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WILLIAM'S HEMATOLOGY > 98 Follicular Lymphoma > Flashcards

98 Follicular Lymphoma Flashcards

1
Q

TRUE OR FALSE

Follicular lymphoma (FL) is an indolent lymphoid neoplasm that is derived from germinal center (GC) B cells and has a nodular or follicular histologic pattern.

A

TRUE

Follicular lymphoma (FL) is an indolent lymphoid neoplasm that is derived from germinal center (GC) B cells and has a nodular or follicular histologic pattern.

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2
Q

The most often cause of patients receiving treatment to fail or relapse within the first 2 years of initiating treatment

20%

A

Histologic transformation (HT) into diffuse large B-cell lymphoma (DLBCL)

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3
Q

Median age at diagnosis

A

63 years

Male-to-female ratio tends to be greater than 1

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4
Q

Conditions that increase risk for FL

A
  • Family history of NHL
  • Greater body mass index as young adults
  • Women with Sjögren syndrome
  • (heavy) smokers (particularly women)
  • Pesticides
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5
Q

A genetic hallmark of FL, is detectable in the blood of 50% to 70% of healthy individuals

A

t(14;18)

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6
Q

Histologic appearance of FL

A

Nodular and predominantly follicular pattern
Centroblasts and centrocytes are randomly distributed with a loss of the polarization

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7
Q

The follicular pattern can be highlighted by CD_ staining (follicular dendritic cell marker).

A

CD23

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8
Q

The hallmark of FL and can be useful in distinguishing neoplastic from reactive follicles

A

BCL2 overexpression

No BCL2 expression is found in 10% to 15% of cases

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9
Q

FL cells typically express:
* Monoclonal surface immunoglobulin (IgM with or without IgD, IgG, or rarely IgA)
* B-cell associated antigens (CD19, CD20, CD22, and CD79a)
* BCL6 and CD10

but not ___________

A

CD5 or CD43

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10
Q

FL Grade 1

A

0–5 per HPF

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11
Q

FL Grade 2

A

6-15 per HPF

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12
Q

FL Grade 3A

A

15 centroblasts per HPF; centrocytes still present

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13
Q

FL Grade 3B

A

15 centroblasts per HPF; composed only of centroblasts

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14
Q

Encompass the large majority of the cases (80%)

A

FL Grade 1 & 2

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15
Q

A distinct variant of FL differs from nodal FL in that it lacks BCL2 gene rearrangement, and patients usually present with grade 3 disease.

A

Testicular Follicular Lymphoma

It is reported in children and adolescents but can also occur in adults.
Usually localized, it is associated with a good prognosis after surgery.

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16
Q

Duodenal-Type Follicular Lymphoma : lesions, usually reported as incidental findings in the ________ portion of the duodenum, present as small polyps.

A

Second portion of the duodenum

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17
Q

Similar histology but is BCL2 negative without BCL2, BCL6, IRF4, or any aberrant IG rearrangement

A

Pediatric Follicular Lymphoma

It usually presents with stage I–II nodal disease, and there is a marked male predominance.

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18
Q

Detected by immunohistochemistry (IHC) for BCL2, is defined by partial or total colonization of GCs by clonal B cells carrying the t(14;18) in an otherwise reactive lymph node.

A

In Situ Follicular Neoplasia

Progression to overt FL is infrequent (~5%).

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19
Q

The most indolent asymptomatic disease not requiring immediate therapeutic intervention occurs in about__________of patients with FL

A

One-third

Grade 3B FL is a more aggressive disease requiring DLBCL-like management at diagnosis

20
Q

FL is an incurable disease except for these 2 conditions:

A

(a) localized FL might be cured with nonchemotherapy therapeutic options, and

(b) grade 3B has a low rate of relapse 2 years after immunochemotherapy, suggesting it may be a curable disease, in accord with its similarity to DLBCL

21
Q

The leading cause of death in patients with FL

A

Lymphoma

Especially after disease transformation

22
Q

Defined by the documentation of an increased number of large cells that eliminate the follicular structure, with a similar microscopic appearance to DLBCL.

A

Histologic Transformation

The annual incidence has been estimated at approximately 3%.

23
Q

TRUE OR FALSE

Patients with de novo HT (ie, a DLBCL histology with pathological findings that favors the existence of the FL component at the time of diagnosis) have a better outcome than those with a transformation event occurring after FL therapy.

A

TRUE

Patients with de novo HT (ie, a DLBCL histology with pathological findings that favors the existence of the FL component at the time of diagnosis) have a better outcome than those with a transformation event occurring after FL therapy.

24
Q

Mutations at diagnosis that might be associated with a shorter time to transformation

A

NOTCH2, DTX1, UBE2A, and HISTHIE

25
Q

The first disease manifestation of FL

A

Incidental discovery of one or several enlarged lymph nodes

Approximately 10% to 20% of patients with FL present with B symptoms (fever, weight loss, night sweats) at diagnosis.

26
Q

The optimal diagnostic procedure for disgnosis of FL

A

Surgical removal of an enlarged lymph node

The removal of several lymph nodes is not recommended in the context of FL and may expose the patient to surgical sequelae.

27
Q

TRUE OR FALSE

Most, if not all, cases of FL lesions are FDG avid, and the PET scan is the most sensitive staging procedure.

A

TRUE

Most, if not all, cases of FL lesions are FDG avid, and the PET scan is the most sensitive staging procedure.

28
Q

Diagnostic Criteria that is used to separate FL patients with a low or high tumor burden

A

Groupe d’Etude des Lymphomes Folliculaires (GELF) and the British National Lymphoma Investigation (BNLI)

29
Q

Treatment initiation criteria

GELF CRITERIA

A

Presence of at least one of the following:
* Involvement of ≥3 nodal sites, each with a diameter ≥3 cm
* Tumor mass ≥7 cm
* Symptomatic splenomegaly
* Pleural effusion or ascites
* Organ compression
* Any B symptom
* Serum LDH or β2M above upper limit of normal

30
Q

Treatment initiation criteria

BNLI CRITERIA

A

Presence of at least one of the following:
* Pruritus or B symptom(s)
* Rapid generalized disease progression in the preceding 3 months
* Life-endangering organ involvement
* Significant marrow infiltration
* Localized bone lesions detected on radiography or isotope scan
* Renal infiltration
* “Macroscopic” as opposed to “microscopic” liver involvement.

31
Q

A more objective measurement of tumor burden

A

TMTV on the initial PET

It has been proposed that a threshold of 510 cm3 could separate patients with a low or high tumor burden, with significant differences in PFS.

32
Q

Prognostic indices used in FL

A

Follicular International Prognostic Index (FLIPI)
FLIPI2
PRIMA-PI

33
Q

The PRIMA PI is based on two easily available parameters:

A

β2M and marrow infiltration

34
Q

Frontline therapy for Stage I/II Low tumor burden disease

A
  • “watch and wait,”
  • radiotherapy (24 Gy)
  • rituximab (4 weekly infusions)

Immunochemotherapy, as described later for symptomatic disseminated stages, should be given to patients with stage I disease if they have poor prognosis

35
Q

Treatment for Disseminated Follicular Lymphoma Without Treatment Initiation Criteria

A
  • “watch and wait,”
  • rituximab (4 weekly infusions)
36
Q

Treatment for Disseminated Follicular Lymphoma with Treatment Initiation Criteria

A

Immunochemotherapy

Rituximab was used in combination with chemotherapy (BR, RCHOP, RCVP)

37
Q

The benefit of maintenance treatment with rituximab was demonstrated in what trial

A

PRIMA trial

38
Q

Chemotherapy for patients with a contraindication to anthracyclines

A

BR

39
Q

Chemotherapy for older patients given its lower hematologic toxicity

A

R-CVP

40
Q

Chemotherapy with efficacy in case of HT

A

R-CHOP

41
Q

TRUE OR FALSE

Regarding the choice of anti-CD20, obinutuzumab is preferred to rituximab if a longer time before the next treatment is particularly desired.

A

TRUE

Regarding the choice of anti-CD20, obinutuzumab is preferred to rituximab if a longer time before the next treatment is particularly desired.

42
Q

Monitoring during treatment: it is usual to check the tumor response after how many months

A

After three or four cycles of treatment by CT, at the end of induction, and every 6 months during maintenance

PET results do not influence treatment in that patients in complete metabolic response (Deauville score 1, 2, or 3) should continue maintenance treatment.

43
Q

Recommend clinical monitoring for FL patients

A

Every 3 months in the first year, every 6 months for the next 4 years, and then annually

44
Q

Recommended imaging for FL patients

A

Imaging (CT) is recommended at 6 months and 1 year after the end of treatment and then every 1–2 years, although the impact of systematic imaging on patient survival is not demonstrated.

PET is not recommended for the follow-up of these patients.

45
Q

Treatment options for Early Relapse or Progression During Initial Treatment

Relapses occurring early (ie, within 2 years from treatment initiation) have a poor prognosis.

A
  • High-dose chemotherapy followed by a peripheral autologous stem cell transplant (ASCT)* OR
  • Combination of bendamustine–obinutuzumab followed by maintenance with obinutuzumab

*preceded by a platinum– cytarabine-based salvage immunochemotherapy

46
Q

Treatment options for Late Relapse

A
  • Immunotherapy with four weekly infusions of rituximab at 375 mg/m2*
  • Immunochemotherapy followed by maintenance treatment with rituximab (every 3 months for 2 years)**
  • Immunotherapy combination such as lenalidomide and rituximab***

*In cases of progressive disease with low tumor burden or for the most fragile patients

**more aggressive disease relapse

***12 cycles of lenalidomide (20 mg/day on days 1–21) plus rituximab once per week for 4 weeks in cycle 1 and day 1 of cycles 2 through 5)

47
Q

Treatment options for Subsequent Relapses

A
  • Alkylating agents and/or purine analogues
  • Low-dose radiation therapy (2 × 2 Gy)*
  • Radioimmunotherapy (ibritumomab tiuxetan or tositumomab)**
  • PI3K inhibitor (idelalisib, duvelisib, copanlisib) monotherapy***
  • EZH2 inhibitors monotherapy: clinical trial with promising results

localized tumor lesions
**more disseminated relapse providing there is not extensive marrow involvement
**previous treatments have included an alkylating agent and rituximab

WILLIAM'S HEMATOLOGY (109 decks)

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