Haematology Flashcards

1
Q

Heinz bodies, bite and blister cells on film = ?

A

G6PD deficiency

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2
Q

Howell-Jolly bodies = ?

A

Post splenectomy

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3
Q

Smear/parachute/smudge cells on film = ?

A

CLL

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4
Q

Cerebriform cells on film = ?

A

Peripheral T cell lymphoma

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5
Q

Granular lymphocytes on film = ?

A

Large granular lymphocytic leukaemia

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6
Q

Tear drop red cells on film = ?

A

Myelofibrosis

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7
Q

Reed-Sternberg cells = ?

A

Hodgkin’s lymphoma

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8
Q

Starry sky on bone marrow biopsy = ?

A

Burkitt’s lymphoma

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9
Q

Dutcher bodies and flaming cells on bone marrow biopsy = ?

A

Myeloma

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10
Q

Normal T cell surface markers?

A

CD3/4/5/8

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11
Q

Normal B cell surface markers?

A

CD 19/20/kappa/lambda

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12
Q

Normal stem cell surface markers?

A

CD34

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13
Q

Normal monocyte cell surface markers?

A

CD14, CD64

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14
Q

Normal granulocytes cell surface markers?

A

CD13/15/33

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15
Q

Normal plasma cell surface markers?

A

CD138

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16
Q

Definition of MGUS?

A

Paraprotein < 30g/l, clonal plasma cells in BM < 10%, no myeloma defining events

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17
Q

RIsk factors for MGUS progression?

A

Paraprotein > 15g/L, non-IgG, FLC < 0.26 or > 1.65

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18
Q

Smoulder myeloma definition?

A

Paraprotein > 30g/L or > 0.5g/d in urine, BM plasma cells 10-60%, no myeloma defining events

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19
Q

Myeloma definition

A

Bone marrow plasma cells > 10% OR biopsy proven plasmacytoma + any CRAB features
OR if BM plasma cells > 60%, FLC ratio > 100, or 2 or more lesions > 5mm on MRI

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20
Q

Prognostic markers for myeloma (staging)?

A

Albumin < 35, B2-microglobulin > 5.5, LDH

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21
Q

Most common cause of renal failure in myeloma?

A

Cast nephropathy

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22
Q

Most common glomerular cause of renal failure in myeloma?

A

Amyloidosis

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23
Q

Treatment for myeloma if young and fit?

A

Bortezomib/len/dex triplets
Melphalan + autoSCT
Len maintenance

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24
Q

Treatment for myeloma if not fit for transplant

A

Bortez/len/dex trip/doublets

Other options - carfilzomib, pom, daratumumab

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25
Q

Side effects of proteasome inhibitors?

A

Bortezomib - neuropathy

Carfilzomib - cardiac

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26
Q

Side effects of immunomodulators?

A

Thalidomide - neuropathy
Lenalidomide - cytopaenia
Pom - cytopaenia

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27
Q

Mechanism of action of daratumumab?

A

anti-CD38

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28
Q

Common indications for autologous transplant?

A

Myeloma, lymphoma (mantle, T-cell, HL, NHL)

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29
Q

Diagnostic test for hereditary spherocytosis?

A

Flow cytometry showing reduced binding eosin-5 maleimide

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30
Q

Pathophysiology of myelofibrosis

A

Clonal proliferation of megakaryocytes, leading to fibroblast proliferation

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31
Q

Leukoerythroblastic blood film = ?

A

Myelofibrosis with nucleated and tear drop red cells

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32
Q

Bone marrow biopsy in MF?

A

Dry tap

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33
Q

Treatment of myelofibrosis

A

Ruxolitinib

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34
Q

What causes PNH?

A

Mutation in PIGA gene, leads to defective glycoprotein anchors for CD55/59, therefore uncontrolled complement activation

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35
Q

Consequences of PNH?

A

IV haemolysis, pancytopaenia, thrombosis

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36
Q

Diagnosis of PNH?

A

Flow cytometry showing lack of CD59 on RBCs and CD16 on neutrophils, and FLAER (fluorescent aerolysin)

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37
Q

Treatment of PNH?

A

Eculizumab, alloSCT

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38
Q

Good prognostic mutations in AML?

A

t(8;21)
t(16;16)
inv16
Mutated NPM1 without FLT3-ITD or with FLT3-ITD low

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39
Q

Poor prognostic markers in AML?

A
FLT3-ITD-high, esp with wild type NPM1
Del5q
Complex karyotype, monosomal karyotype
t(6;9)
t(9;22)
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40
Q

Standard induction therapy for AML?

A

7 days cytarabine, 3 days anthracycline

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41
Q

Specific treatment for FLT3 mutated AML

A

Midostaurin

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42
Q

APML mechanism of DIC?

A

Immature granulocytes express tissue factor and activate plasmin –> massive DIC

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43
Q

Prominent granulation and Auer rods on film = ?

A

APML

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44
Q

Diagnosis of APML?

A

PCR/FISH for PML-RAR protein, t(15;17)

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45
Q

Management of APML?

A

ATRA and arsenic

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46
Q

What types of leukaemia may be treated with imatinib?

A

CML, ALL (if Ph chromosome positive)

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47
Q

What is bilinatumumab?

A

Bispecific antibody CD3/19, brings T/B cells together

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48
Q

Treatment of MDS with del5q?

A

Lenalidomide

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49
Q

Management of high risk MDS?

A

Azacitadine, consider alloSCT

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50
Q

Management of low risk MDS?

A

Supportive

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51
Q

Time period for definition of ACUTE GVHD?

A

< 100 days

52
Q

Chronic GVHD time period

A

> 100 days

53
Q

Mechanism of action of bivalirudin

A

Direct thrombin inhibitor

54
Q

APTT checks which factors and pathway?

A

Intrinsic, factors 8/9/11/12

55
Q

Which DOAC does normal thrombin time exclude?

A

Dabigatran

56
Q

Which DOAC has least effect on coagulation studies?

A

Apixaban

57
Q

Which DOAC increases INR the most?

A

Rivaroxaban

58
Q

For which DOAC does a normal APTT exclude high drug levels?

A

Dabigatran

59
Q

Types of vWD?

A
1 = partial deficiency
2 = qualitative defect
3 = severe quantitative defect
60
Q

Severity tiers of haemophilia?

A
<1% = severe
1-5% = moderate
>5% = mild
61
Q

How do you manage inhibitors in haemophilia A?

A

FEIBA or rVIIa (Novoseven)

62
Q

FEIBA contains which factors?

A

2/7/9/10

63
Q

Prothrombin complex concentrate includes which factors?

A

2/9/10 (sometimes 7)

64
Q

What is emicizumab?

A

Bispecific antibody for factors 9a and 10, allows formation of 10a (for haemophilia A)

65
Q

What is Glanzmann’s thrombasthenia?

A

Defect in Gp2b/3a synthesis, shows no response in platelet aggregation studies

66
Q

Genetic mutation in follicular lymphoma

A

t(14;18) - juxtaposition of Ig heavy chain promoter to BCL-2, causing over-expression of BCL-2

67
Q

Cell surface markers in follicular lymphoma

A

CD10/19/20, and BCL2/6

68
Q

Treatment for limited stage follicular lymphoma?

A

Curative intent radiotherapy

69
Q

When to treat advanced stage follicular lymphoma?

A

GELF criteria (3 LN sites > 3cm, or 1 site > 7cm –> treat)

70
Q

Treatment for follicular lymphoma?

A

Obinutuzumab + bendamustine (obin better than ritux, bendamustine better than CHOP) induction followed by obin maintenance for 2 years. If grade 3b+ treat as aggressive DLBCL (R-CHOP)

71
Q

Treatment agents for relapse follicular lymphoma?

A
PI3Kinase inhibitors (idelalisib) - PBS for double refractory
Lenalidomide
CAR T-cells (trial)
72
Q

How to tell difference between Mantle cell and CLL?

A

Mantle cell is CD23 negative

73
Q

What is another name for Waldenstrom’s macroglobulinaemia?

A

Lymphoplasmacytic lymphoma

74
Q

What is most common genetic mutation in Waldenstrom’s?

A

95% have single AA mutation in MYD88, which triggers IRAK-mediated NFkB signalling and proliferation

75
Q

Typical cytogenetics for Mantle cell?

A

WHO diagnosis requires cyclin D1 nuclear expression (translocation, >90% cases) or t(11;14)

76
Q

What is gastric MALT associated with?

A

H. pylori - treat with H. pylori eradication

77
Q

What monoclonal protein is present in Waldenstrom’s?

A

IgM - level > 50g is indication for treatment

78
Q

What is the treatment for hairy cell leukaemia?

A

Cladribine

79
Q

What is most common cytogenetic mutation in Burkitt’s?

A

t(8;14)

Translocated IgH promoter and myc oncogene

80
Q

What is double hit lymphoma?

A

MYC and BCL2 (less commonly BCL6, also can rarely have triple hit)

81
Q

What is prognostic scoring system for DLBCL?

A

IPI. Use acronym APLES (age, performance status, LDH, EN involvement, stage [Ann Arbor])

82
Q

Treatment of DLBCL?

A

R-CHOP (ritux, cyclophos, doxorub, vincristine, pred)

83
Q

What is polatuzumab?

A

Humanised Mab to CD79b, combined with MMAE to form antibody drug conjugate (ADC) and deliver MMAE to target B cell

84
Q

What is brentuximab-MMAE?

A

Antibody drug conjugate (ADC) combining Mab to CD30 and MMAE, releasing toxin MMAE at target (used in Hodkin’s lymphoma and CD30+ve anaplastic large cell lymphoma)

85
Q

Most common subtype of HL?

A

Nodular sclerosing

86
Q

HL subtype with best prognosis?

A

Lymphocyte rich

87
Q

HL subtype with worst prognosis?

A

Lymphocyte depleted

88
Q

Common surface markers for HL?

A

CD15, CD30

89
Q

Treatment for HL?

A

ABVD or BEACOPP

Can also use immunotherapy (pembro) as uniformly over-express PDL1

90
Q

What are cell surface markers for CLL?

A

CD5, 19, 20, 23

CD5 is aberrant as this is usually a T cell marker

91
Q

What is MBUS?

A

MGUS equivalent for CLL

Lymphocytes < 5x10^9/L

92
Q

How do you stage CLL?

A

Rai or Binet

Binet uses Hb, plts, #LN stations

93
Q

Mutation that confers chemo resistance in CLL?

A

p53

94
Q

Poor prognostic markers in CLL?

A
Elevated B2MG
Elevated LDH
Unmutated IgH
17p deletion
p53 mutation/del
95
Q

Favourable mutation in CLL?

A

del13q

96
Q

Indications for treatment in CLL?

A

Cytopaenias (Hb < 100, neut < 1, plt < 100)
Lymphocyte doubling time < 6/12 or increasing >50% in 2/12
Symptomatic lymphadenopathy
B symptoms
Massive splenomegaly
Immune complications (AIHA, ITP)

97
Q

Treatment for CLL?

A

FCR (fludarabine, cyclophos, ritux)

98
Q

Non-chemo treatment options for CLL?

A

Ibrutinib (BTK inhibitor), venetoclax (BCL-2 inhibitor), idelalisib (pi3 inhibitor)

99
Q

Main risk with venetoclax?

A

Tumour lysis

100
Q

Genetic mutation in CML?

A

t(9;22) -> BCR-ABL

101
Q

What are the phases of CML?

A

Chronic
Accelerated
Blast phase (if 20% blasts in marrow = acute leukaemia)

102
Q

Genetic mutations in MPNs?

A

JAK2
CAL3
MPL

103
Q

What is given to 100% patients with PCV?

A

Aspirin

104
Q

When to treat MPNs with aspirin and hydroxyurea?

A

If platelets > 1000

If platelets > 60 and JAK2/MPL positive

105
Q

Treatment for myelofibrosis?

A

Ruxolitinib

106
Q

When to treat myelofibrosis with hydroxyurea?

A

If high platelets, also give aspirin

107
Q

What is HbH composed of?

A

beta4

108
Q

What is HbBart composed of?

A

gamma4

109
Q

What is HbF composed of?

A

alpha2gamma2

110
Q

What is HbA composed of?

A

alpha2beta2

111
Q

What is HbA2 composed of?

A

alpha2delta2

112
Q

Chromosome of alpha globin genes?

A

16p

113
Q

Chromosome of beta globin genes?

A

11

114
Q

Type of genetic mutation in beta thal?

A

Point mutations

115
Q

Type of genetic mutation in alpha thal?

A

Usually deletions

116
Q

What is HbE?

A

Structurally abnormal globin, behaves like beta thal. Point mutation leads to unstable alpha-beta interaction

117
Q

Test to do before starting Aspirin in ET patients with high plt count >1000K

A

Must do factor VIII/vWF studies (need to consider acquired vWD in those w. marked thrombocytosis, avoid aspirin if ristocetin cofactor activity <30%)

118
Q

What beta thal genetic composition leads to transfusion dependent thal?

A

Beta thal / beta thal

Beta thal / HbE

119
Q

What disease do you get with 3 abnormal alpha thal genes?

A

HbH

120
Q

What disease do you get with 3 abnormal alpha thal genes?

A

HbH disease

121
Q

What is genetic basis of sickle cell?

A

Single amino acid mutation in beta chain

122
Q

Target HbS % in SCD?

A

< 30%

123
Q

What is function of protein C?

A

Inactivates factors V and VIII (anti-coagulant)

124
Q

What is function of protein S?

A

Co-factor for protein C

125
Q

What are the types of amyloidosis?

A

AA
AL
ATTR