MedEd Haem 1 Flashcards
name myeloid cells
RBCs
mast cells
basophils / neutrophils / eosinophils
platelets
PC of acute leukaemias
anaemia
thrombocytopaenia
neutropenia
(pancytopaenia)
bone pain
fever
which type of leukaemia has splenomegaly
chronic > acute
PC of chronic leukaemias
lymphadenopathy
splenomegaly
why does chronic leukaemia not have same Sx as acute
chronic is slower proliferation of malignant cells
less burden of disease in BM in chronic
what is the leukaemia emergency
leukostasis
what is leukostasis
EMERGENCY
high WCC –> increased viscosity of blood –> end organ damage
PC of leukostasis
retinopathy
pulmonary infiltrates
bleeding
thrombosis
Tx of leukostasis
leukaphoresis
chemo
steroids
which type of leukaemia is leukostasis common / uncommon
AML common
then ALL / CML
CLL unusual
in what type of leukaemia can pts be asymptomatic
CLL
classical Hx of ALL
2-5 y/o child
hepatosplenomegaly
bone pain / limp
fevers
what % of pts have CNS disease in ALL
10%
what sign is rare but very specific to ALL in boys
testicular swelling
how do adults present with ALL
similar to AML
lymphadenopathy
BTs and results of ALL
FBC
thrombocytopaenia
anaemia
high WCC but low neutrophils
film - blasts in peripheral blood
can circulating blast cells be normal
NO - indicates leukaemia or a few other conditions
features of blast cells on blood film
high nucelus:cytoplasm ratio
larger than RBCs (normal lymphocytes are same size as RBCs)
what cell shape is indicative of ALL vs AML
‘hand mirror’/’table tennis bat’ shaped
how is ALL Dx
BM biopsy
flow cytometry
(hard to tell AML from ALL on blood film)
what markers are typically present in ALL flow cytometry and what do they indicate
TdT+ / CD34+ (immature cell marker)
CD19 (B cell marker)
CD3 (T cell marker)
what genetic mutation causes ALL in 20-30% of adults
BCR-ABL1 t(9;22)
(also causes CML)
Tx of ALL
chemo
transplant of BM
RFs for AML
incidence increasing with age
previous history of myelodysplastic syndrome / chemo
BTs and results of AML
FBC - anaemia, thrombocytopaenia, high WCC, low neutrophils
film - high blasts
normal or high INR
blood film of AML
auer rods (stick in the lymphocytes)
what % of AML has auer rods
20-30%
flow cytometry markers present in AML
MPO CD34
CD13 / 33 / 117
(xs detail but may come up)
Tx of AML
chemo - shorter duration than ALL
PC of APML (acute promyelocytic leukaemia)
DIC - inc intracranial bleeding
good prognosis after induction
Tx of APML
ATRA (vitamin A)
all trans retinoic acid
which of these is most concerning for acute leukaemia?
neutrophils 12
10cm splenomegaly
microcytic anaemia
blast cells on blood film
cervical lymphadenopathy
blast cells on blood film
what are myeloproliferative neoplasms (MPNs)
increased production of myeloid lineage
what can cause a high platelet count (in order of most common)
acute infection
chronic inflammatiom
malignancy (5-10%)
essential thrombocytopaenia
polycythaemia rubra vera
what is ET
platelet count >450 consistently with no other cause
complications of ET
thrombotic events arterial / venous
small risk transformation to myelofibrosis / AML
what mutations are present in ET
JAK2 55%
CALR / MPL in most other pts
Tx of ET
aspirin - reduce stroke risk
hydroxycarbamide - lower platelets
what is PCV
polycthaemia rubra vera
overproduction of RBCs
Hct levels of PCV in men / women
men >0.52
women >0.48
complications of PCV
high risk thrombotic events eg stroke
Budd Chiari (hepatic vein thrombosis)
risk of transformation to MF / AML
what mutation do 95% of PCV pts have
JAK2
TX of PCV
aspirin - reduce stroke risk
venesection - lower Hct
hydroxycarbamide - lower plts
causes of high HCt
PCRV
altitude
chronic hypoxia - severe COPD / cyantoic heart disease / OSA
erythropoeitin secreting renal cancers
what is myelofibrosis
clonal proliferation of stem cells in BM. also overproduction of cytokines that cause fibrosis of BM –> reduced production of all cell lineages.
complications of myelofibrosis
risk of transformation to AML
what conditions can develop into myelofibrosis
ET
PCV
mutations causing myelofibrosis
JAK2 50%
CALR
2 buzzwords for myelofibrosis
‘dry tap’ on BM aspirate
‘tear drop poikilocytes’ on blood film
Tx of myelofibrosis
stem cell transplant
ruloxitinib - JAK inhibitor
name 4 myeloproliferative neoplasms
essential thrombocytopaenia
polycythaemia vera
myelofibrosis
CML
typical history of CML
35-55 y/o
LUQ pain with splenomegaly
asymptomatic if in chronic phase
FLAWS OR acute leukaemia Sx if in blast phase (10%)
3 key causes of splenogemaly
CML
myelofibrosis
leishmaniasis
BTs of CML and results
FBC - anaemia, high WCC, normal or high platelet count, neutrophillia, may have elevated basophils, low monocyte count
film - precursor cells (maybe)
other causes of high basophil count
worry about CML !! not much other causes
causes of high WCC with which cell in particular would be raised in each
acute bacterial infection - neutrophils high
acute viral infection - lymphocytes high
fungal / parasitic infection - eosinophils high
TB / endocarditis / inflammation - high monocytes
another cause of increase precursor cells on film
severe acute infections
elevated basophil and eosinophils
CML !!
blood film features of CML
left shift (more precursors)
leukocytosis
eosinophils
basophilia
hypoblasted megakaryocytes
how is CML Dx
almost all with philadelphia chromosome (BCR - ABL1 t(9;22)) using FISH
phases of CML
chronic 85-90%
accelerated (recently removed from classification but increasing number of blasts in BM)
blast phase - >20% blasts in BM, PC like acute leukaemia
Tx of CML
tyrosine kinase inhibitors (eg imatinib)