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White cell disease Flashcards

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1
Q

Myeloperoxidase (MPO) is a marker of…

A

AML

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2
Q

PML-RARA

A

APL

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3
Q

t(15;17)

A

APL (PML-RARA)

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4
Q

Which FAB classification does APL belong to?

A

AML-M3

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5
Q

Pathogenesis and management of APL differentiation syndrome

A

Pathogenesis: release of inflammatory cytokines from malignant promyelocytes after ATRA –> capillary leak
Management: Dexamethasone, restart ATRA after resolution

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6
Q

Myeloblast vs Lymphoblast (5)

A

relatively low N:C ratio; very high
fine granules; agranular
fine chromatin; clumped
prominent nucleoli; indistinct
Auer rods

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7
Q

Cytogenetic studies for prognosis of ALL (3)

A

t(9;22) (poor)
t(12;21) (good)
hyperdiploidy (good)

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8
Q

Diagnostic criteria for Polycythaemia vera

A

Major:
- erythrocytosis
1. Hb > 18.5 / 16
2. Hct >49%
3. ↑ red cell mass
- BM briopsy: hyperceullar with trilineage growth
- JAK2
Minor:
- subnormal EPO

all majors, or major 1+2 + minor

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9
Q

Diagnostic criteria for essential thrombocytopenia

A
  • Plt >450
  • BM biopsy: megakaryocytic proliferation
  • JAK2 / CALR / MPL
  • ⨉ other MPN
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10
Q

Diagnostic criteria for PMF

A

Major:
- BM biopsy: reticulin +/- collagen fibrosis
- BM biopsy: megakaryocytic proliferation & atypia
- JAK2 / CALR / MPL
- ⨉ other MPN
Minor:
- leucoerythroblastosis
- ↑ serum LDH
- anaemia
- splenomegaly

all majors + 1 minor

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11
Q

Which MPN can directly transform into AML? (3)

A

CML, PV, PMF

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12
Q

t(9;22)

A

Philadelphia (Ph) chromosome / BCR-ABL1
CML

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13
Q

3 phases of CML (blast %)

A
  1. Chronic phase (blast <10%)
  2. Accelerated phase (blast 10~19%)
  3. Blast crisis (blast >=20%)
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14
Q

TKI for CML (1+3+1)

A

1st generation: Imatinib
2nd generation: Nilotinib 尼洛, Dasatinib 達沙, Bosutinib 博舒
3rd generation: Ponatinib 普納

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15
Q

Role of treatment of Ponatinib in CML

A

for T315I mutation

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16
Q

Role of treatment of hydroxyurea in CML

A

cytoreduction while awaiting confirmation of diagnosis

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17
Q

3 types of responses in treatment of CML

A
  1. Complete haematological response
  2. Complete cytogenetic response
  3. Molecular response
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18
Q

Major molecular response definition

A

3 log reduction (of BCR-ABL1 transcript) compared to international scale

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19
Q

Management for PV (4)

A

aspirin, venesection
[high risk] hydroxyurea, ruxolitinib

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20
Q

Management for ET (2)

A

aspirin, anagrelide

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21
Q

Assessment for PMF

A

DIPSS Plus scoring

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22
Q

Cytogenetics studies for prognosis of MDS

A

del 5q (good)

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23
Q

Risk stratification of MDS (4 parameters)

A

IPSS
1. dysplasia (single / multiple lineage)
2. % of blasts
3. del 5q
4. % of sideroblasts

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24
Q

PIG-A mutation

A

Paroxysmal nocturnal haemoglobinuria

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25
BRAF V600E
Hairy cell leukaemia
26
Which haematological malignancy is associated with HTLV-1?
Adult T-cell leukaemia / lymphoma (ATLL)
27
Which haematological malignancy is associated with TCR rearrangement?
Adult T-cell leukaemia / lymphoma (ATLL)
28
Classification of Hodgkin lymphoma (4+1)
1. Classical (CD30+) a. Nodular sclerosing b. Mixed cellularity c. Lymphocyte rich d. Lymphocyte depleted 2. Nodular lymphocyte predominant HL
29
Staging for Hodgkin lymphoma
Ann Arbor staging
30
t(14;18) (associated protein)
Follicular lymphoma ↑ Bcl-2
31
t(11;14) (associated protein)
Mantle cell lymphoma ↑ cyclin D1
32
MYD88
Lymphoplasmacytic lymphoma
33
t(8;14) / IGH-MYC fusion
Burkitt lymphoma
34
t(2;5) / NPM1-ALK fusion
Anaplastic T-cell lymphoma
35
Which blood cancer comes with DIC?
APL
36
Which blood cancer often comes with monocytopenia?
Hairy cell leukaemia
37
Which blood cancer often comes with gum hypertrophy?
AML-M5 / AMoL
38
Which blood cancer comes often with CNS involvement?
ALL
39
Which blood cancer comes often with mediastinal involvement (2)?
T-ALL, nodular sclerosing classical HL
40
Which blood cancer often comes with serous effusion?
T-PLL
41
Which blood cancer often comes with skin involvement?
ATLL
42
Which blood cancer often comes with hypercalcaemia and bone lesions? (2)
ATLL, MM
43
Which blood cancer often comes with oropharyngeal involvement?
non-Hodgkin lymphoma
44
Which blood cancer often comes with massive splenomegaly? (3)
CMF, PMF, Hairy cell leukaemia
45
Which blood cancer often comes with plethora and erythromelalgia (2)?
PV, ET
46
Which blood cancers are associated with significant thrombotic and bleeding risk?
PV, ET
47
DDx of Lymphadenopathy (5)
(MIAMI) Malignancies: lymphoma, metastasis Infection Autoimmune diseases Miscellaneous: storage diseases (e.g. Gaucher disease) Iatrogenic: drugs
48
DDx of pancytopenia (3+1)
1. BM disorders a. Malignancy: MDS, AA, PNH b. Infiltration: leukaemia, lymphoma, carcinoma, TB c. Iatrogenic: chemotherapy, RT, chloramphenicols 2. Hypersplenism
49
DDx of erythrocytosis (1+1+3)
1. Pseudoerythrocytosis: dehydration, burns, stress... 2. Primary: PV 3. Secondary: a. persistent hypoxia b. renal hypoxia c. ectopic EPO production
50
DDx of thrombocytosis (1+3)
1. Primary: MPN 2. Secondary: a. anaemia / blood loss b. inflammation (inc. infection, malignancy, autoimmune diseases) c. post-splenectomy
51
Flow cytometer components (3)
1. Fluidics system 2. Optical system 3. Computer / Electronic system
52
Functions of forward scatter, side scatter and gating in flow cytometry respectively
FS: assess cell size SS: assess granularity Gating: isolating single populations of interest within one sample
53
Immunophenotyping: markers for haematological progenitor cells (4)
CD34, CD45, TdT, HLA-DR
54
Immunophenotyping: myeloid markers (4)
MPO, CD13, CD33, CD117 (13 ⨉ 3 ⨉ 3=117)
55
Immunophenotyping: B-lymphoid markers (5)
CD10, 19, 20, 22, 79a
56
Immunophenotyping: T-lymphoid markers
CD2, cCD3, CD5, CD7
57
Blood cancer with CD20-, CD138+
Multiple myeloma
58
Blood cancer with CD5+, CD23+
CLL
59
Blood cancer with TRAP+
Hairy cell leukaemia
60
Blood cancer with CD7-, CD25++
ATLL
61
Blood cancer with CD30+, CD15+
Classical Hodgkin lymphoma
62
Lymphoma with CD30-, CD20+
Non-classical Hodgkin lymphoma
63
Lymphoma with CD10+
FL, BL
64
Lymphoma with CD3+, CD30+, ALK+
Anaplastic T-cell lymphoma
65
Acute leukaemia definition
>=20% blast in BM
66
Which acute leukaemia is associated with Down syndrome?
AML-M7 (acute megakaryoblastic leukaemia)
67
Classification scheme of acute leukaemia (2)
1. WHO 2. FAB
68
Investigation findings for AML
Peripheral blood film: myeloblasts BM aspirate & trephine biopsy: hypercellularity Immunophenotyping Cytogenetics: PML-RARA / t(15;17)
69
Molecular studies for prognosis in AML (3)
FLT3-ITD (poor) NPM1 (good) CEBPA (good)
70
Chemotherapy for AML
1. Remission induction: Cytarabine (7d) + Anthracyclin (3d) 2. Consolidation
71
Management for APL
All-trans-retinoic acid (ATRA), Arsenic trioxide, Ascorbic acid
72
Chemotherapy steps for ALL
remission induction + consolidation + maintenance
73
Investigation findings for CML
CBC: WCC >200, basophilia Peripheral blood film: complete spectrum of immature white cells BM trephine biopsy: - hypercellularity, with granulopoietic dominance - ↑ hypolobated megakaryocytes Cytogenetics: Ph+ / BCR-ABL1 fusion
74
Investigation findings for MDS
CBC: pancytopenia, ↑ RDW BM aspirate: - blast cells - ring sideroblasts... - pseudo-Pelger Huet anomaly... (hypolobuted, hypogranulated, hypersegmented) - hypolobulated megakaryocytes BM trephine biopsy: hypercellularity
75
Congenital aplastic anaemia (2+1)
Multi-lineage: Fanconi's anaemia, Dyskeratosis congenita Single-lineage: Diamond-Blackfan syndrome
76
Aetiology of secondary aplastic anaemia
Iatrogenic: chemotherapy, radiation, drugs Industrial: benzene Infectious Immune: SLE
77
Pathogenesis of paroxysmal nocturnal haemoglobinuria
PIG-A mutation --> ↓ GPI anchor --> CD55, CD59 cannot link to red cells --> complement attack --> intravascular haemolysis
78
Diagnosis of paroxysmal nocturnal haemoglobinuria
Flow cytometry
79
Management for paroxysmal nocturnal haemoglobinuria
Eculizumab (Anti-C5 : “E””C”ulizumab)
80
Investigation findings for CLL
WCC: monoclonal B cells Peripheral blood film: small lymphocytes, smear cells, smudge cells BM aspirate: lymphocytosis Immunophenotyping: CD5+, CD23+, light chain restriction
81
Genetics studies for prognosis of CLL (3)
del 13q14 (good) trisomy 12 (intermediate) del 17p / TP53, del 11q23 / ATM (poor)
82
Staging methods for CLL (2)
1. Binet staging (A~C) 2. RAI staging (0~IV)
83
Management for CLL
asymptomatic: watchful waiting symptomatic: chemotherapy +/- targeted therapy
84
Targeted therapy for CLL (3)
Rituximab, Ibrutinib, Venetoclax
85
Name for the transformation from CLL to (...)
Richter transformation (DLBCL)
86
Hodgkin's lymphoma vs Non-Hodgkin's lymphoma (prevalence, stage during presentation, spread, extranodal diseases)
Prevalence: (1:9) Stage: early; later Spread: contiguous nodal groups; remote Extranodal diseases: fewer; more
87
MC lymphadenopathy in lymphoma (Describe the features of the lymph nodes)
cervical non-tender, multible, rubbery, asymmetrical
88
Ann Arbor staging
1. single LN region 2. >1 regions, same side of diaphragm 3. both sides of diaphragm 4. >= 1 extra-lymphatic organs
89
MC blood cancer
Multiple myeloma
90
Classifications of plasma cell dyscrasia (3)
1. MGUS (serum M-protein <30 and BM plasma cells <10%) 2. Smouldering myeloma 3. Multiple myeloma (BM plasma cells >10% and >=1 CRAB features)
91
Specific investigations for multiple myeloma (serum 3 + urine)
Serum protein electrophoresis Serum immunofixation electrophoresis Serum FLC ratio Bence Jones proteins
92
MC M proteins
IgG (>IgA > FLC)
93
Prognostic studies for multiple myeloma (1+3)
Good: hyperdiploidy Poor: del 13q, ↑ serum albumin, ↑ serum β2-microglobulin
94
Which white cell disease shows raindrop skull?
Multiple myeloma
95
Describe Reed Sternberg cells
large atypical lymphoid cells with Owl-eye appearance of nuclei & inclusion-like nucleoli

MED4 ACP (13 decks)

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