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MED4 ACP > White cell disease > Flashcards

White cell disease Flashcards

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
Q

BRAF V600E

A

Hairy cell leukaemia

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26
Q

Which haematological malignancy is associated with HTLV-1?

A

Adult T-cell leukaemia / lymphoma (ATLL)

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27
Q

Which haematological malignancy is associated with TCR rearrangement?

A

Adult T-cell leukaemia / lymphoma (ATLL)

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28
Q

Classification of Hodgkin lymphoma (4+1)

A
  1. Classical (CD30+)
    a. Nodular sclerosing
    b. Mixed cellularity
    c. Lymphocyte rich
    d. Lymphocyte depleted
  2. Nodular lymphocyte predominant HL
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29
Q

Staging for Hodgkin lymphoma

A

Ann Arbor staging

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30
Q

t(14;18) (associated protein)

A

Follicular lymphoma
↑ Bcl-2

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31
Q

t(11;14) (associated protein)

A

Mantle cell lymphoma
↑ cyclin D1

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32
Q

MYD88

A

Lymphoplasmacytic lymphoma

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33
Q

t(8;14) / IGH-MYC fusion

A

Burkitt lymphoma

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34
Q

t(2;5) / NPM1-ALK fusion

A

Anaplastic T-cell lymphoma

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35
Q

Which blood cancer comes with DIC?

A

APL

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36
Q

Which blood cancer often comes with monocytopenia?

A

Hairy cell leukaemia

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37
Q

Which blood cancer often comes with gum hypertrophy?

A

AML-M5 / AMoL

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38
Q

Which blood cancer comes often with CNS involvement?

A

ALL

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39
Q

Which blood cancer comes often with mediastinal involvement (2)?

A

T-ALL, nodular sclerosing classical HL

40
Q

Which blood cancer often comes with serous effusion?

A

T-PLL

41
Q

Which blood cancer often comes with skin involvement?

A

ATLL

42
Q

Which blood cancer often comes with hypercalcaemia and bone lesions? (2)

A

ATLL, MM

43
Q

Which blood cancer often comes with oropharyngeal involvement?

A

non-Hodgkin lymphoma

44
Q

Which blood cancer often comes with massive splenomegaly? (3)

A

CMF, PMF, Hairy cell leukaemia

45
Q

Which blood cancer often comes with plethora and erythromelalgia (2)?

A

PV, ET

46
Q

Which blood cancers are associated with significant thrombotic and bleeding risk?

A

PV, ET

47
Q

DDx of Lymphadenopathy (5)

A

(MIAMI)
Malignancies: lymphoma, metastasis
Infection
Autoimmune diseases
Miscellaneous: storage diseases (e.g. Gaucher disease)
Iatrogenic: drugs

48
Q

DDx of pancytopenia (3+1)

A
  1. BM disorders
    a. Malignancy: MDS, AA, PNH
    b. Infiltration: leukaemia, lymphoma, carcinoma, TB
    c. Iatrogenic: chemotherapy, RT, chloramphenicols
  2. Hypersplenism
49
Q

DDx of erythrocytosis (1+1+3)

A
  1. Pseudoerythrocytosis: dehydration, burns, stress…
  2. Primary: PV
  3. Secondary:
    a. persistent hypoxia
    b. renal hypoxia
    c. ectopic EPO production
50
Q

DDx of thrombocytosis (1+3)

A
  1. Primary: MPN
  2. Secondary:
    a. anaemia / blood loss
    b. inflammation (inc. infection, malignancy, autoimmune diseases)
    c. post-splenectomy
51
Q

Flow cytometer components (3)

A
  1. Fluidics system
  2. Optical system
  3. Computer / Electronic system
52
Q

Functions of forward scatter, side scatter and gating in flow cytometry respectively

A

FS: assess cell size
SS: assess granularity
Gating: isolating single populations of interest within one sample

53
Q

Immunophenotyping: markers for haematological progenitor cells (4)

A

CD34, CD45, TdT, HLA-DR

54
Q

Immunophenotyping: myeloid markers (4)

A

MPO, CD13, CD33, CD117

(13 ⨉ 3 ⨉ 3=117)

55
Q

Immunophenotyping: B-lymphoid markers (5)

A

CD10, 19, 20, 22, 79a

56
Q

Immunophenotyping: T-lymphoid markers

A

CD2, cCD3, CD5, CD7

57
Q

Blood cancer with CD20-, CD138+

A

Multiple myeloma

58
Q

Blood cancer with CD5+, CD23+

A

CLL

59
Q

Blood cancer with TRAP+

A

Hairy cell leukaemia

60
Q

Blood cancer with CD7-, CD25++

A

ATLL

61
Q

Blood cancer with CD30+, CD15+

A

Classical Hodgkin lymphoma

62
Q

Lymphoma with CD30-, CD20+

A

Non-classical Hodgkin lymphoma

63
Q

Lymphoma with CD10+

A

FL, BL

64
Q

Lymphoma with CD3+, CD30+, ALK+

A

Anaplastic T-cell lymphoma

65
Q

Acute leukaemia definition

A

> =20% blast in BM

66
Q

Which acute leukaemia is associated with Down syndrome?

A

AML-M7 (acute megakaryoblastic leukaemia)

67
Q

Classification scheme of acute leukaemia (2)

A
  1. WHO
  2. FAB
68
Q

Investigation findings for AML

A

Peripheral blood film: myeloblasts
BM aspirate & trephine biopsy: hypercellularity
Immunophenotyping
Cytogenetics: PML-RARA / t(15;17)

69
Q

Molecular studies for prognosis in AML (3)

A

FLT3-ITD (poor)
NPM1 (good)
CEBPA (good)

70
Q

Chemotherapy for AML

A
  1. Remission induction: Cytarabine (7d) + Anthracyclin (3d)
  2. Consolidation
71
Q

Management for APL

A

All-trans-retinoic acid (ATRA), Arsenic trioxide, Ascorbic acid

72
Q

Chemotherapy steps for ALL

A

remission induction + consolidation + maintenance

73
Q

Investigation findings for CML

A

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
Q

Investigation findings for MDS

A

CBC: pancytopenia, ↑ RDW
BM aspirate:
- blast cells
- ring sideroblasts…
- pseudo-Pelger Huet anomaly… (hypolobuted, hypogranulated, hypersegmented)
- hypolobulated megakaryocytes
BM trephine biopsy: hypercellularity

75
Q

Congenital aplastic anaemia (2+1)

A

Multi-lineage: Fanconi’s anaemia, Dyskeratosis congenita
Single-lineage: Diamond-Blackfan syndrome

76
Q

Aetiology of secondary aplastic anaemia

A

Iatrogenic: chemotherapy, radiation, drugs
Industrial: benzene
Infectious
Immune: SLE

77
Q

Pathogenesis of paroxysmal nocturnal haemoglobinuria

A

PIG-A mutation –> ↓ GPI anchor –> CD55, CD59 cannot link to red cells –> complement attack –> intravascular haemolysis

78
Q

Diagnosis of paroxysmal nocturnal haemoglobinuria

A

Flow cytometry

79
Q

Management for paroxysmal nocturnal haemoglobinuria

A

Eculizumab

(Anti-C5 : “E””C”ulizumab)

80
Q

Investigation findings for CLL

A

WCC: monoclonal B cells
Peripheral blood film: small lymphocytes, smear cells, smudge cells
BM aspirate: lymphocytosis
Immunophenotyping: CD5+, CD23+, light chain restriction

81
Q

Genetics studies for prognosis of CLL (3)

A

del 13q14 (good)
trisomy 12 (intermediate)
del 17p / TP53, del 11q23 / ATM (poor)

82
Q

Staging methods for CLL (2)

A
  1. Binet staging (A~C)
  2. RAI staging (0~IV)
83
Q

Management for CLL

A

asymptomatic: watchful waiting
symptomatic: chemotherapy +/- targeted therapy

84
Q

Targeted therapy for CLL (3)

A

Rituximab, Ibrutinib, Venetoclax

85
Q

Name for the transformation from CLL to (…)

A

Richter transformation
(DLBCL)

86
Q

Hodgkin’s lymphoma vs Non-Hodgkin’s lymphoma (prevalence, stage during presentation, spread, extranodal diseases)

A

Prevalence: (1:9)
Stage: early; later
Spread: contiguous nodal groups; remote
Extranodal diseases: fewer; more

87
Q

MC lymphadenopathy in lymphoma (Describe the features of the lymph nodes)

A

cervical
non-tender, multible, rubbery, asymmetrical

88
Q

Ann Arbor staging

A
  1. single LN region
  2. > 1 regions, same side of diaphragm
  3. both sides of diaphragm
  4. > = 1 extra-lymphatic organs
89
Q

MC blood cancer

A

Multiple myeloma

90
Q

Classifications of plasma cell dyscrasia (3)

A
  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
Q

Specific investigations for multiple myeloma (serum 3 + urine)

A

Serum protein electrophoresis
Serum immunofixation electrophoresis
Serum FLC ratio
Bence Jones proteins

92
Q

MC M proteins

A

IgG (>IgA > FLC)

93
Q

Prognostic studies for multiple myeloma (1+3)

A

Good: hyperdiploidy
Poor: del 13q, ↑ serum albumin, ↑ serum β2-microglobulin

94
Q

Which white cell disease shows raindrop skull?

A

Multiple myeloma

95
Q

Describe Reed Sternberg cells

A

large atypical lymphoid cells with Owl-eye appearance of nuclei & inclusion-like nucleoli

MED4 ACP (13 decks)

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