Lymphomes – ClinCaseQuest https://clincasequest.hospital Medical education in clinical cases Tue, 11 Jan 2022 13:40:11 +0000 en-US hourly 1 https://wordpress.org/?v=6.2.11 https://clincasequest.hospital/wp-content/uploads/sites/7/2020/02/logo-ru-project-90x90.png Lymphomes – ClinCaseQuest https://clincasequest.hospital 32 32 Follicular Lymphoma International Prognostic Index (FLIPI). Online calculator https://clincasequest.hospital/flipi/ Mon, 10 Jan 2022 09:27:11 +0000 https://clincasequest.hospital/?p=32403 Follicular Lymphoma International Prognostic Index (FLIPI) estimates overall survival based on clinical information. FLIPI is designed to be used in patients with follicular lymphoma and…

Сообщение Follicular Lymphoma International Prognostic Index (FLIPI). Online calculator появились сначала на ClinCaseQuest.

]]> Follicular Lymphoma International Prognostic Index (FLIPI) estimates overall survival based on clinical information.

FLIPI is designed to be used in patients with follicular lymphoma and to risk stratify patients based on prognosis. This can help guide when and how to treat such patients.

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(See Evidence for nodal diagram)

Stage III-IV Stage I: disease is located in a single region, usually one lymph node and the surrounding area. Stage II: disease is located in two separate regions, an affected lymph node or organ and a second affected area. Both affected areas are confined to one side of the diaphragm. Stage III: disease involves both sides of the diaphragm, including one organ or area near the lymph nodes or the spleen. Stage IV: diffuse or disseminated involvement of one or more extranodal organs, with or without associated lymph node involvement. (See Evidence for stage diagram)

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The FLIPI score was developed based on 4,167 patients with follicular lymphoma providing characteristics at diagnosis. Univariate and multivariate analyses were then used to develop a prognostic index. This was then validated in a cohort of 919 patients. Age, Ann Arbor stage, hemoglobin level, number of nodal areas, and serum LDH level were identified as adverse prognostic factors. The FLIPI was validated in a subsequent study and the performance improved by further stratifying age and including the presence of cerebrovascular disease.

The FLIPI, while a useful general prognostic tool, does not reliably predict those at diagnosis who will have an extremely rapid decline. Rather it assists with providing prognostic information. Ultimately, clinical judgement and comprehensive review of the patient’s previous workup are critical to make this assessment.

The GELF Criteria can help identify those patients with follicular lymphoma at higher risk for rapid disease progression and in whom immediate therapy may be needed.

The FLIPI score is easy to use with information most providers will already have available. Clinical evaluation, laboratory testing, and imaging are all that is needed to risk stratify patients and estimate prognosis.

Age > 60 yearsNo0
 Yes+1
>4 nodal sites
See Nodal Sites diagram		No0
 Yes+1
LDH elevatedNo0
 Yes+1
Hemoglobin <120 g/L or 12 g/dLNo0
 Yes+1
Stage III-IV
Stage I: disease is located in a single region, usually one lymph node and the surrounding area.
Stage II: disease is located in two separate regions, an affected lymph node or organ and a second affected area. Both affected areas are confined to one side of the diaphragm.
Stage III: disease involves both sides of the diaphragm, including one organ or area near the lymph nodes or the spleen.
Stage IV: diffuse or disseminated involvement of one or more extranodal organs, with or without associated lymph node involvement.
See Staging Diagram		No0
 Yes+1

Score Interpetation

ScoreRisk Group10-year Overall Survival
≤1Low70%
2Intermediate50%
≥3High35%

Useful Mnemonic – NoLash

  • Nodal areas
  • LDH
  • Age
  • Stage
  • Hemoglobin level

Nodal Sites

  • follicular lymphoma, follicular lymphoma therapy, tumor, follicular lymphoma detection, follicular lymphoma treatment, lymphoma treatment, lymphoma, B symptoms, nodal site, node, nodal sites, tumor, extranodal tumor, nodular tumor, calculator, medical calculators, medcalk, calculate online, estimate, online calculator, severity estimate, criteria, GELF

Staging Diagram

  • Follicular Lymphoma International Prognostic Index, FLIPI, follicular lymphoma, follicular lymphoma therapy, tumor, follicular lymphoma detection, follicular lymphoma treatment, lymphoma treatment, lymphoma, nodal site, node, nodal sites, calculator, medical calculators, medcalk, calculate online, estimate, online calculator, severity estimate, criteria, Prognostic Index, Index

A FLIPI score of 0 to 1 is considered “low risk” with a 10 year overall survival of 70%. A score of 2 is considered “intermediate risk” with a 10 year overall survival of 50%. Finally, a score of ≥ 3 is considered “high risk” with a 10 year overall survival of 35%. Patients with an initial remission duration less than the median progression free survival for a particular treatment regimen and/or with a high FLIPI score are more likely to have a shorter remission duration.

Treatment is varied depending on the stage and tumor burden.

Stages I-II

  • Treatment options include:
    • Involved field radiotherapy (IFRT)
    • Clinical trials
    • Active surveillance

Stages III-IV or Bulky stage II – asymptomatic

  • Treatment options include:
    • Clinical trials
    • Active surveillance

Stages III-IV or Bulky Stage II – with GELF/BNLI risk factors or high-risk FLIPI score

  • Treatment options include:
    • Rituximab + cyclophosphamide, vincristine, doxorubicin, prednisone
    • Rituximab alone
    • Bendamustine + rituximab
    • Rituximab + cyclophosphamide, vincristine, prednisone
    • Clinical trials

There is continued controversy around when and how to treat follicular lymphoma, including in patients with relapsed/refractory disease. Questions to consider when making treatment decisions:

  • When to initiate therapy versus active surveillance
    • Deciding amongst various treatment options
    • Balancing efficacy and toxicities of treatment options
    • Determining what constitutes a complete response/endpoint of treatment
  • Finally, with follicular lymphoma, a provider should be aware of transformation to diffuse large B-cell lymphoma, which is a more aggressive disease and would require prompt treatment.

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Literature:

  1. Solal-Celigny et al. Follicular Lymphoma International Prognostic Index. Blood 104:1258-1265. (2004) https://pubmed.ncbi.nlm.nih.gov/15126323/
  2. van de Schans SAM, et. al. Validation, revision and extension of the Follicular Lymphoma International Prognostic Index (FLIPI) in a population-based settingAnn Oncol (2009) 20 (10): 1697-1702 first published online June 23, 2009 doi:10.1093/annonc/mdp053 https://www.journals.elsevier.com/annals-of-oncology

See also:

Groupe d’Etude des Lymphomes Folliculaires (GELF) Criteria. Online calculator

Сообщение Follicular Lymphoma International Prognostic Index (FLIPI). Online calculator появились сначала на ClinCaseQuest.

]]> Groupe d’Etude des Lymphomes Folliculaires (GELF) Criteria. Online calculator https://clincasequest.hospital/gelf/ Mon, 10 Jan 2022 08:21:39 +0000 https://clincasequest.hospital/?p=32397 Groupe d’Etude des Lymphomes Folliculaires (GELF) Criteria determines if immediate therapy for follicular lymphoma is required. The criteria can help identify those patients with follicular…

Сообщение Groupe d’Etude des Lymphomes Folliculaires (GELF) Criteria. Online calculator появились сначала на ClinCaseQuest.

]]> Groupe d’Etude des Lymphomes Folliculaires (GELF) Criteria determines if immediate therapy for follicular lymphoma is required.

The criteria can help identify those patients with follicular lymphoma at higher risk for rapid disease progression and in whom immediate therapy may be needed.

Any nodal or extranodal tumor mass >7 cm diameter:
Yes
No

Involvement of at least 3 nodal sites, each with diameter >3 cm View more nodal site information in the More Info section:
Yes
No

Presence of any systemic or B symptoms:
Yes
No

Splenic enlargement with inferior margin below the umbilical line:
Yes
No

Compression syndrome (ureteral, orbital, gastrointestinal):
Yes
No

Pleural or peritoneal serous effusion (irrespective of cell content):
Yes
No

Leukemic phase (>5.0 x10⁹/L circulating malignant cells):
Yes
No

Cytopenia (granulocyte count < 1.0x10⁹/L and/or platelets < 100x10⁹/L):
Yes
No


Number of criteria Feasibility of GELF criteria Recommendation
0 0 0
Number of criteriaFeasibility of GELF criteriaRecommendation
000

This tool is best used as an instantaneous assessment of a patient’s disease burden. Ultimately, clinical judgement and comprehensive review of the patient’s previous workup are critical to make this assessment.

For overall prognosis, the FLIPI can risk stratify further than the GELF criteria.

The GELF criteria is easy to use with information most providers will already have available. Clinical evaluation, laboratory testing, and imaging are all that is needed to determine who has a high tumor burden based on the GELF criteria.

Any nodal or extranodal tumor mass >7 cm diameterNo0
 Yes+1
Involvement of at least 3 nodal sites, each with diameter >3 cm
View more nodal site information in the Nodal Sites section		No0
 Yes+1
Presence of any systemic or B symptomsNo0
 Yes+1
Splenic enlargement with inferior margin below the umbilical lineNo0
 Yes+1
Compression syndrome (ureteral, orbital, gastrointestinal)No0
 Yes+1
Pleural or peritoneal serous effusion (irrespective of cell content)No0
 Yes+1
Leukemic phase (>5.0 x10⁹/L circulating malignant cells)No0
 Yes+1
Cytopenia (granulocyte count < 1.0×10⁹/L and/or platelets < 100×10⁹/L)No0
 Yes+1

FORMULA

Patients must meet ≥1 criteria to be considered “high” tumor burden.

Сriteria:

  • Any nodal or extranodal tumor mass >7 cm diameter
  • Involvement of at least 3 nodal sites, each with diameter >3 cm
  • Presence of any systemic or B symptoms
  • Splenic enlargement with inferior margin below the umbilical line
  • Compression syndrome (ureteral, orbital, gastrointestinal)
  • Pleural or peritoneal serous effusion (irrespective of cell content)
  • Leukemic phase (>5.0 x109/L circulating malignant cells)
  • Cytopenia (granulocyte count < 1.0 x109/L and/or platelets < 100×109/L)

Nodal Sites

  • follicular lymphoma, follicular lymphoma therapy, tumor, follicular lymphoma detection, follicular lymphoma treatment, lymphoma treatment, lymphoma, B symptoms, nodal site, node, nodal sites, tumor, extranodal tumor, nodular tumor, calculator, medical calculators, medcalk, calculate online, estimate, online calculator, severity estimate, criteria, GELF

Refer to the staging systems FLIPI and Lugano classification to assist with risk stratifying patients. The GELF criteria is a quick test to determine which patients should be monitored versus treated now.

MANAGEMENT

Treatment is varied depending on the stage and tumor burden.

Stages I-II

  • Treatment options include:
    • Involved field radiotherapy (IFRT)
    • Clinical trials
    • Active surveillance

Stages III-IV or Bulky Stage II – asymptomatic

  • Treatment options include:
    • Clinical trials
    • Active surveillance

Stages III-IV or Bulky Stage II – with GELF/BNLI risk factors or high-risk FLIPI score

  • Treatment options include:
    • RiTUXimab + cyclophosphamide, vinCRIStine, DOXOrubicin, predniSONE
    • RiTUXimab alone
    • Bendamustine + riTUXimab
    • RiTUXimab + cyclophosphamide, vinCRIStine, predniSONE
    • Clinical trials

CRITICAL ACTIONS

There is continued controversy around when and how to treat follicular lymphoma, including in patients with relapsed/refractory disease. Questions to consider when making treatment decisions:

  • When to initiate therapy versus active surveillance
  • Deciding amongst various treatment options
  • Balancing efficacy and toxicities of treatment options
  • Determining what constitutes a complete response/endpoint of treatment
  • Finally, with follicular lymphoma, a provider should be aware of transformation to diffuse large B-cell lymphoma, which is a more aggressive disease and would require prompt treatment.

EVIDENCE APPRAISAL

The original study compared three different intervention arms: active surveillance until clinical progression, immediate treatment with an oral alkylating agent (prednimustine), or treatment with a biologic response modifier (interferon-alpha). 193 in total were enrolled. The overall response rate of deferred treatment was 70%, with prednimustine was 78%, and with interferon-alpha was 70%. Similarly, the overall survival time at 5 years was 78%, 70%, and 84%, respectively. This showed that delayed treatment was a viable option for such an indolent lymphoma. However, those with early progression warrant treatment sooner.

Register on our website right now to have access to more learning materials!

Subscribe to our pages:

LITERATURE
Brice P, Bastion Y, Lepage E, et al. Comparison in low-tumor-burden follicular lymphomas between an initial no-treatment policy, prednimustine, or interferon alfa: a randomized study from the Groupe d’Etude des Lymphomes Folliculaires. J Clin Oncol. 1997. 15:1110-7. https://pubmed.ncbi.nlm.nih.gov/9060552/
Solal-Céligny P, Lepage E, Brousse N, Tendler CL, Brice P, Haïoun C, Gabarre J, Pignon B, Tertian G,Bouabdallah R, Rossi JF, Doyen C, Coiffier B. DOXOrubicin-containing regimen with or without interferon alfa-2b for advanced follicular lymphomas: final analysis of survival and toxicity in the Groupe https://pubmed.ncbi.nlm.nih.gov/9667247/

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Сообщение Groupe d’Etude des Lymphomes Folliculaires (GELF) Criteria. Online calculator появились сначала на ClinCaseQuest.

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