MedEd Haem 1 Flashcards

1
Q

name myeloid cells

A

RBCs
mast cells
basophils / neutrophils / eosinophils
platelets

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2
Q

PC of acute leukaemias

A

anaemia
thrombocytopaenia
neutropenia
(pancytopaenia)
bone pain
fever

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3
Q

which type of leukaemia has splenomegaly

A

chronic > acute

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4
Q

PC of chronic leukaemias

A

lymphadenopathy
splenomegaly

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5
Q

why does chronic leukaemia not have same Sx as acute

A

chronic is slower proliferation of malignant cells
less burden of disease in BM in chronic

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6
Q

what is the leukaemia emergency

A

leukostasis

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7
Q

what is leukostasis

A

EMERGENCY
high WCC –> increased viscosity of blood –> end organ damage

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8
Q

PC of leukostasis

A

retinopathy
pulmonary infiltrates
bleeding
thrombosis

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9
Q

Tx of leukostasis

A

leukaphoresis
chemo
steroids

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10
Q

which type of leukaemia is leukostasis common / uncommon

A

AML common
then ALL / CML
CLL unusual

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11
Q

in what type of leukaemia can pts be asymptomatic

A

CLL

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12
Q

classical Hx of ALL

A

2-5 y/o child
hepatosplenomegaly
bone pain / limp
fevers

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13
Q

what % of pts have CNS disease in ALL

A

10%

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14
Q

what sign is rare but very specific to ALL in boys

A

testicular swelling

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15
Q

how do adults present with ALL

A

similar to AML
lymphadenopathy

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16
Q

BTs and results of ALL

A

FBC
thrombocytopaenia
anaemia
high WCC but low neutrophils
film - blasts in peripheral blood

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17
Q

can circulating blast cells be normal

A

NO - indicates leukaemia or a few other conditions

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18
Q

features of blast cells on blood film

A

high nucelus:cytoplasm ratio
larger than RBCs (normal lymphocytes are same size as RBCs)

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19
Q

what cell shape is indicative of ALL vs AML

A

‘hand mirror’/’table tennis bat’ shaped

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20
Q

how is ALL Dx

A

BM biopsy
flow cytometry
(hard to tell AML from ALL on blood film)

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21
Q

what markers are typically present in ALL flow cytometry and what do they indicate

A

TdT+ / CD34+ (immature cell marker)
CD19 (B cell marker)
CD3 (T cell marker)

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22
Q

what genetic mutation causes ALL in 20-30% of adults

A

BCR-ABL1 t(9;22)
(also causes CML)

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23
Q

Tx of ALL

A

chemo
transplant of BM

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24
Q

RFs for AML

A

incidence increasing with age
previous history of myelodysplastic syndrome / chemo

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25
Q

BTs and results of AML

A

FBC - anaemia, thrombocytopaenia, high WCC, low neutrophils
film - high blasts
normal or high INR

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26
Q

blood film of AML

A

auer rods (stick in the lymphocytes)

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27
Q

what % of AML has auer rods

A

20-30%

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28
Q

flow cytometry markers present in AML

A

MPO CD34
CD13 / 33 / 117
(xs detail but may come up)

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29
Q

Tx of AML

A

chemo - shorter duration than ALL

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30
Q

PC of APML (acute promyelocytic leukaemia)

A

DIC - inc intracranial bleeding
good prognosis after induction

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31
Q

Tx of APML

A

ATRA (vitamin A)
all trans retinoic acid

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32
Q

which of these is most concerning for acute leukaemia?
neutrophils 12
10cm splenomegaly
microcytic anaemia
blast cells on blood film
cervical lymphadenopathy

A

blast cells on blood film

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33
Q

what are myeloproliferative neoplasms (MPNs)

A

increased production of myeloid lineage

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34
Q

what can cause a high platelet count (in order of most common)

A

acute infection
chronic inflammatiom
malignancy (5-10%)
essential thrombocytopaenia
polycythaemia rubra vera

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35
Q

what is ET

A

platelet count >450 consistently with no other cause

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36
Q

complications of ET

A

thrombotic events arterial / venous
small risk transformation to myelofibrosis / AML

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37
Q

what mutations are present in ET

A

JAK2 55%
CALR / MPL in most other pts

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38
Q

Tx of ET

A

aspirin - reduce stroke risk
hydroxycarbamide - lower platelets

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39
Q

what is PCV

A

polycthaemia rubra vera
overproduction of RBCs

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40
Q

Hct levels of PCV in men / women

A

men >0.52
women >0.48

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41
Q

complications of PCV

A

high risk thrombotic events eg stroke
Budd Chiari (hepatic vein thrombosis)
risk of transformation to MF / AML

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42
Q

what mutation do 95% of PCV pts have

A

JAK2

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43
Q

TX of PCV

A

aspirin - reduce stroke risk
venesection - lower Hct
hydroxycarbamide - lower plts

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44
Q

causes of high HCt

A

PCRV
altitude
chronic hypoxia - severe COPD / cyantoic heart disease / OSA
erythropoeitin secreting renal cancers

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45
Q

what is myelofibrosis

A

clonal proliferation of stem cells in BM. also overproduction of cytokines that cause fibrosis of BM –> reduced production of all cell lineages.

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46
Q

complications of myelofibrosis

A

risk of transformation to AML

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47
Q

what conditions can develop into myelofibrosis

A

ET
PCV

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48
Q

mutations causing myelofibrosis

A

JAK2 50%
CALR

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49
Q

2 buzzwords for myelofibrosis

A

‘dry tap’ on BM aspirate
‘tear drop poikilocytes’ on blood film

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50
Q

Tx of myelofibrosis

A

stem cell transplant
ruloxitinib - JAK inhibitor

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51
Q

name 4 myeloproliferative neoplasms

A

essential thrombocytopaenia
polycythaemia vera
myelofibrosis
CML

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52
Q

typical history of CML

A

35-55 y/o
LUQ pain with splenomegaly
asymptomatic if in chronic phase
FLAWS OR acute leukaemia Sx if in blast phase (10%)

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53
Q

3 key causes of splenogemaly

A

CML
myelofibrosis
leishmaniasis

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54
Q

BTs of CML and results

A

FBC - anaemia, high WCC, normal or high platelet count, neutrophillia, may have elevated basophils, low monocyte count
film - precursor cells (maybe)

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55
Q

other causes of high basophil count

A

worry about CML !! not much other causes

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56
Q

causes of high WCC with which cell in particular would be raised in each

A

acute bacterial infection - neutrophils high
acute viral infection - lymphocytes high
fungal / parasitic infection - eosinophils high
TB / endocarditis / inflammation - high monocytes

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57
Q

another cause of increase precursor cells on film

A

severe acute infections

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58
Q

elevated basophil and eosinophils

A

CML !!

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59
Q

blood film features of CML

A

left shift (more precursors)
leukocytosis
eosinophils
basophilia
hypoblasted megakaryocytes

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60
Q

how is CML Dx

A

almost all with philadelphia chromosome (BCR - ABL1 t(9;22)) using FISH

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61
Q

phases of CML

A

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

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62
Q

Tx of CML

A

tyrosine kinase inhibitors (eg imatinib)

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63
Q

survival of CML

A

> 90% 10 year survival

64
Q

PC of CLL

A

asymptomatic
high WCC
>50 y/o M>F
some have small lymphadenopathy / splenomegaly
some present with ITP / haemolytic anaemia

65
Q

BTs of CLL and results

A

FBC - normal Hb, high WCC usually >100, normal plts, normal neutrophils, high lymphocyte count

66
Q

what does anaemia at presentation in CLL indicate

A

more aggressive disease or haemolytic anaemia

67
Q

blood film of CLL

A

smear / smudge cells - BUZZWORD
lymphocytosis

68
Q

Dx of CLL

A

flow cytometry
- immunophenotyping for clonal population of cells
- same cell markers eg kappa/lamba light chains

69
Q

what disease has same pathology as CLL but different distribution

A

small lymphocytic lymphoma
- blood / marrow vs lymph nodes

70
Q

how is CLL staged and how does this determine Tx

A

Binet staging
- A = no cytopaenia, <3 areas of lymphoid involvement
- B = no cytopaenia, 3+ areas of lymphoid involvement
- C = cytopaenia

A = watch and wait
B = consider Tx
C = Tx

71
Q

what aggressive condition can CLL transform into

A

Richters syndrome

72
Q

Tx of CLL and what does it depend on

A

if IGHV mutated = rituximab + chemo eg FCR
if TP53 mutated = BTK inhibitors eg ibrutinib
can use venetoclax (BCL2 inhibitor)

73
Q

causes of pancytopaenia

A

b12 / folate deficiency
medications - co-trimoxazole / linezolid
cancer - acute leukaemia, BM mets
aplastic anaemia - eg due to sepsis / AID / parvovirus b19
myelodysplastic syndromes
myelofibrosis

74
Q

what blast % would diagnose acute leukaemia

A

> 20%

75
Q

what is myelodysplastic syndrome

A

dysplatic changes (abnormal cells) in 1 or more myeloid cell lines (erythroid, megakaryocytes, granulocytes)

76
Q

PC of myelodysplastic syndrome

A

asymptomatic
incidental cytopaenia

77
Q

what can myelodysplastic syndrome progress to

A

AML

78
Q

characterise the anaemia in MDS

A

macrocytic anaemia
normal b12 / folate levels

79
Q

blood film of MDS - buzzwords

A

pseudo-pelger anomaly
bilobed neutrophils
ring sideroblasts

80
Q

4 prognostic factors for MDS

A

number /extent of cytopaenias
BM blast % (>20 = AML)
cytogenetic changes
specific mutations eg TP53 (poor)

81
Q

what % of MDS progress to AML

A

30%

82
Q

how is risk of progression to AML from MDS assessed

A

IPSS-R / IPSS-M

83
Q

Tx of MDS

A

higher risk - chemo
younger pts - stem cell transplant

84
Q

contrast age peak of HL and NHL

A

HL = young
NHL = increases with age

85
Q

contrast lymph node involvement in HL and NHL

A

HL = mediastinum mainly. painful on drinking alcohol
NHL = anywhere

86
Q

contrast course and prognosis of HL and NHL

A

HL = aggressive but mostly curable
NHL = variable course and prognosis

87
Q

roughly how are lymphomas categorised

A

HL or NHL
of NHL - B cell or T cell
(just 4 types of HL)
of B cell - subtypes eg Burkitts / mantle cell / follicular / DLBCL
(T cell is basically just ATLL)

88
Q

most common subtypes of lymphomas

A

NHL > HL
B cell > T cell

89
Q

which types of lymphomas are low grade

A

follicular B cell

90
Q

which types of lymphomas are high grade

A

diffuse large b cell lymphoma
burkitt’s (++++ high grade)

91
Q

staging of lymphoma

A

Ann-Arbour
1 = one nodal group
2 = 2+ nodal groups all same side of diaphgram
3 = 2+ nodal groups on both side of diaphragm
4 = infiltration of another organ that isn’t LN

A = no B Sx present
B = B Sx present

92
Q

age peaks of HL

A

bimodal
mainly <30 but also peak in 70s

93
Q

PC of HL

A

B Sx with lymphadenopathy (mediastinal, worse after alcohol)

94
Q

Dx feature of HL on blood film

A

reed - sternburg cells (BUZZWORD)
- large cell with 2+ nucleus
- may only see 1 per LN, still diagnostic

95
Q

most common type of HL

A

nodular sclerosing

96
Q

what is HL associated with

A

EBV

97
Q

Tx of HL

A

ABVD chemo + radio
stem cell transplant if Tx fails

98
Q

prognosis of HL

A

good chance of cure

99
Q

age peak of NHL

A

unimodal
75-85

100
Q

most common subtype of NHL

A

b cell > t cell

101
Q

PC of NHL

A

B Sx or lymphadenopathy

102
Q

most common indolent NHL

A

follicular

103
Q

name another indolent NHL that is similar to CLL but with disease in nodes

A

small lymphocytic lymphoma

104
Q

how do indolent NHL present

A

very large lymphadenopathy

105
Q

risk of indolent lymphoma

A

transformation to high grade

106
Q

what is seen on lymph node biopsy of follicular lymphoma

A

large number of centroblasts

107
Q

Tx of follicular lymphoma

A

watchful waiting unless high burden of disease

108
Q

cause of follicular lymphoma

A

t(14;18) causing fusion of BCL2 gene

109
Q

how does the course of indolent lymphomas vary from high grade

A

indolent - often have relapsing remitting
high grade - more aggressive but once cured, they won’t come back

110
Q

most common high grade lymphoma

A

diffuse large b cell

111
Q

PC of diffuse large b cell lymphoma + one association

A

lymphadenopathy, B Sx +/- BM involvement
can be associated with EBV

112
Q

risk of Tx of diffuse large b cell lymphoma

A

tumour lysis syndrome

113
Q

name another high grade lymphoma

A

mantle cell (may have leukaemic phase)

114
Q

cause of mantle cell lymphoma

A

t(11;14) –> overexpression of cyclin d1

115
Q

Tx of mantle cell lymphoma

A

chemo

116
Q

blood film of mantle cell lymphoma

A

cleft in mantle cell - nucleus looks like a heart

117
Q

tx of diffuse large b cell

A

R-CHOP (rituximab-CHOP) chemo

118
Q

name the most common very high grade lymphoma

A

burkitts

119
Q

PC of burkitts

A

very fast growing lump in lymph nodes of neck / abdo

120
Q

risk of tx with burkitts

A

tumour lysis syndrome ++

121
Q

histology buzzword of burkitts

A

starry sky apperance
(bright white pearly vacuoles in blue cells)

122
Q

associations of burkitts

A

EBV / HIV
t(8;14)

123
Q

who gets burkitts

A

younger adults

124
Q

2 examples of T cell lymphoma with a common feature of each

A

ATLL (adult t cell lymphoma) - flower cells on blood film
cutaneous T cell lymphoma - rare, PC = weird rashes

125
Q

who gets ATLL

A

ppl from far east

126
Q

tumour lysis syndrome results

A

high creatinine / urea
high K
high PO43
high uric acid
low Ca

127
Q

Tx of tumour lysis syndrome

A

rasburicase
renal replacement therapy

128
Q

medication to prevent tumour lysis sydrome

A

allopurinol
rasburicase if high risk

129
Q

how does rasburicase work in TLS

A

depletes uric acid levels

130
Q

what is multiple myeloma

A

clonal population of plasma cells which proliferate and produce monoclonal Ig light chains

131
Q

diagnostic finding of multiple myeloma in blood / urine (buzzwords)

A

blood = paraprotein
urine = bence jones protein

132
Q

describe the progression to myeloma

A

MGUS –> MM
MGUS –> smouldering myelome –> MM

133
Q

diagnosis criteria of myeloma

A

clonal BM plasma cells >10% in marrow or plasmacytoma
PLUS
end organ damage - indicated by 1+ of CRABS:
Calcium >2.75
Renal - creatinine >177 / creatinine clearance <40ml/min
Anaemia - Hb <100
Bone lesions - lytic lesions
SLIM - >60% plasma cells in BM / light chain ratio >100, MRI lesions

134
Q

what is smouldering myeloma

A

aka asymptomatic myeloma
serum monoclonal protein (IgG or IgA) >30g/L OR bence jones protein
AND / OR
clonal BM plasma cells 10-60% in marrow

+ NO end organ damage !!

135
Q

progression of smouldering myeloma

A

most will progress to myeloma if untreated
no point treating them tho unless they become myeloma

136
Q

what does MGUS stand for

A

monoclonal gammopathy of unknown significance

137
Q

features of MGUS

A

serum monoclonal protein <30g/L
plasma cells <10% in BM
no end organ damage
no evidence of another disorder

138
Q

what % of MGUS progress to myeloma every year

A

1-2%

139
Q

who gets MGUS

A

very common in elderly

140
Q

what % of SM progress to myeloma every year

A

10%

141
Q

paraprotein level of MGUS / SM / MM

A

MGUS <30
SM >30
MM = ANY but usually >30

142
Q

plasma cell % in BM of of MGUS / SM / MM

A

MGUS <10%
SM 10-60%
MM >10%

143
Q

which type of myeloma is the only to have CRAB Sx (end organ damage) and MRI lesions

A

MM

144
Q

free light chain ratio of of MGUS / SM / MM

A

MGUS <100
SM <100
MM >100

145
Q

what rare condition is similar to myeloma but with a few key differences

A

waldenstrom’s macroglobulinaemia

146
Q

key differences between waldenstrom’s macroglobulinaemia and myeloma

A

in waldenstroms:
- paraprotein is always IgM
- present with lymphadenopathy (similar to follicular lymphoma) and not CRAB Sx
- abnormality in lymphoplasmatoid cells as well as plasma cells
- high risk of hyperviscosity syndrome

147
Q

blood film features of myeloma

A

rouleaux - stacking of RBCs
plasma cells in peripheral blood

148
Q

Mx of MGUS

A

annual blood test

149
Q

Mx of smouldering myeloma

A

3 monthly review to detect progression to myeloma

150
Q

Tx of myeloma inc older / younger / baseline tx

A

autologous stem cell transplant - esp in younger ppl
chemo - older ppl
initial tx - proteasome inhibitors, dexamethasone, thalidomide

151
Q

Ix for a well man with paraprotein >10

A

refer to haematology !
imaging to look for bone lesions
BM biopsy

152
Q

55M PC fatigue.
BTs - Anaemia
serum electrophoresis = IgG kappa paraprotein 37.
BM aspirate = clonal population of plasma cells, 15% of marrow cells
Dx?

A

multiple myeloma
- if paraprotein >30, then it can only be SM or MM.
- Presence of anaemia (CRAB Sx) = only MM.

153
Q

55M 2/52 fatigue and easy bruising.
BTs - high WCC, anaemia, low plts.
blood film - blasts, 27% blasts in BM
flow cytometry - clonal population of cells CD34+, CD19+, TdT+
cytogenetic analysis - t(9;22)
Dx?

A

ALL
- >20% blasts in BM = acute leukaemia.
- CD19+ = B cell marker. TdT = B cell pre-cursor marker
- t(9;22) is CML or ALL

154
Q

25M drenching night sweats.
BTs - normal ish
LN biopsy - multinucleated cell, very large.
Dx?

A

HL
- multinucleated cell = reed sternburg cell

155
Q

50F splenomegaly.
high WCC
blood film = left shifted granulocytes.
FISH = BCR-ABL1 fusion gene
Dx?

A

CML
- splenogemaly = CML or myelofibrosis or leishmaniasis
- high WCC = more likely CML or myelofibrosis
- left shift = favours CML
- BCR-ABL1 = diagnostic of CML