Haematology Flashcards
heparin
full vs lmwh
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
Hep Induced Thrombocytopaenia
HIT
actually pro thrombotic state
5-10 days post treatment
low vit c
bleeding gums
low niacin
syndorme called pellagra
dermatitis
dementia
diarrhoea
warfarin
vit k antag
affects extrinsic pathway
monitor prothrombin time - INR
low vit d
osteo rickets
low thiamin
from starvation - ie in alcoholics
Beriberi
- wet - heart failure
- dry - affects cns
affects brain: wernicke korskoff
low b12
megaloblastic anaemia mouth tongue (glossitis) dementia neuropathy eyes and gut
low vit a
blindness
low folate
anaemia
ntd in pregnancy
anti CCP
RA
ANA
SLE (anti ds-dna, anti smith)
autoimmune hep
SSc
- cutaneous: anti centromere
- diffuse: anti-topoisom, scl-70
AMA
primary biliary cirrhosis
SMA
autoimmune hep
TTG, endomysial antibodies
coeliac
anti GBM
goodpastures
C-ANCA
wegeners
acute laeukaemias
- 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
acute leakaemia diagnostics
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
hyponatraemia <137
hypovol
- diuretics (thiazide), addisons
- vom, diarhh, burns
euvol
- SIADH
hypervol
- liver, kidney, heart fail
drugs- SSRI, TCA, carbamez, cyclophosp
CLL
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
iron deficiency
microcytic
low iron, ferritin, high tibc
? gi bleed
also - coeliac, pregnancy, menorrhagia
koilonychia
angular stomatitis
tongue atrophy
CLL prognosis
thirds: no progress, slow, active
transformation to aggressive lymphoma = richters
treatment chemo (fludarabine, chlorambucil, rituximab) bm transplant if young
CML
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
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
normocytic anaemia
chronic disease
EPO deficiency (renal fail)
haemolytic
macrocytic anaemia
megaloblastic
hypothyroid (mild)
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
pernicious anaemia
autoimmune destruc of parietal cells and intrinisc factor
anti GPC, anti IF
no IF –> no B12 abs
inc risk gastric ca
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
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
spherocytosis
autosomal dominant cause of anaemia
abnormal membrane
SPLENOMEGALY
often no need for treat
if severe - splenectomy
G6PD defic
Inherited cause of anaemia X linked - affects males common in tropics usually asympto until trigger (infect etc) usually self limiting, supportive tx
HUS haemolytic uraemic syndrome
acquired cause of anaemia
assoc w diarrhoea e coli 0157
see degree of renal failure –> uraemia
low platelets
TTP thrombotic thrombocyto pupura
pentad
- haem anaem
- thrombocyto
- neuro signs (ie stroke)
- renal imp (occlusion)
- fever
fatal w/out treat
- plasmaphoresis, FFP
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
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
bone marrow failure
anaemia, infection, rash/bleeding
malignancy radiotherapy, cytotoxic drugs aplastic anaemia (idiopathic, hep c) - hypocellular bm myelodisplasia/fibrosis sepsis, alcohol, b12 def
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
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
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
burkitts lymphoma
high grade b cell lymphoma
endemic in africa - jaw lymphadenopathy
may see tumour lysis syndrome post chemo - confsion, muscle pain, renal probs
low grade NHL
elderly
T: chemo - chlorambucil or FCR
1) follicular - t(14:18)
2) waldestroms - IgM paraprotein - hyperviscosity
- headache, visual dist, lethargy
- - plasmaphoresis
Myeloproliferative disorders
CML
PRV
essential thrombocythaemia
myelofibrosis
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
essential thrombocythaemia
plt may be over 1000
type of myeloprolif disorder
asympto or thombotic event
aspirin vs thrombosis
? hydroxylurea
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
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%
myeloma treat
often start w thalidomide and prednis
bisphosphonates vs bone lesions
plasmaphoresis vs viscosity
MGUS
monocloneal gammopathy of uncertain significance
aka benegn paraproteinaemia
paraprotein but no path symtpoms
1% devel myeloma
myelodysplasia
elderly
bm produces abnormal cells
anaemia common
- macrocytic but folate/b12 normal
? infection
? low plt
support: transfuse, plt, abx
?bm transplant
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
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)
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
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
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,
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
