Treatment of grade 3 FL or aggressive presentation
R-CHOP chemotherapy
what is R-CHOP
rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone
Clinical benefit of maintenance rituximab
Controversial – improves PFS but no survival benefit.
Management of advanced FL
IF asymptomatic –> monitor (can live for many years)
Typical clinical course of indolent lymphomas
Respond well to therapy, but most eventually progress (largely incurable)
survival of FL
Many have long term survival (20 years)
typical course
waxing and waning
characteristic immunophenotype of FL
CD20+, CD10+, BCL2+, CD5-, cyclin D1-
lymphoma grade that tends to behave more aggressively
Grade 3 and above
Branch point in management
- Grade 1-2 – treated as indolent lymphoma
- Grade 3-4 – treated aggressively with R-CHOP
Scoring system for prognosis
FLIPI
When to initiate treatment in advanced stage:
1) B-symptoms 2) Compromise of normal organ function due to progressive or bulky disease 3) Pleural effusions or peritoneal ascites 4) Cytopenias due to extensive bone marrow infiltration 7) autoimmune hemolytic anemia 8) splenomegaly 9) involvement of 3 or more sites, each with a diameter of 3 cm or larger
Why are asymptomatic patients with single site of disease commonly treated?
There’s good evidence that these people will never relapse
Why watchful waiting is an effective strategy?
- 1) Many can live for long time
- 2) Some go into spontaneous remission
- 3) Treatments haven’t shown a survival benefit
First line Treatment options for advanced stage
- R-CHOP
- BR
- R2
- R-CVP
How to select induction therapy
- age, comorbidities (cardiac disease)
- Disease grade
Best option in research in terms of PFS
BR
What is CVP regimen?
Cyclophosphomide, vincristine, prednisone
BR is
Bendamustine, rituxan
Role for maintenance therapy + agent?
Used in PR, benefit in CR remains controversial rituximab
Management of Relapse
No standard second line
Pi3K inhibitors approved for FL
Idelalisib, duvelisib, Copanlisib
Main SE to know with Idelalisib
Severe diarrhea and colitis
Management of colitis with Idelalisib
Steroids
What is R^2?
Rituximab + lenalidomide
Stage III disease means
Nodes on both sides of the diaphragm
Stage IV disease means
noncontiguous extralymphatic involvement
Stage I means
one node or a group of adjacent nodes
Stage II means
Two or more nodal groups on the same side of the diaphragm
Induction therapy for double hit DLBCL
da-EPOCH-R vs. R-hyperCVAD/MA
Response assessment for DLBCL
PET/CT 6-8 weeks after completion of chemotherapy
transformation is referred to as
“histologic transformation”
Role for RT in FL?
Limited to palliative radiation for locally symptomatic disease.
Role or transplant in FL?
- reserved for patients with relapsed or refractory FL or for those with histologic transformation
Important distinction in grading of FL
Grade 3b is more aggressive and referred t
Follicular large cell lymphoma is also referred to as
Grade 3b disease
immunophenotype of FL
CD10+
CD19+
CD20+
BCL2
characteristic chromosomal abnormality of FL
t(14;18) (leads to transcription of excessively high levels of BCL-2, which inhibits cells from undergoing apoptosis)
Definition of FL grade 3b
- greater than 15 centroblasts per HPF with solid sheets of centroblasts
Clinical characteristics of grade 3b FL
- distinct subset that shares many features with DLBCL
Variables included in FLIPI score
- age over 60
- stage III or IV
- hbg less than 12
- LDH greater than the ULN
typical presentation
DISSEMINATED, not localized
- multistation lymphadenopathy + splenomegaly + bone marrow involvement
chromosome abnormality characteristic of FL
t(14;18)
FL immunophenotype
CD10 + CD19 + CD20 + often BCL2
indolent biology in FL refers to
Grade I-II
Can you retreat with rituxan in FL?
yes
Gene on chromosome 18
BCL2
Criteria used for starting treatment in FL
GELF criteria
Management of patients with low grade low tumor burden or ineligible for combination therapy
rituximab monotherapy