MedEd Haem 1 Flashcards
(155 cards)
1
Q
name myeloid cells
A
RBCs
mast cells
basophils / neutrophils / eosinophils
platelets
2
Q
PC of acute leukaemias
A
anaemia
thrombocytopaenia
neutropenia
(pancytopaenia)
bone pain
fever
3
Q
which type of leukaemia has splenomegaly
A
chronic > acute
4
Q
PC of chronic leukaemias
A
lymphadenopathy
splenomegaly
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
6
Q
what is the leukaemia emergency
A
leukostasis
7
Q
what is leukostasis
A
EMERGENCY
high WCC –> increased viscosity of blood –> end organ damage
8
Q
PC of leukostasis
A
retinopathy
pulmonary infiltrates
bleeding
thrombosis
9
Q
Tx of leukostasis
A
leukaphoresis
chemo
steroids
10
Q
which type of leukaemia is leukostasis common / uncommon
A
AML common
then ALL / CML
CLL unusual
11
Q
in what type of leukaemia can pts be asymptomatic
A
CLL
12
Q
classical Hx of ALL
A
2-5 y/o child
hepatosplenomegaly
bone pain / limp
fevers
13
Q
what % of pts have CNS disease in ALL
A
10%
14
Q
what sign is rare but very specific to ALL in boys
A
testicular swelling
15
Q
how do adults present with ALL
A
similar to AML
lymphadenopathy
16
Q
BTs and results of ALL
A
FBC
thrombocytopaenia
anaemia
high WCC but low neutrophils
film - blasts in peripheral blood
17
Q
can circulating blast cells be normal
A
NO - indicates leukaemia or a few other conditions
18
Q
features of blast cells on blood film
A
high nucelus:cytoplasm ratio
larger than RBCs (normal lymphocytes are same size as RBCs)
19
Q
what cell shape is indicative of ALL vs AML
A
‘hand mirror’/’table tennis bat’ shaped
20
Q
how is ALL Dx
A
BM biopsy
flow cytometry
(hard to tell AML from ALL on blood film)
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)
22
Q
what genetic mutation causes ALL in 20-30% of adults
A
BCR-ABL1 t(9;22)
(also causes CML)
23
Q
Tx of ALL
A
chemo
transplant of BM
24
Q
RFs for AML
A
incidence increasing with age
previous history of myelodysplastic syndrome / chemo
25
BTs and results of AML
FBC - anaemia, thrombocytopaenia, high WCC, low neutrophils
film - high blasts
normal or high INR
26
blood film of AML
auer rods (stick in the lymphocytes)
27
what % of AML has auer rods
20-30%
28
flow cytometry markers present in AML
MPO CD34
CD13 / 33 / 117
(xs detail but may come up)
29
Tx of AML
chemo - shorter duration than ALL
30
PC of APML (acute promyelocytic leukaemia)
DIC - inc intracranial bleeding
good prognosis after induction
31
Tx of APML
ATRA (vitamin A)
all trans retinoic acid
32
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
33
what are myeloproliferative neoplasms (MPNs)
increased production of myeloid lineage
34
what can cause a high platelet count (in order of most common)
acute infection
chronic inflammatiom
malignancy (5-10%)
essential thrombocytopaenia
polycythaemia rubra vera
35
what is ET
platelet count >450 consistently with no other cause
36
complications of ET
thrombotic events arterial / venous
small risk transformation to myelofibrosis / AML
37
what mutations are present in ET
JAK2 55%
CALR / MPL in most other pts
38
Tx of ET
aspirin - reduce stroke risk
hydroxycarbamide - lower platelets
39
what is PCV
polycthaemia rubra vera
overproduction of RBCs
40
Hct levels of PCV in men / women
men >0.52
women >0.48
41
complications of PCV
high risk thrombotic events eg stroke
Budd Chiari (hepatic vein thrombosis)
risk of transformation to MF / AML
42
what mutation do 95% of PCV pts have
JAK2
43
TX of PCV
aspirin - reduce stroke risk
venesection - lower Hct
hydroxycarbamide - lower plts
44
causes of high HCt
PCRV
altitude
chronic hypoxia - severe COPD / cyantoic heart disease / OSA
erythropoeitin secreting renal cancers
45
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.
46
complications of myelofibrosis
risk of transformation to AML
47
what conditions can develop into myelofibrosis
ET
PCV
48
mutations causing myelofibrosis
JAK2 50%
CALR
49
2 buzzwords for myelofibrosis
'dry tap' on BM aspirate
'tear drop poikilocytes' on blood film
50
Tx of myelofibrosis
stem cell transplant
ruloxitinib - JAK inhibitor
51
name 4 myeloproliferative neoplasms
essential thrombocytopaenia
polycythaemia vera
myelofibrosis
CML
52
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%)
53
3 key causes of splenogemaly
CML
myelofibrosis
leishmaniasis
54
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)
55
other causes of high basophil count
worry about CML !! not much other causes
56
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
57
another cause of increase precursor cells on film
severe acute infections
58
elevated basophil and eosinophils
CML !!
59
blood film features of CML
left shift (more precursors)
leukocytosis
eosinophils
basophilia
hypoblasted megakaryocytes
60
how is CML Dx
almost all with philadelphia chromosome (BCR - ABL1 t(9;22)) using FISH
61
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
62
Tx of CML
tyrosine kinase inhibitors (eg imatinib)
63
survival of CML
>90% 10 year survival
64
PC of CLL
asymptomatic
high WCC
>50 y/o M>F
some have small lymphadenopathy / splenomegaly
some present with ITP / haemolytic anaemia
65
BTs of CLL and results
FBC - normal Hb, high WCC usually >100, normal plts, normal neutrophils, high lymphocyte count
66
what does anaemia at presentation in CLL indicate
more aggressive disease or haemolytic anaemia
67
blood film of CLL
smear / smudge cells - BUZZWORD
lymphocytosis
68
Dx of CLL
flow cytometry
- immunophenotyping for clonal population of cells
- same cell markers eg kappa/lamba light chains
69
what disease has same pathology as CLL but different distribution
small lymphocytic lymphoma
- blood / marrow vs lymph nodes
70
how is CLL staged and how does this determine Tx
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
what aggressive condition can CLL transform into
Richters syndrome
72
Tx of CLL and what does it depend on
if IGHV mutated = rituximab + chemo eg FCR
if TP53 mutated = BTK inhibitors eg ibrutinib
can use venetoclax (BCL2 inhibitor)
73
causes of pancytopaenia
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
what blast % would diagnose acute leukaemia
>20%
75
what is myelodysplastic syndrome
dysplatic changes (abnormal cells) in 1 or more myeloid cell lines (erythroid, megakaryocytes, granulocytes)
76
PC of myelodysplastic syndrome
asymptomatic
incidental cytopaenia
77
what can myelodysplastic syndrome progress to
AML
78
characterise the anaemia in MDS
macrocytic anaemia
normal b12 / folate levels
79
blood film of MDS - buzzwords
pseudo-pelger anomaly
bilobed neutrophils
ring sideroblasts
80
4 prognostic factors for MDS
number /extent of cytopaenias
BM blast % (>20 = AML)
cytogenetic changes
specific mutations eg TP53 (poor)
81
what % of MDS progress to AML
30%
82
how is risk of progression to AML from MDS assessed
IPSS-R / IPSS-M
83
Tx of MDS
higher risk - chemo
younger pts - stem cell transplant
84
contrast age peak of HL and NHL
HL = young
NHL = increases with age
85
contrast lymph node involvement in HL and NHL
HL = mediastinum mainly. painful on drinking alcohol
NHL = anywhere
86
contrast course and prognosis of HL and NHL
HL = aggressive but mostly curable
NHL = variable course and prognosis
87
roughly how are lymphomas categorised
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
most common subtypes of lymphomas
NHL > HL
B cell > T cell
89
which types of lymphomas are low grade
follicular B cell
90
which types of lymphomas are high grade
diffuse large b cell lymphoma
burkitt's (++++ high grade)
91
staging of lymphoma
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
age peaks of HL
bimodal
mainly <30 but also peak in 70s
93
PC of HL
B Sx with lymphadenopathy (mediastinal, worse after alcohol)
94
Dx feature of HL on blood film
reed - sternburg cells (BUZZWORD)
- large cell with 2+ nucleus
- may only see 1 per LN, still diagnostic
95
most common type of HL
nodular sclerosing
96
what is HL associated with
EBV
97
Tx of HL
ABVD chemo + radio
stem cell transplant if Tx fails
98
prognosis of HL
good chance of cure
99
age peak of NHL
unimodal
75-85
100
most common subtype of NHL
b cell > t cell
101
PC of NHL
B Sx or lymphadenopathy
102
most common indolent NHL
follicular
103
name another indolent NHL that is similar to CLL but with disease in nodes
small lymphocytic lymphoma
104
how do indolent NHL present
very large lymphadenopathy
105
risk of indolent lymphoma
transformation to high grade
106
what is seen on lymph node biopsy of follicular lymphoma
large number of centroblasts
107
Tx of follicular lymphoma
watchful waiting unless high burden of disease
108
cause of follicular lymphoma
t(14;18) causing fusion of BCL2 gene
109
how does the course of indolent lymphomas vary from high grade
indolent - often have relapsing remitting
high grade - more aggressive but once cured, they won't come back
110
most common high grade lymphoma
diffuse large b cell
111
PC of diffuse large b cell lymphoma
lymphadenopathy, B Sx +/- BM involvement
can be associated with EBV
112
risk of Tx of diffuse large b cell lymphoma
tumour lysis syndrome
113
name another high grade lymphoma
mantle cell (may have leukaemic phase)
114
cause of mantle cell lymphoma
t(11;14) --> overexpression of cyclin d1
115
Tx of mantle cell lymphoma
chemo
116
blood film of mantle cell lymphoma
cleft in mantle cell - nucleus looks like a heart
117
tx of diffuse large b cell
R-CHOP (rituximab-CHOP) chemo
118
name the most common very high grade lymphoma
burkitts
119
PC of burkitts
very fast growing lump in lymph nodes of neck / abdo
120
risk of tx with burkitts
tumour lysis syndrome ++
121
histology buzzword of burkitts
starry sky apperance
(bright white pearly vacuoles in blue cells)
122
associations of burkitts
EBV / HIV
t(8;14)
123
who gets burkitts
younger adults
124
2 examples of T cell lymphoma with a common feature of each
ATLL (adult t cell lymphoma) - flower cells on blood film
cutaneous T cell lymphoma - rare, PC = weird rashes
125
who gets ATLL
ppl from far east
126
tumour lysis syndrome results
high creatinine / urea
high K
high PO43
high uric acid
low Ca
127
Tx of tumour lysis syndrome
rasburicase
renal replacement therapy
128
medication to prevent tumour lysis sydrome
allopurinol
rasburicase if high risk
129
how does rasburicase work in TLS
depletes uric acid levels
130
what is multiple myeloma
clonal population of plasma cells which proliferate and produce monoclonal Ig light chains
131
diagnostic finding of multiple myeloma in blood / urine (buzzwords)
blood = paraprotein
urine = bence jones protein
132
describe the progression to myeloma
MGUS --> MM
MGUS --> smouldering myelome --> MM
133
diagnosis criteria of myeloma
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
what is smouldering myeloma
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
progression of smouldering myeloma
most will progress to myeloma if untreated
no point treating them tho unless they become myeloma
136
what does MGUS stand for
monoclonal gammopathy of unknown significance
137
features of MGUS
serum monoclonal protein <30g/L
plasma cells <10% in BM
no end organ damage
no evidence of another disorder
138
what % of MGUS progress to myeloma every year
1-2%
139
who gets MGUS
very common in elderly
140
what % of SM progress to myeloma every year
10%
141
paraprotein level of MGUS / SM / MM
MGUS <30
SM >30
MM = ANY but usually >30
142
plasma cell % in BM of of MGUS / SM / MM
MGUS <10%
SM 10-60%
MM >10%
143
which type of myeloma is the only to have CRAB Sx (end organ damage) and MRI lesions
MM
144
free light chain ratio of of MGUS / SM / MM
MGUS <100
SM <100
MM >100
145
what rare condition is similar to myeloma but with a few key differences
waldenstrom's macroglobulinaemia
146
key differences between waldenstrom's macroglobulinaemia and myeloma
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
blood film features of myeloma
rouleaux - stacking of RBCs
plasma cells in peripheral blood
148
Mx of MGUS
annual blood test
149
Mx of smouldering myeloma
3 monthly review to detect progression to myeloma
150
Tx of myeloma
autologous stem cell transplant - esp in younger ppl
chemo - older ppl
initial tx - proteasome inhibitors, dexamethasone, thalidomide
151
Ix for a well man with paraprotein >10
refer to haematology !
imaging to look for bone lesions
BM biopsy
152
55M PC fatigue.
BTs - Anaemia
serum electrophoresis = IgG kappa paraprotein 37.
BM aspirate = clonal population of plasma cells, 15% of marrow cells
Dx?
multiple myeloma
- if paraprotein >30, then it can only be SM or MM.
- Presence of anaemia (CRAB Sx) = only MM.
153
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?
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
25M drenching night sweats.
BTs - normal ish
LN biopsy - multinucleated cell, very large.
Dx?
HL
- multinucleated cell = reed sternburg cell
155
50F splenomegaly.
high WCC
blood film = left shifted granulocytes.
FISH = BCR-ABL1 fusion gene
Dx?
CML
- splenogemaly = CML or myelofibrosis or leishmaniasis
- high WCC = more likely CML or myelofibrosis
- left shift = favours CML
- BCR-ABL1 = diagnostic of CML
