Haematology Flashcards
(125 cards)
1
Q
Heinz bodies, bite and blister cells on film = ?
A
G6PD deficiency
2
Q
Howell-Jolly bodies = ?
A
Post splenectomy
3
Q
Smear/parachute/smudge cells on film = ?
A
CLL
4
Q
Cerebriform cells on film = ?
A
Peripheral T cell lymphoma
5
Q
Granular lymphocytes on film = ?
A
Large granular lymphocytic leukaemia
6
Q
Tear drop red cells on film = ?
A
Myelofibrosis
7
Q
Reed-Sternberg cells = ?
A
Hodgkin’s lymphoma
8
Q
Starry sky on bone marrow biopsy = ?
A
Burkitt’s lymphoma
9
Q
Dutcher bodies and flaming cells on bone marrow biopsy = ?
A
Myeloma
10
Q
Normal T cell surface markers?
A
CD3/4/5/8
11
Q
Normal B cell surface markers?
A
CD 19/20/kappa/lambda
12
Q
Normal stem cell surface markers?
A
CD34
13
Q
Normal monocyte cell surface markers?
A
CD14, CD64
14
Q
Normal granulocytes cell surface markers?
A
CD13/15/33
15
Q
Normal plasma cell surface markers?
A
CD138
16
Q
Definition of MGUS?
A
Paraprotein < 30g/l, clonal plasma cells in BM < 10%, no myeloma defining events
17
Q
RIsk factors for MGUS progression?
A
Paraprotein > 15g/L, non-IgG, FLC < 0.26 or > 1.65
18
Q
Smoulder myeloma definition?
A
Paraprotein > 30g/L or > 0.5g/d in urine, BM plasma cells 10-60%, no myeloma defining events
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
20
Q
Prognostic markers for myeloma (staging)?
A
Albumin < 35, B2-microglobulin > 5.5, LDH
21
Q
Most common cause of renal failure in myeloma?
A
Cast nephropathy
22
Q
Most common glomerular cause of renal failure in myeloma?
A
Amyloidosis
23
Q
Treatment for myeloma if young and fit?
A
Bortezomib/len/dex triplets
Melphalan + autoSCT
Len maintenance
24
Q
Treatment for myeloma if not fit for transplant
A
Bortez/len/dex trip/doublets
Other options - carfilzomib, pom, daratumumab
25
Side effects of proteasome inhibitors?
Bortezomib - neuropathy
| Carfilzomib - cardiac
26
Side effects of immunomodulators?
Thalidomide - neuropathy
Lenalidomide - cytopaenia
Pom - cytopaenia
27
Mechanism of action of daratumumab?
anti-CD38
28
Common indications for autologous transplant?
Myeloma, lymphoma (mantle, T-cell, HL, NHL)
29
Diagnostic test for hereditary spherocytosis?
Flow cytometry showing reduced binding eosin-5 maleimide
30
Pathophysiology of myelofibrosis
Clonal proliferation of megakaryocytes, leading to fibroblast proliferation
31
Leukoerythroblastic blood film = ?
Myelofibrosis with nucleated and tear drop red cells
32
Bone marrow biopsy in MF?
Dry tap
33
Treatment of myelofibrosis
Ruxolitinib
34
What causes PNH?
Mutation in PIGA gene, leads to defective glycoprotein anchors for CD55/59, therefore uncontrolled complement activation
35
Consequences of PNH?
IV haemolysis, pancytopaenia, thrombosis
36
Diagnosis of PNH?
Flow cytometry showing lack of CD59 on RBCs and CD16 on neutrophils, and FLAER (fluorescent aerolysin)
37
Treatment of PNH?
Eculizumab, alloSCT
38
Good prognostic mutations in AML?
t(8;21)
t(16;16)
inv16
Mutated NPM1 without FLT3-ITD or with FLT3-ITD low
39
Poor prognostic markers in AML?
```
FLT3-ITD-high, esp with wild type NPM1
Del5q
Complex karyotype, monosomal karyotype
t(6;9)
t(9;22)
```
40
Standard induction therapy for AML?
7 days cytarabine, 3 days anthracycline
41
Specific treatment for FLT3 mutated AML
Midostaurin
42
APML mechanism of DIC?
Immature granulocytes express tissue factor and activate plasmin --> massive DIC
43
Prominent granulation and Auer rods on film = ?
APML
44
Diagnosis of APML?
PCR/FISH for PML-RAR protein, t(15;17)
45
Management of APML?
ATRA and arsenic
46
What types of leukaemia may be treated with imatinib?
CML, ALL (if Ph chromosome positive)
47
What is bilinatumumab?
Bispecific antibody CD3/19, brings T/B cells together
48
Treatment of MDS with del5q?
Lenalidomide
49
Management of high risk MDS?
Azacitadine, consider alloSCT
50
Management of low risk MDS?
Supportive
51
Time period for definition of ACUTE GVHD?
< 100 days
52
Chronic GVHD time period
> 100 days
53
Mechanism of action of bivalirudin
Direct thrombin inhibitor
54
APTT checks which factors and pathway?
Intrinsic, factors 8/9/11/12
55
Which DOAC does normal thrombin time exclude?
Dabigatran
56
Which DOAC has least effect on coagulation studies?
Apixaban
57
Which DOAC increases INR the most?
Rivaroxaban
58
For which DOAC does a normal APTT exclude high drug levels?
Dabigatran
59
Types of vWD?
```
1 = partial deficiency
2 = qualitative defect
3 = severe quantitative defect
```
60
Severity tiers of haemophilia?
```
<1% = severe
1-5% = moderate
>5% = mild
```
61
How do you manage inhibitors in haemophilia A?
FEIBA or rVIIa (Novoseven)
62
FEIBA contains which factors?
2/7/9/10
63
Prothrombin complex concentrate includes which factors?
2/9/10 (sometimes 7)
64
What is emicizumab?
Bispecific antibody for factors 9a and 10, allows formation of 10a (for haemophilia A)
65
What is Glanzmann's thrombasthenia?
Defect in Gp2b/3a synthesis, shows no response in platelet aggregation studies
66
Genetic mutation in follicular lymphoma
t(14;18) - juxtaposition of Ig heavy chain promoter to BCL-2, causing over-expression of BCL-2
67
Cell surface markers in follicular lymphoma
CD10/19/20, and BCL2/6
68
Treatment for limited stage follicular lymphoma?
Curative intent radiotherapy
69
When to treat advanced stage follicular lymphoma?
GELF criteria (3 LN sites > 3cm, or 1 site > 7cm --> treat)
70
Treatment for follicular lymphoma?
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
Treatment agents for relapse follicular lymphoma?
```
PI3Kinase inhibitors (idelalisib) - PBS for double refractory
Lenalidomide
CAR T-cells (trial)
```
72
How to tell difference between Mantle cell and CLL?
Mantle cell is CD23 negative
73
What is another name for Waldenstrom's macroglobulinaemia?
Lymphoplasmacytic lymphoma
74
What is most common genetic mutation in Waldenstrom's?
95% have single AA mutation in MYD88, which triggers IRAK-mediated NFkB signalling and proliferation
75
Typical cytogenetics for Mantle cell?
WHO diagnosis requires cyclin D1 nuclear expression (translocation, >90% cases) or t(11;14)
76
What is gastric MALT associated with?
H. pylori - treat with H. pylori eradication
77
What monoclonal protein is present in Waldenstrom's?
IgM - level > 50g is indication for treatment
78
What is the treatment for hairy cell leukaemia?
Cladribine
79
What is most common cytogenetic mutation in Burkitt's?
t(8;14)
| Translocated IgH promoter and myc oncogene
80
What is double hit lymphoma?
MYC and BCL2 (less commonly BCL6, also can rarely have triple hit)
81
What is prognostic scoring system for DLBCL?
IPI. Use acronym APLES (age, performance status, LDH, EN involvement, stage [Ann Arbor])
82
Treatment of DLBCL?
R-CHOP (ritux, cyclophos, doxorub, vincristine, pred)
83
What is polatuzumab?
Humanised Mab to CD79b, combined with MMAE to form antibody drug conjugate (ADC) and deliver MMAE to target B cell
84
What is brentuximab-MMAE?
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
Most common subtype of HL?
Nodular sclerosing
86
HL subtype with best prognosis?
Lymphocyte rich
87
HL subtype with worst prognosis?
Lymphocyte depleted
88
Common surface markers for HL?
CD15, CD30
89
Treatment for HL?
ABVD or BEACOPP
| Can also use immunotherapy (pembro) as uniformly over-express PDL1
90
What are cell surface markers for CLL?
CD5, 19, 20, 23
| CD5 is aberrant as this is usually a T cell marker
91
What is MBUS?
MGUS equivalent for CLL
| Lymphocytes < 5x10^9/L
92
How do you stage CLL?
Rai or Binet
| Binet uses Hb, plts, #LN stations
93
Mutation that confers chemo resistance in CLL?
p53
94
Poor prognostic markers in CLL?
```
Elevated B2MG
Elevated LDH
Unmutated IgH
17p deletion
p53 mutation/del
```
95
Favourable mutation in CLL?
del13q
96
Indications for treatment in CLL?
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
Treatment for CLL?
FCR (fludarabine, cyclophos, ritux)
98
Non-chemo treatment options for CLL?
Ibrutinib (BTK inhibitor), venetoclax (BCL-2 inhibitor), idelalisib (pi3 inhibitor)
99
Main risk with venetoclax?
Tumour lysis
100
Genetic mutation in CML?
t(9;22) -> BCR-ABL
101
What are the phases of CML?
Chronic
Accelerated
Blast phase (if 20% blasts in marrow = acute leukaemia)
102
Genetic mutations in MPNs?
JAK2
CAL3
MPL
103
What is given to 100% patients with PCV?
Aspirin
104
When to treat MPNs with aspirin and hydroxyurea?
If platelets > 1000
| If platelets > 60 and JAK2/MPL positive
105
Treatment for myelofibrosis?
Ruxolitinib
106
When to treat myelofibrosis with hydroxyurea?
If high platelets, also give aspirin
107
What is HbH composed of?
beta4
108
What is HbBart composed of?
gamma4
109
What is HbF composed of?
alpha2gamma2
110
What is HbA composed of?
alpha2beta2
111
What is HbA2 composed of?
alpha2delta2
112
Chromosome of alpha globin genes?
16p
113
Chromosome of beta globin genes?
11
114
Type of genetic mutation in beta thal?
Point mutations
115
Type of genetic mutation in alpha thal?
Usually deletions
116
What is HbE?
Structurally abnormal globin, behaves like beta thal. Point mutation leads to unstable alpha-beta interaction
117
Test to do before starting Aspirin in ET patients with high plt count >1000K
Must do factor VIII/vWF studies (need to consider acquired vWD in those w. marked thrombocytosis, avoid aspirin if ristocetin cofactor activity <30%)
118
What beta thal genetic composition leads to transfusion dependent thal?
Beta thal / beta thal
| Beta thal / HbE
119
What disease do you get with 3 abnormal alpha thal genes?
HbH
120
What disease do you get with 3 abnormal alpha thal genes?
HbH disease
121
What is genetic basis of sickle cell?
Single amino acid mutation in beta chain
122
Target HbS % in SCD?
< 30%
123
What is function of protein C?
Inactivates factors V and VIII (anti-coagulant)
124
What is function of protein S?
Co-factor for protein C
125
What are the types of amyloidosis?
AA
AL
ATTR
