Haematology Flashcards
(57 cards)
1
Q
heparin
full vs lmwh
A
affects intrinsic pathway
activate antithrombin iii
se bleeding, HIT, osteo, hyperkal
FULL
monitor aptt
given IV
LMWH
monitor anti factor X (not routine)
subcut inject long lasting fewer side effects
2
Q
Hep Induced Thrombocytopaenia
HIT
A
actually pro thrombotic state
5-10 days post treatment
2
Q
low vit c
A
bleeding gums
3
Q
low niacin
syndorme called pellagra
A
dermatitis
dementia
diarrhoea
4
Q
warfarin
A
vit k antag
affects extrinsic pathway
monitor prothrombin time - INR
5
Q
low vit d
A
osteo rickets
6
Q
low thiamin
A
from starvation - ie in alcoholics
Beriberi
- wet - heart failure
- dry - affects cns
affects brain: wernicke korskoff
7
Q
low b12
A
megaloblastic anaemia mouth tongue (glossitis) dementia neuropathy eyes and gut
8
Q
low vit a
A
blindness
9
Q
low folate
A
anaemia
ntd in pregnancy
10
Q
anti CCP
A
RA
11
Q
ANA
A
SLE (anti ds-dna, anti smith)
autoimmune hep
SSc
- cutaneous: anti centromere
- diffuse: anti-topoisom, scl-70
12
Q
AMA
A
primary biliary cirrhosis
13
Q
SMA
A
autoimmune hep
14
Q
TTG, endomysial antibodies
A
coeliac
15
Q
anti GBM
A
goodpastures
16
Q
C-ANCA
A
wegeners
17
Q
acute laeukaemias
A
- present unwell - fever, sweat, wl, malaise
- splenomeg, lymphadenop
- signs of marrow failure
1) resus - abx, transfuse
2) chemo (+ allopurinol vs tls)
- - BM transplant
ALL - kids, treat for years
AML - inc w age, AUER RODS, treat for months
18
Q
acute leakaemia diagnostics
A
blood
- pancytopenia
- wcc normal or raised
- blasts present
BM
- hypercellular
- > 30% blasts
in AML
- auer rods, myeloid enzymes
in ALL
- lymphoid cells eg b/t cells
19
Q
hyponatraemia <137
A
hypovol
- diuretics (thiazide), addisons
- vom, diarhh, burns
euvol
- SIADH
hypervol
- liver, kidney, heart fail
drugs- SSRI, TCA, carbamez, cyclophosp
20
Q
CLL
A
malig of B cell prolif - functionally immature
most common leuk - age, male, fh
massively raised wbc - may be chance finding
- may be >15000
- many lymphocytes
b symptoms (sweat, fever, wl) lymphadenopathy /organomeg can get autoimmune haemolysis (coombes) marrow faliure - anaemia - infection (low neutrophils) - platelet
anaemia - may be haemolytic or normocytic
may see monoclonal gammopathy
*peripheral blood flow cytometry more useful than BM
22
Q
iron deficiency
A
microcytic
low iron, ferritin, high tibc
? gi bleed
also - coeliac, pregnancy, menorrhagia
koilonychia
angular stomatitis
tongue atrophy
22
Q
CLL prognosis
A
thirds: no progress, slow, active
transformation to aggressive lymphoma = richters
treatment chemo (fludarabine, chlorambucil, rituximab) bm transplant if young
23
Q
CML
A
middle age (rarely affects kids)
b symptoms, splenomeg, anaemia
platelets less affected
get ‘blast’ transformation (aml or all)
PHILADELPHIA CHROMOSOME t9:22
wcc raised >30 often >100
- esp neutrophils
normocytic anaemia
BM
- hypercellular
- granulocytic hyperplasia
- megakaryocytes
t: imatininb v effective
24
thalassaemia
trait - mild microcytic anaemia
disease = serious
- beta-globulin (alpha disease die inutero)
- anaemia in baby
- affects growth, infections, ulcers, skull bossing (hair on end)
- SPLENOMEGALY
tx: transfusio dependant, iron chelators (Vs overload)
bm transplant
survival 40's
25
normocytic anaemia
chronic disease
EPO deficiency (renal fail)
haemolytic
26
macrocytic anaemia
megaloblastic
| hypothyroid (mild)
27
megaloblastic anaemia
macrocytic
commonest cause: ALCOHOL
- low folate (can deplete quickly)
- diet, coeliac, methotrexate
- low b12 (chronic) ie pernicious anaemia
* neurology - periph, sc degen, --> IM B12 5 days, then every 3 m for life
* glossitis
hyper segmented neutrophils
28
pernicious anaemia
autoimmune destruc of parietal cells and intrinisc factor
anti GPC, anti IF
no IF --> no B12 abs
inc risk gastric ca
29
haemolytic anaemia
hereditary
- sphero, G6PD, pyruvate kinase def, sickle, thalass
acquired
- TTP, HUS, autoimmune, Cll
- cardiac, drugs, infection
tests - inc uncong bilirubin, inc urinary urobilinogen
inc LDH
reticulocytes
polychromasia
30
autoimmune haemolysis
coombes +ve
fatigue
splenomegaly
red cell agglutination on blood film
warm or cold
warm IgG
splenic phagocytosis
can be idio or assoc w sle, lymphoprolif, ibd, drugs
T: pred -> blood transfusion -> splenectomy
cold IgM, purple colour to extremeties
idio or post infection (1-2 weeks)
can be precip by cold
may be assoc w lymphoprolif, chlorambucil
31
spherocytosis
autosomal dominant cause of anaemia
abnormal membrane
SPLENOMEGALY
often no need for treat
if severe - splenectomy
32
G6PD defic
```
Inherited cause of anaemia
X linked - affects males
common in tropics
usually asympto until trigger (infect etc)
usually self limiting, supportive tx
```
33
HUS haemolytic uraemic syndrome
acquired cause of anaemia
assoc w diarrhoea e coli 0157
see degree of renal failure --> uraemia
low platelets
34
TTP thrombotic thrombocyto pupura
pentad
- haem anaem
- thrombocyto
- neuro signs (ie stroke)
- renal imp (occlusion)
- fever
fatal w/out treat
- plasmaphoresis, FFP
35
sickle
beta globin
electrophoresis shows HbS band (with normal HbA band if trait)
sickle solubility test - +ve in train and disease
trait - one sickle and one normal beta Hb
- asympto, normal blood film
disease - both sickle beta Hb's
- change shape when deox
- prone to occlusions, mild anaemia
- HYPOSLENISM due to infarct - need penicillin, folic acid
- prone to crises
36
sickle crises
T; transfuse
THROMBOTIC
painful vascular occlusion - often to bone
maybe post infec/dehydration/stress
APLASTIC
post PAROVIRUS
sudden drop Hb
SEQUESTRATION
- acute chest syndrome - cough, cp
- splenic
HAEMOLYTIC
rare sudden drop hb, raised reticulocytes
37
bone marrow failure
anaemia, infection, rash/bleeding
```
malignancy
radiotherapy, cytotoxic drugs
aplastic anaemia (idiopathic, hep c)
- hypocellular bm
myelodisplasia/fibrosis
sepsis, alcohol, b12 def
```
38
complications/features of aplastic anaemia
See hypocellular blood film
treatment of aa
support (transfuse, abx) -> bm transplant -> immunosupp
paroxysmal noctunal haemaglobinuria
- may cause budd chiari
fanconi's
- early onset aa, with skeletal, growth, pigment probs
39
hodgkin lymphoma
REED STERNBERG - large, eosinophilic, multinucleated or bilobed nucleus
** Ann Arbor Staging
good cure rate w chemo
lymphadenopathy in neck
b sympotms: wl, fever, night sweats
EBV link
lymphocyte predominant - best progn but rare
lymphocy depleted - worst prog
'nodular sclerosing'
-most common
40
non hodgkin lympyhoma types
80% B cell 20% T cell
high grade or low grade
- B symptoms and lymphadenopathy (rubbery non tender)
low grade - slow incurable
- follicular
- waldenstroms macroglobulinaemia
high grade - fast, 30% curable w chemo
* * Ann Arbor Staging
- DLBCL
- Burkitts t(8:14)
- 1) african endemic, EBV, mandibular
- 2) non endemic, HIV
41
burkitts lymphoma
high grade b cell lymphoma
endemic in africa - jaw lymphadenopathy
may see tumour lysis syndrome post chemo - confsion, muscle pain, renal probs
42
low grade NHL
elderly
T: chemo - chlorambucil or FCR
1) follicular - t(14:18)
2) waldestroms - IgM paraprotein - hyperviscosity
- headache, visual dist, lethargy
- - plasmaphoresis
43
Myeloproliferative disorders
CML
PRV
essential thrombocythaemia
myelofibrosis
44
myelofibrosis
proliferation and release of cytokines -> fibrosis
affects rbc --> anaemia
massive splenomegaly
also fever wl
nb wcc and plt may be norm, high or low
blood film - immature wbc and rbc
- tear drop rbc
t difficult, includes tranfuse, splenect, thalid/pred, hydroxyurea
4 yr survival
45
essential thrombocythaemia
plt may be over 1000
type of myeloprolif disorder
asympto or thombotic event
aspirin vs thrombosis
? hydroxylurea
46
polycythaemia rubra vera
inc red cells, 50% also rasied wcb and plt
risk thromboembolic events
treat venesection -> hydroxyureea
- ?aspirin
JAK2
pruritis, bruing, red/blue discol
splenomegaly
flushed
may convert -> myelofib, or aml
47
myeloma
malig prolif of plasma cells
monocal Ig paraproteinaemia
back pain in elderly
infection
anaemia
renal fail
anaemia from marrow infiltrate, renal imp, chronic disease
hypercalcaemia in 25%
excess light chains - levels can be monitored
bence jone in urine
--> renal failure in 50%
48
myeloma treat
often start w thalidomide and prednis
bisphosphonates vs bone lesions
plasmaphoresis vs viscosity
49
MGUS
monocloneal gammopathy of uncertain significance
aka benegn paraproteinaemia
paraprotein but no path symtpoms
1% devel myeloma
50
myelodysplasia
elderly
bm produces abnormal cells
anaemia common
- macrocytic but folate/b12 normal
? infection
? low plt
support: transfuse, plt, abx
?bm transplant
51
rasie wbc - leukocytosis
neutrophils - bacterial infec
also
- steroids, tissue necrosis (MI), maignancy, inflamm disord, dka
lymphocyte - viral
eosinophil
- allergy, atopy, asthma
- parasitic
- vasculitis - esp PAN
- hodgkin lymphoma
52
thrombocytopenia
ITP
- autoimmune
- acute, self limitng in kids post viral
- also middle aged women (other autoimmue)
- exclude bm problems
- give stroid only if plt less than 30
- IgG in emergency
post transfusion
heparin induced
HUS and TTP (see sep cards)
53
coag disorders
Haemophilia - inherited
- bleed into muscles and large joints
- haemophilia A - defic factor 8 (affects APTT) - x linked
- haemophilia B - defic factro 9
- von Willebrand's disease
Acquired - DIC, liver disease, vit K defic (in malnut and obstruc jaundice)
DIC - both microvascular thromobisis with bleeding due to consumption
- give ffp, blood to buy time, address underlying cause
54
von Willebrands disease
Most common inherited bleeding disorder
vW factor either deficient or defective
- its usual job is to help platelet clot formation
Type 1 - partial deficiency
2 - total deficiency
3 - defective
Bruising, bleeding - menorrhagia, epistaxis
Not affect PT or APTT
Treat: desmopressin
55
thrombophilia
- mainly venous thrombosis
- may see dvt/pe, budd chiari
inherited
- include defic of prot c,s, antithrombin
- also factor V leiden (resistant to activated protein c) - defect in factor 5
acquired
* * antiphospholipid syndrome
- smoking, malig, cocp, polycythaemia,
56
antiphospholipid
either primary or assoc w sle etc
thrombosis and misacrriages
arterial (cva, ihd, renal) and venous (dvt/pe)
anti cardiolipin
anti phospholipid binding protein
platelets may be low (TTP)
may see anaemia
