Lymphoma and Myeloma FRCR CO2A Flashcards
1
Q
What is the incidence of lymphoma cases per year?
A
Around 1700 cases per year.
2
Q
In children under 14 years of age, what is lymphoma’s rank among common cancers?
A
Third most common cancer after leukaemia and brain tumours.
3
Q
What percentage of cancers in teenagers and young adults does lymphoma account for?
A
21% of cancers.
4
Q
What is the most significant risk factor for developing lymphoma?
A
Immune dysfunction.
5
Q
Name three viral infections associated with immune dysfunction that may lead to lymphoma.
A
- HIV * HBV * HCV
6
Q
What are MALT lymphomas associated with?
A
Antigenic stimulation by infectious agents including Helicobacter pylori, Chlamydia psittaci, and Borrelia burgdorferi.
7
Q
What is one common clinical presentation of lymphoma?
A
Painless lymphadenopathy.
8
Q
According to NICE guidance, what investigations should be done for unexplained lymphadenopathy?
A
- FBC * Blood film * ESR * Plasma viscosity * CRP
9
Q
What are ‘B’ symptoms in lymphoma?
A
- Fever > 38°C * Drenching night sweats * Weight loss > 10% of body weight
10
Q
What is essential for planning management of lymphoma?
A
Accurate diagnosis and staging.
11
Q
What is the standard staging system for Hodgkin lymphoma?
A
Ann Arbor staging system.
12
Q
What is the crude incidence rate of lymphoma in the UK?
A
Ranges from 0.2 to 9 cases per 100,000.
13
Q
What percentage of all cancer cases does Hodgkin lymphoma account for?
A
0.6%.
14
Q
What is the most common subtype of lymphoma in the UK?
A
Diffuse large B cell lymphoma (DLBCL).
15
Q
What factors are used in the International Prognostic Index (IPI) for DLBCL?
A
- Age > 60 years * Performance status (PFS) 2 to 4 * Stage 3-4 * LDH above normal * > 1 extranodal site
16
Q
What treatment has significantly improved outcomes in DLBCL?
A
Addition of rituximab (R) to CHOP chemotherapy.
17
Q
What is the median age at diagnosis for diffuse large B-cell lymphoma?
A
64 years.
18
Q
What is the definition of bulky disease in lymphoma?
A
Nodal masses measuring ≥ 10 cm.
19
Q
What is the role of PET-CT in lymphoma management?
A
Staging, interim response assessment, and post-treatment evaluation.
20
Q
True or False: PET-CT can detect bone marrow involvement even in patients with a negative iliac crest bone marrow biopsy.
A
True.
21
Q
Fill in the blank: The criteria for defining disease bulk in Hodgkin lymphoma is set at _______.
A
10 cm.
22
Q
What is the purpose of baseline cardiac assessment before chemotherapy?
A
To assess cardiac risk factors.
23
Q
What are the lymph node regions involved in lymphoma staging?
A
- Right and left cervical * Right and left axillary * Right and left infra-clavicular * Mediastinal * Right and left hilar * Para-aortic * Mesenteric * Right and left pelvic * Right and left inguinal/femoral
24
Q
What should be discussed with all patients regarding treatment?
A
Fertility preservation options.
25
What is the significance of a negative post-treatment PET scan result?
Indicates an excellent outcome.
26
What are the two major subtypes of DLBCL according to gene expression profiling?
* Germinal centre B cell-like (GCB) * Activated B cell-like (ABC)
27
What is the survival rate for patients with low-risk factors in the International Prognostic Index (IPI)?
73%.
28
What is the survival rate for patients with high-risk factors in the International Prognostic Index (IPI)?
26%.
29
What is indicated for patients with elevated serum uric acid due to tumor lysis syndrome?
Rasburicase.
30
What chemotherapy regimen has significantly improved outcomes in DLBCL?
R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
## Footnote
R-CHOP has shown better survival rates compared to CHOP alone.
31
What were the 2-year survival rates for R-CHOP compared to CHOP in the GELA study?
76% for R-CHOP compared to 63% for CHOP
## Footnote
This study involved patients aged 60–80 years.
32
What did the MInT study reveal about the addition of rituximab to chemotherapy for low-risk patients under 60?
Improved 6-year progression-free survival from 55.8% to 74.3%
## Footnote
The study focused on patients with low-risk IPI scores.
33
What radiation therapy dosage is given to initial sites of disease larger than 5 cm?
30–40 Gy
## Footnote
This dosage is used for patients with bulky disease.
34
What size of bulky disease is considered an independent poor prognostic factor?
Larger than 7.5 cm
## Footnote
Bulky disease is a significant factor for prognosis.
35
Did dose intensification using two-weekly R-CHOP-14 show significant differences in survival?
No significant difference
## Footnote
Comparison was made to three-weekly R-CHOP-21.
36
What is considered the current standard treatment for all except stage I non-bulky DLBCL?
6 cycles of R-CHOP-21
## Footnote
This regimen is widely accepted in treatment protocols.
37
Has high-dose chemotherapy consolidation shown a survival benefit in the R-CHOP era?
No survival benefit has been shown
## Footnote
This applies to first remission settings.
38
What may help identify patients for whom chemotherapy intensification may be beneficial in the future?
Determination of high-risk molecular phenotypes
## Footnote
This approach could lead to more personalized treatment strategies.
39
What is the outcome of CHOP plus IFRT compared to CHOP alone in patients with low-risk IPI scores?
CHOP plus IFRT had higher rates of 5-year progression-free (76% vs 67%) and overall survival (82% vs 74%)
## Footnote
Based on the SWOG study findings.
40
What was the follow-up result of the ECOG study regarding the addition of RT in complete responders?
Improved 10-year disease-free survival (57% vs 46%) but not overall survival (64% vs 60%)
## Footnote
This involved patients in partial remission receiving 40 Gy IFRT.
41
What is the recommended treatment for stage 1A non-bulky or stage IIA less-fit elderly patients?
3 cycles of R-CHOP-21 and 30 Gy IFRT/ISRT
## Footnote
Treatment recommendation based on patient fitness and disease stage.
42
What distinguishes primary mediastinal B-cell lymphoma from DLBCL?
Unique clinical and molecular features, more common in adolescents and young adults
## Footnote
Median age at presentation is in the fourth decade.
43
What is the standard treatment for primary mediastinal B-cell lymphoma?
Six cycles of R-CHOP
## Footnote
Response rates may be poorer than those for DLBCL.
44
What is the 5-year overall survival range for primary mediastinal B-cell lymphoma?
0% to 65%, depending on the patient’s IPI score.
45
True or False: CNS relapse occurs in more than 3% of patients with DLBCL treated in the R-CHOP era.
False
## Footnote
CNS relapse occurs in fewer than 3% of patients.
46
What are the risk factors for CNS relapse in DLBCL?
High IPI scores, particularly more than one extranodal site, para-meningeal or testicular involvement.
47
What is the most widely used regimen for high-dose chemotherapy in relapsed/refractory disease?
BEAM (BCNU, etoposide, cytarabine, melphalan)
## Footnote
This regimen is used with autologous stem cell transplant.
48
What is the median survival following treatment with whole-brain RT alone for PCNSL?
12–18 months.
49
What is the role of high-dose cytarabine (HD-AC) in the treatment of PCNSL?
Improves response rates when added to HD-MTX.
50
What is the standard treatment volume for whole-brain radiotherapy?
Includes the whole brain, eyes, and optic nerves
## Footnote
Shielding the anterior chamber and lens is also standard.
51
What is the median age at diagnosis for primary CNS lymphoma (PCNSL)?
Fifth decade of life.
52
What characterizes follicular lymphoma (FL)?
Relapsing and remitting pattern, incurable except for non-bulky stage IA disease.
53
What is the typical age at diagnosis for patients with follicular lymphoma?
60 years.
54
What chromosomal abnormality is commonly found in follicular lymphoma?
t(14;18) translocation.
55
What is the typical clinical presentation of patients with advanced-stage follicular lymphoma?
Bulky lymphadenopathy and bone marrow involvement.
56
What is the incidence of primary CNS lymphoma (PCNSL) in non-HIV patients?
90% of PCNSL cases, but only 1% of NHL cases.
57
What does the Follicular Lymphoma International Prognostic Index (FLIPI) assess?
FLIPI assesses prognosis in follicular lymphoma based on various clinical factors.
## Footnote
These factors include age, stage, number of nodal sites involved, LDH levels, and hemoglobin levels.
58
What are the three risk groups according to the FLIPI score?
* Low (0-1 risk factors)
* Intermediate (2 risk factors)
* High (3-5 risk factors)
## Footnote
Corresponding 3-year PFS and OS rates vary by group.
59
What is the recommended treatment for stage IA non-bulky follicular lymphoma?
Local radiotherapy can provide progression-free survival rates of approximately 50%.
## Footnote
Studies indicate that specific radiation doses can be more effective.
60
What is the impact of early treatment with rituximab in asymptomatic patients with follicular lymphoma?
There is no survival benefit for immediate treatment compared to a 'watch-and-wait' approach.
## Footnote
Early treatment may defer the need for chemotherapy.
61
What factors influence the treatment of relapsed follicular lymphoma?
* Age
* Duration of remission
* Previous treatment
* Burden of follicular lymphoma
* Symptoms
* Performance status
## Footnote
These factors guide management decisions.
62
Which treatment may be considered for younger patients with short remissions in follicular lymphoma?
Intensive induction regimens such as R-CHOP may be considered.
## Footnote
Discussion of transplant options for consolidation is also recommended.
63
What percentage of patients with mantle cell lymphoma (MCL) are typically male?
MCL has a male-to-female ratio of 4:1.
## Footnote
It accounts for around 5% of non-Hodgkin lymphoma (NHL).
64
What chromosomal translocation is characteristic of mantle cell lymphoma?
t(11;14) leads to overexpression of cyclin D1.
## Footnote
This results in deregulation of cell cycle progression.
65
What is the median survival for patients diagnosed with mantle cell lymphoma?
The median survival is 4–5 years.
## Footnote
MCL is considered an incurable lymphoma with an aggressive course.
66
What is the recommended first-line treatment for patients with MALT lymphoma?
Local treatments such as surgery or radiotherapy are effective.
## Footnote
Chemotherapy is usually weighed against the patient's performance status and comorbidities.
67
What percentage of gastric MALT lymphoma cases are associated with H. pylori infection?
Up to 90% of gastric MALT lymphoma cases are associated with H. pylori infection.
## Footnote
H. pylori eradication therapy is crucial for management.
68
What is the recommended regimen for H. pylori eradication in gastric MALT lymphoma?
* Omeprazole 20 mg b.d.
* Metronidazole 400 mg b.d.
* Amoxicillin 1 g b.d. (or clarithromycin if allergic)
## Footnote
This regimen should be followed for 14 days.
69
What is a potential outcome of H. pylori eradication therapy in gastric MALT lymphoma?
Complete and prolonged histological remission can occur in up to 70% of patients.
## Footnote
Monitoring through tests like urea breath tests is important.
70
What is a significant complication of using fludarabine in treatment for relapsed follicular lymphoma?
Fludarabine can lead to prolonged lymphopenia and increased risk of opportunistic infections.
## Footnote
This is particularly concerning for younger patients.
71
What is the main cause of death in patients with follicular lymphoma?
Transformation to high-grade lymphoma, usually DLBCL.
## Footnote
Recent data suggests improved outcomes over time for these patients.
72
What is chemotherapy effective for in the context of lymphoma?
Chemotherapy is effective but the t(11;18) translocation predicts poor response to alkylating agents as sole treatment.
## Footnote
The addition of rituximab to chlorambucil improves 5-year event-free survival.
73
What is the treatment of choice for stage 1A extranodal MALT lymphoma?
Local radiotherapy
## Footnote
It results in good long-term control rates with minimal toxicity.
74
What is the CTV definition for gastric MALT?
The whole stomach
75
What is the CTV definition for salivary glands?
The whole gland
76
What is the recommended dose for local radiotherapy?
24–30 Gy in daily 2 Gy fractions.
77
What are the 5-year disease-free survival rates for stage 1A extranodal MALT lymphoma?
Higher than 90%.
78
What percentage of non-Hodgkin lymphoma (NHL) do T- and NK-cell NHL account for?
Approximately 12%.
79
What is a specific entity associated with EBV in T- and NK-cell NHL?
Extranodal NK-/T-cell lymphoma
## Footnote
It is more common in the Far East and causes destructive lesions in nasal or paranasal areas.
80
What is the optimal dose of radiotherapy for localized T- and NK-cell NHL?
At least 50 Gy to the nasal cavity and both antra.
81
What treatment showed improved results compared to historical controls for localized T- and NK-cell NHL?
Chemoradiotherapy.
82
What is the median survival for stage IA cutaneous T-cell lymphoma?
Not reached at 35.5 years.
83
What is the median survival for stage IVB cutaneous T-cell lymphoma?
0.9 years.
84
What are the risk factors for early-stage cutaneous T-cell lymphoma?
* Male gender
* Age > 60 years
* Presence of plaques
* Folliculotropic MF
* Palpable nodes
85
What is the treatment approach for patients with early-stage cutaneous T-cell lymphoma?
Observation until symptomatic, then skin-directed treatment such as topical steroids or PUVA therapy.
86
What is the standard dose for total skin electron beam therapy (TSEBT)?
30–36 Gy in 2 Gy fractions spread over 9–10 weeks.
87
What are the acute complications of TSEBT?
* Erythema
* Dry desquamation
* Hair and nail loss
* Reduced sweating
88
What is the bimodal incidence pattern of Hodgkin lymphoma?
First peak in childhood in developing countries; second peak in older adults in developed countries.
89
What is the male-to-female ratio for Hodgkin lymphoma incidence?
1.5:1.
90
What is the standard treatment for early-stage Hodgkin lymphoma?
Combined-modality treatment based on ABVD chemotherapy followed by radiotherapy.
91
What are the risk factors according to the German Hodgkin Study Group for early-stage Hodgkin lymphoma?
* Age > 45 years
* Male
* Serum albumin < 40 g/L
* Hb < 105 g/L
* Stage IV disease
* Leucocytosis > 15 × 10^9/L
* Lymphopenia < 0.6 × 10^9/L or < 8% WBC count
92
What is the recommended treatment for early-stage Hodgkin lymphoma with no risk factors?
2 cycles ABVD and 20 Gy ISRT.
93
What is the treatment for patients with early-stage Hodgkin lymphoma with risk factors?
4 cycles ABVD and 30 Gy ISRT.
94
True or False: The omission of radiotherapy increases the relapse rate in Hodgkin lymphoma.
True.
95
What is the 3-year progression-free survival rate for Hodgkin lymphoma patients who received radiotherapy?
94.6%.
96
What is the 3-year progression-free survival rate for Hodgkin lymphoma patients who received no further therapy?
90.8%.
97
What is the 5-year progression-free survival rate for patients with 0–2 risk factors?
74%
## Footnote
Patients with 4 or more risk factors have a 47% progression-free survival rate.
98
List the GHSG risk factors for Hodgkin lymphoma.
* Age > 45 years
* Male
* Serum albumin < 40 g/L
* Hb < 105 g/L
* Stage IV disease
* Leucocytosis > 15 × 10^9/L
* Lymphopenia < 0.6 × 10^9/L or < 8% WBC count
99
What is the standard treatment after chemotherapy for patients with bulky mediastinal or residual PET positive masses?
Consolidation radiotherapy
## Footnote
Typically administered at a dose of 30–40 Gy in 1.8–2 Gy fractions.
100
What chemotherapy regimen is used for paediatric Hodgkin lymphoma patients in the low-risk group after induction?
No further chemotherapy
## Footnote
Patients in intermediate- and high-risk groups receive additional chemotherapy.
101
What are the second-line chemotherapy regimens for refractory Hodgkin lymphoma?
* IGEV
* miniBEAM
* ESHAP
102
True or False: Monoclonal antibodies against CD30 have shown significant effectiveness as single agents in Hodgkin lymphoma.
False
## Footnote
They are ineffective as single agents but show activity when combined with an anti-tubulin agent.
103
What is the median progression-free survival when using brentuximab vedotin for relapsed and refractory Hodgkin lymphoma?
5.6 months
## Footnote
The overall response rate was 75%, with complete remission in 34% of patients.
104
What characterizes nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL)?
Slowly progressive lymphadenopathy at peripheral sites
## Footnote
It is more common in men and rarely affects the mediastinum.
105
What is the recommended treatment for stage IA Hodgkin lymphoma on PET-CT, non-bulky?
IFRT/ISRT alone, 30 Gy in 15 fractions
106
What is the annual incidence of myeloma in the UK?
60–70 per million
107
What is the median age at presentation for myeloma?
70 years
108
What are the screening tests for multiple myeloma?
* FBC
* Serum urea, electrolytes, calcium
* Serum protein electrophoresis
* Serum immunoglobulins
* ESR or plasma viscosity
* Urine sample for Bence–Jones proteins
* Skeletal survey
* Serum albumin
* Serum uric acid
* Bone marrow aspirate and trephine
109
What defines symptomatic myeloma in terms of M-protein and bone marrow clonal plasma cells?
* M-protein in serum ≥ 30 g/L
* Bone marrow clonal plasma cells ≥ 10%
* Myeloma-related organ impairment or bone lesions
110
What is the common pathophysiological factor in the development of myeloma?
Overproduction of IL-6
111
What is the treatment aim for patients with active myeloma?
Control disease, maximize quality of life, and prolong survival
112
What is the treatment of choice for solitary bone plasmacytoma (SBP)?
Radical radiotherapy
## Footnote
There have been no randomized trials supporting this treatment.
113
What is the most common site for solitary bone plasmacytoma?
Axial skeleton, including thoracic and lumbar vertebrae, skull, ribs, and sternum
114
What criteria are required for the diagnosis of solitary plasmacytoma?
* Histologically confirmed single lesion
* Normal bone marrow biopsy (< 10% plasma cells)
* Negative skeletal survey
* Normal haemoglobin, renal function, and calcium
115
What is the median survival for patients with stage III solitary plasmacytoma?
29 months
116
What is the median time for progression to multiple myeloma in patients?
2–4 years
## Footnote
More than 75% of patients progress to multiple myeloma during this period.
117
What is the median survival for patients with multiple myeloma?
7–11 years
118
What role does surgery play in the management of spinal plasmacytoma (SPB)?
Surgery can address structural instability or symptoms from spinal cord compression but is not definitive.
119
What is the treatment of choice for solitary plasmacytoma of bone (SPB)?
Radical radiotherapy
120
What were the 10-year overall survival rates for patients treated with radiotherapy alone for SPB?
51%
121
What is the local control rate for radiotherapy in SPB?
78%
122
What is the progression rate to myeloma for patients treated with radiotherapy for SPB?
71%
123
What is the recommended margin for the clinical target volume (CTV) in radiotherapy for SPB?
At least 2 cm
124
Where do 90% of solitary extramedullary plasmacytomas (SEPs) occur?
In the head and neck
125
What is the primary treatment of choice for solitary extramedullary plasmacytoma (SEP)?
Radiotherapy
126
What were the 5-year local control rates for patients treated with radiotherapy for EMP of the head and neck?
95%
127
What is the optimal dose of radiotherapy for extramedullary plasmacytoma (EMP)?
Between 45 and 50 Gy
128
What is the role of adjuvant chemotherapy in the management of high-grade extramedullary tumours?
Uncertain, but may be considered
129
What is the advantage of allogeneic stem cell transplantation (SCT) in lymphoma treatment?
Uses a tumour cell-free graft and can result in a graft-versus-lymphoma effect
130
What are the two types of conditioning regimens for SCT?
* Myeloablative
* Reduced-intensity (RIC)
131
What is the standard dose regimen for total body irradiation (TBI)?
14.4 Gy in 8 fractions over 4 days
132
What are the dose-limiting toxicities associated with total body irradiation?
* Interstitial pneumonitis
* Hepatic veno-occlusive disease
133
What are the three different radiotherapy techniques used in lymphoma?
* Involved node radiotherapy (INRT)
* Involved site radiotherapy (ISRT)
* Involved field radiotherapy (IFRT)
134
What does involved node radiotherapy (INRT) aim to treat?
Only the initial involved node as the CTV
135
What is the key difference between ISRT and IFRT?
ISRT includes initially involved nodes with a safety margin, while IFRT irradiates entire lymph node regions.
136
What is the recommended margin for CTV to PTV in ISRT?
1 cm considered acceptable
137
What is the purpose of target delineation in radiotherapy?
To accurately define treatment volumes
138
What is the risk of developing subclinical hypothyroidism after neck irradiation?
About one third of patients
139
What late complications can arise from mediastinal irradiation?
* Increased risk of coronary artery disease
* Valvular abnormalities
140
What is the relationship between age at treatment and breast cancer risk in patients receiving supradiaphragmatic radiotherapy?
Inversely related
141
What screening is recommended for women treated for Hodgkin lymphoma under the age of 36?
Mammography or MRI starting 8 years after radiotherapy
