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Aggressive and Indolent Lymphomas

  

About Lymphoma > Types of Lymphoma > Aggressive and Indolent Lymphomas

Last update: 07/25/2008

Lymphomas, a cancer of immune cells called lymphocytes, are classified in many ways.  This page groups lymphomas by how fast or slow they tend to grow - often referred to as the grade: high grade or low grade. 

When a lymphocyte becomes malignant it's maturation stage -- and there are many more than depicted below -- is arrested (stopped) at that stage, and it's behavior (such as fast growing, refusing to die) is determined, in part, by the behavior of it's normal counterpart.  

Lymphomas are further categorized by the shape of the cells, how the cells cluster (diffuse vs. follicular), as well as genetic expression that controls all of the above.

 
Overview - About Lymphomas

Overview of genes and cancer |  Lymphoma is a cancer 

About Lymphoma - general
| Characteristics of lymphomas   Cell type | Histology | Grading | Staging

 Ann Arbor Staging | Extranodal notations 

Host/tumor
interaction 

Lymphatic System | Prognostic Indicators | Risk Factors | Statistics | Symptoms | Transformation

 Guidelines at diagnosis | Treatment Decisions | Watch & Wait
  
Indolent Lymphomas

TOPIC SEARCH - PubMed: Diagnosis | Review | Therapies | Prognosis

Generally describes low grade - slow growing-- lymphomas

Indolent lymphomas can progress steadily - behave aggressively.

Cellular Classifications
Cancer.gov

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B-cell types

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma 
CLL/SLL

Follicular lymphoma

follicular small cleaved cell

follicular mixed small cleaved and large cell 

Diffuse small cleaved cell

Hairy-cell leukemia  

Lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia

Marginal zone - MALT (extranodal)

Marginal zone - Nodal 

Splenic lymphoma with villous lymphocytes 
(splenic marginal zone lymphoma)  

Waldenström’s Macroglobulinemia - Lymphoplasmacytic lymphoma

T-cell types

Mycosis fungoides/Sézary syndrome

T-Cell CLL

T-Cell - Large granular leukemia/lymph (T-cell/NK cell)

Indolent Lymphomas
Treatment Resources

Treatment is often deferred until the patient becomes symptomatic. Goal of treatment is often management as indolent lymphomas are rarely cured, unless it is diagnosed when still localized. Treatment options are more varied -- there is no standard treatment.  

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Treatment - Standard of care
Indolent, Stage I and Contiguous Stage II Adult Non-Hodgkin’s Lymphoma  Cancer.gov
Indolent, Noncontiguous Stage II/III/IV Adult Non-Hodgkin’s Lymphoma   Cancer.gov
Indolent, Recurrent Adult Non-Hodgkin’s Lymphoma  Cancer.gov
Low grade Lymphoma  asheducationbook.org /2004 full text 

In Section I, Dr. Randy Gascoyne describes the histologic, cytogenetic and biologic features of FL that underlie its clinical variability. Key aspects of the pathologic diagnosis of FL that have particular relevance to the clinician are highlighted. A proposed model for follicular lymphomagenesis and diffuse large B cell lymphoma transformation has emerged and continues to evolve as the molecular story unfolds. A biologic basis for clinical outcome in FL also appears to be forthcoming.  
 
In Section II, Dr. Jane Winter addresses the complex process of selecting among the many treatment options for patients with FL. Previously a simple matter of deciding between oral or intravenous alkylators, clinicians and patients must now struggle to choose among vastly different approaches ranging from "watch and wait" to stem cell transplantation. The introduction of rituximab and radioimmunoconjugates is changing the treatment paradigm, but the optimal approach to integrating these and other new agents remains to be determined. At every decision point, the best approach is always a clinical trial. 
 
In Section III, Dr. Koen Van Besien provides a well-documented update on outcomes associated with autologous and allogeneic stem cell transplantation for FL. The results of trials of autologous stem cell transplantation in first remission and recent data supporting a role for graft purging are discussed. Based on the premise that a graft-versus-lymphoma effect is operative in FL, reduced-intensity allogeneic transplantation is the preferred approach in many cases, and recently reported results are summarized. Criteria for patient selection and the optimal role of transplantation in the overall therapeutic plan for the patient with FL are presented.
Related Articles
Related resources  Chemotherapy | Refractory disease | Targeted and Immune-based | Transplants
Treatment approaches, overview for indolent and aggressive  MSKCC
Early stage localized disease?  See Radiotherapy
Lymphoma Diagnosis and Treatment: CHOP, MALT, PET, and More  Medscape (free login, req.)
Lymphomas: Lessons in Overcoming Indolence,  Levine  Medscape
Factors that determine treatment and timing
Follicular Lymphoma, Treatment Policy - Dr. Louise Bordeleau  PDF | PDF-Help
Strategy for treating indolent NHL - FDA Flow chart
Hematopathology Approaches to Diagnosis and Prognosis of Indolent B-Cell Lymphomas  asheducationbook.org 2005
Aggressive Lymphomas

Related PubMed abstracts for last year - Diagnosis | Review | Therapies | Prognosis

Generally describes  intermediate and high grade - fast growing - lymphomas

Sometimes, perhaps rarely, types of lymphomas expected to be aggressively can progress slowly - behave indolently. 

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B-cell types 

AIDS-associated lymphoma

Large noncleaved cell lymphomas

Large cell immunblastic, plasmacytoid

Small noncleaved cell

Adult T-cell leukemia/lymphoma (HTLV-1+)

Primary Mediastinal large B-cell

Diffuse large cell lymphoma

diffuse mixed cell

diffuse large cell

Burkitt's lymphoma/diffuse small non-cleaved cell lymphoma

Central nervous system (CNS) lymphoma

Large Cell Immunoblastic

Lymphoblastic lymphoma 

Mantle-cell lymphoma
(Sometimes behaves indolently)

Post-transplantation Lymphoproliferative disorder

T-cell types - Also see T-cell subtypes

Adult T-cell leukemia/lymphoma

Angioimmunoblastic

Anaplastic large cell (T-cell/null cell)

Lymphoblastic lymphoma/leukemia   

Precursor T-cell

Peripheral T-cell

Aggressive Lymphomas
Treatment Articles

Lymphoma is a type of blood cancer, and as such is rarely localized to one tumor. The cells (even if only a few) are likely to also be in adjacent lymph nodes, in the blood, and or in the bone marrow. 

The good news is that as such, unlike so-called solid cancers, even wide spread disease can be treated effectively or cured with chemotherapy and radiotherapy. 

The goal of treatment is to cure an aggressive lymphoma, and it is often achieved. Removing only the tumor with surgery would be under-treating the disease and would almost certainly do little to change the course of the disease. 

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Treatment - Standard of care
Adult wide spread, stage III/IV - standard of care  Cancer.gov
Adult, localized, stage I/II - standard of care  Cancer.gov
Adult, Aggressive, Recurrent Non-Hodgkin’s Lymphoma
standard of care  Cancer.gov

Autologous Stem Cell Transplantation for Relapsed Aggressive NHL

"The disease sensitivity at the time of autologous stem cell transplantation (ASCT) has remained the most significant prognostic variable for predicting treatment outcome.356 --359 Several large series have shown that patients who undergo ASCT when the disease is resistant to the initial induction therapy have less than 10% probability of disease-free survival. Although many patients die of progressive lymphoma, in some studies the treatment-related mortality has been higher in this patient population (20% to 30%). Those patients in sensitive relapse have a 30% to 60% probability of long-term disease-free survival. In contrast, 10% to 20% of patients with resistant disease are long-term survivors."  ncbi.nlm.nih.gov

 
Related Articles
Outcomes: Dose-escalated CHOP and Tailored Intensification with IFE According to Early Response in Poor Risk Agressive B Cell Lymphoma: A Prospective Study from the GEL TAMO Study Group.  Abstract
No different outcomes were observed between patients achieving an early negative Ga (67)S response treated with MegaCHOP and BEAM/ ASCT and patients with midtreatment positive Ga (67)S who received IFE prior BEAM/ ASCT. Conclusions: This response adapted strategy including early treatment modifications prior HDT/ ASCT have yielded encouraging PFS and OS in patients with poor risk aggressive NHL.
Antibody Therapy in Aggressive Lymphomas  asheducationbook.hematologylibrary.org 

Targeted therapies with monoclonal antibodies and monoclonal antibodies conjugated to radioimmunoconjugates have altered the natural history and the approach to treatment of aggressive non-Hodgkin lymphomas.
Therapeutic approaches according to REAL/WHO classification  ncbi.nlm.nih.gov
Prophylactic intrathecal methotrexate and hydrocortisone reduces central nervous system recurrence and improves survival in aggressive non-Hodgkin lymphoma  http://cat.inist.fr
Rituximab-CHOP-ESHAP vs CHOP-ESHAP-high-dose therapy vs conventional CHOP chemotherapy in high-intermediate and high-risk aggressive non-Hodgkin's lymphoma.
Leuk Lymphoma. 2006 Jul;47(7):1306-14. PMID: 16923561 

It is concluded that rituximab-ESHAP-CHOP is superior over standard CHOP and fares comparably to upfront HDT/ASCT in previously untreated patients with aggressive lymphoma. A prospective randomized controlled trial is warranted to confirm these results.
Combination chemotherapy with adriamycin, cyclophosphamide, vincristine, methotrexate, etoposide and dexamethasone (ACOMED) followed by involved field radiotherapy induces high remission rates and durable long-term survival in patients with aggressive malignant non-Hodgkin's lymphomas: long-term follow-up of a pilot study. Leuk Lymphoma. 2005 Dec;46(12):1729-34. PMID: 16353313

"After a median observation time of 10 years and 2 months, 16/22 (73%) patients are alive in continuous complete response without evidence of any late toxicities."
Mitoxantrone, carboplatin, cytosine arabinoside, and methylprednisolone followed by autologous peripheral blood stem cell transplantation (MiCMA): a salvage regimen for patients with refractory or recurrent non-Hodgkin lymphoma. Cancer. 2006 Feb 15;106(4):859-66. PMID: 16419074
Four versus six courses of a dose-escalated cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen plus etoposide (megaCHOEP) and autologous stem cell transplantation: early dose intensity is crucial in treating younger patients with poor prognosis aggressive lymphoma.
Cancer. 2006 Jan 1;106(1):136-45. PMID: 16331635
Concurrent administration of high-dose rituximab before and after autologous stem-cell transplantation for relapsed aggressive B-cell non-Hodgkin's lymphomas. J Clin Oncol. 2005 Apr 1;23(10):2240-7. PMID: 15800314
Intensive Treatment More Effective Than CHOP for aggressive NHL  CancerConsultants.com 
ACVBP regimen vs. CHOP in the treatment of advanced aggressive non-hodgkin's lymphoma (NHL). Results of the LNH93-5 study with a median follow-up of 5 years.  Abstract No: 2307 
Treating Aggressive Non-Hodgkin's Lymphoma -  Non-Hodgkin's Lymphoma: Where Do We Stand Today? - John D. Hainsworth, MD  Medscape  2003 (free login req.)
Localized aggressive NHL - intent is cure: Chemotherapy alone compared with chemotherapy plus radiotherapy for localized intermediate- and high-grade non-Hodgkin's lymphoma. N Engl J Med. 1998 Jul 2;339(1):21-6. PMID: 9647875  PubMed | Related abstracts
Follicular grade 3 lymphoma - related abstracts
Related resources  Chemotherapy | Refractory disease | Targeted and Immune-based | Transplants
Treatment approaches, overview for indolent and aggressive  MSKCC
Lymphoma Diagnosis and Treatment: CHOP, MALT, PET, and More  Medscape (free login, req.)
Treatment overview - Best Practices of Medicine for NHL & HD  Merck Medicus
 
Disclaimer:  The information presented on Lymphomation.org is not intended to be a substitute for 
professional medical advice or to replace your relationship with a physician.
For all medical concerns,  you should always consult your doctor. 
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