloprolgoerative disorders
JAK2 ca treat eith jak2 inhibtir- ruxoliitinib
166
treatment for primary myelofibrosis
mild= monitor
allogenic stem cell trasnplantation - can cure but has risks
chemo- not currative on own
supoort for anemia, splenomegaly, portal ht
167
treatment for polycythamia vera
first line= venesection - get Hb and hametocrit to normal
aspirin- redice risk of thrombus formation
chemo- control it
168
treamemnt for essentail thrombocytothamia
aspirin- reduce risk of thrombus formation
chemo- control it
169
haemophilia types and what is it
inheirted bleeding disorder
haemophilia A= deficinecy in facotr VIII
haemophilia B (christmas disease) = defieicnecy in factor IX
170
haemophilia is what type of genetic condition
x linked receissve!!!
= mostly males affected
171
presentation of haemophilia
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
diangosis of haemophilia
bleeding scores
fam hx
coagualtion factor assay
genetic testing
173
manamgemnt of hameophilai
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
what drugs not to give pts with haemophilia
avoid nsaids
avoid anticoagulants
175
casues of thrombocytopenia
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
presentation thrombocytopenia
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
differntials of abnormal / prolonged bleeding
too high anti coag meds
thrombocytopenia
haemophilia A and B
von willebrand disease
disseminated intravascualr coagulation
178
whats immune thrombocytopenia purpura
also called idiopathic/ autoimmune/ primary ...
antibodies are created agasint the plateltes leading to destruction of platelets
179
treat immune thromboctyopenic purpura
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
thrombotic thrombocytopenic pupura
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
protien thats deficient/ antobodies agasitn it in thrombotic thrombocytopenic purpura
ADATS13
182
heparin induce thrombocytopenia
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
diagnosie herparin induce thrombocytopnia
look for heprain indudced antibodiesin blood- PF4 antibodies
184
treatment of herparin indce thrombocytopenia
stop heparin and give diff anticoag
185
budd chiari syndrome
thrombosis developsin the hepatic vein blocking out flow of blood
asscoaited with hypercoagualble state
casues acute hepatits
186
abdo pain
hepatomegaly
ascites
budd chiari syndrome
187
whats the triad symptoms of budd chiari syndrome
hepatomegaly
ascites
abdo pain
188
manamget of budd chiari syndrome
hepatin/warfarin
look for casue of hyper coagulation
treat hepatitis
