self evaluation 1 - abhh eventos › tutorial › aulas › pdf › caso7.pdf · • bilateral...
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Self evaluation 1
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Clinical Case • 55-year-old woman
• Bilateral enlargement of cervical, axillary and inguinal lymph nodes, largest diameter > 6 cm
• Hepatosplenomegaly. Enlargement of retroperitoneal, mesenteric and para-aortic lymph nodes.
• B symptoms were absent
• A biopsy of a left cervical lymph node was perfomed
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Histopathologic results
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1- The diagnosis is:
1. Follicular Lymphoma
2. Diffuse large B-cell lymphoma
3. Reactive hyperplasia
4. Peripheral-T-lymphoma
5. Mantle cell lymphoma
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Follicular lymphoma x Reactive hyperplasia
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REACTIVE HYPERPLASIA FOLLICULAR LYMPHOMA
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FOLÍCULO REACIONAL FOLÍCULO NEOPLÁSICO REACTIVE HYPERPLASIA FOLLICULAR LYMPHOMA
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Ki67
Bcl2
Bcl2
REACTIVE HYPERPLASIA x FOLLICULAR LYMPHOMA
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2- According to the updated WHO classification:
1. The follicules, and not the interfollicular regions, contain CD20+ B-
cells
2. The follicules are uniformly BCL2 negative
3. Grade 1 and Grade 2 cases have a marked predominance of
centrocytes and only few centroblasts. Since they represent a
continuum, distinction between them is not encouraged and a grade
“1-2” can be reported
4. Cases with > 50 centroblasts per high power field are considered
Grade 3 FL
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CD20+ B-cells in FL
CD20
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The follicules are uniformly BCL2 positive
Bcl-2
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Follicular lymphoma grading
Grade 1 Grade 2
Grade 3a
Grade 3b
Ki67
Ki67
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Cases with > 15 centroblasts per high power field are considered Grade 3 FL
Grade 1: 0-5 centroblasts
Grade 2: 6-15 centroblasts
Grade 3: > 15 centroblasts
3A - centrocytes present
3B – solid sheets of centroblasts
CRITERIA FOR CLASSIFICATION
Number of large cells per high power field
(centroblasts)
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3- Which option is correct regarding the biology of follicular lymphoma:
1. The tumor cells are usually SIg+. They express B-cell
associated antigens and also CD10 and CD5, but they are
negative for BCL-2.
2. The t(14;18) is present in 50% of patients.
3. This lymphoma usually presents the t(8;14) translocation,
associated with bcl2.
4. The postulated normal counterpart is the germinal center-B-
cell.
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3- Which option is correct regarding the biology of follicular lymphoma:
The t(14;18) is present in 75-85% of patients by cytogenetics
and 100% by FISH
The postulated normal counterpart is the germinal centre-B-
cell.
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4- The Follicular Lymphoma International Prognostic Index (FLIPI), developed by an international consortium, separates patients into three distinct risk categories. Which of these does not belong in
FLIPI?
1. Albumin
2. LDH
3. Hemoglobin
4. Age
5. Number of nodal areas
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Age >= 60
Stage III-IV
Hb < 12
LDH> normal
Nodal sites>4
Blood, 2004
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Blood, 2004
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5- Which choice is correct in regard to treatment of follicular lymphoma:
1. Treatment in early-stage follicular lymphoma is preferentially
Rituximab+chemotherapy.
2. Clinical indications for treatment include symptoms arising from
progressive local disease, systemic symptoms, threatened end-organ
function, significant cytopenia caused by marrow infiltration, and
transformation to an aggressive histology.
3. The incorporation of anthracyclines to chemotherapy regimens clearly
improved the overall survival in follicular lymphoma.
4. Watch-and-wait strategy is no longer used as a therapy approach for
low-tumor burden disease.
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Treatment in early-stage follicular lymphoma
15%-20% of follicular lymphoma patients presented in Stage I-II.
Radiotherapy is the standard of care of these patients
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Randomized trials demonstrated no difference in OS between the expectant approach of observation and
immediate treatment in advanced FL
Young RC, Lancet, 2003
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6- Which would be the best choice in regard to rituximab maintenance for follicular lymphoma
1. Current studies support the role of maintenance with rituximab after treatment of relapsed disease and also after front-line therapy.
2. Rituximab maintenance must be given in a weekly dose every two months for 2 years.
3. The duration of rituximab maintenance in most published studies is more than 5 years.
4. Rituximab is no longer considered for maintenance chemotherapy due to its long-term morbidity.
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R(375 mg/m2) IV once every 3 months until relapse or for a maximum of 2 years). R maintenance treatment achieves improvement in PFS (median survival 3,7 years vs 1,3 years) after induction treatment with chemotherapy plus rituximab. Update results with 6 years of median FUP
JCO, 2010
N=334 FL
1.3 3. 7
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Rituximab Maintenance for 2 Years in Patients with Untreated High Tumor Burden
Follicular Lymphoma After Response to Immunochemotherapy
G. A. Salles, J. F. Seymour, P. Feugier, F. Offner, A. Lopez-Guillermo, R. Bouabdallah,
L. M. Pedersen, P. Brice, D. Belada, L. Xerri on behalf of the PRIMA investigators
Gilles Salles Hospices Civils de Lyon
& Université Claude Bernard, Lyon, France
Salles G, Lancet 2011
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R-CHOP N = 885
Randomized N = 769
* 15 pts in 3 sites closed prematurely Patients evaluable (N = 1202)*
R-CVP N = 272
Patients registered: N = 1217
R-FCM N = 45
Randomized N = 222
Randomized N = 28
Observation N = 513
Rituximab N = 505
‡ 1 pt died during the randomization process
Indu
ctio
n M
aint
enan
ce
9 pts did not receive chemo
147 pts withdrew during or at the end of induction (failure to respond; toxicity)
28 pts failed to be randomized
Patient disposition
Patients randomized: N = 1018‡
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Primary endpoint (PFS) met at the planned interim analysis Rituximab maintenance significantly reduced the risk of progression by 50%
stratified HR=0.50 95% CI 0.39; 0.64 p<.0001
Time (months)
Rituximab maintenance N=505
Observation N=513
6 0 12 18 24 30 36
Prog
ress
ion-
free
rate
0.8
0.6
0.4
0.2
0
1.0 82%
66%
Patients at risk 505 513
472 443 336 230 103 18 469 411 289 195 82 15