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Types of Lymphoma > Aggressive versus Indolent Lymphomas

Last update: 02/01/2013

Lymphomas are a family of blood cell cancers which are classified or grouped 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 - but also aggressive or indolent.

When a lymphocyte becomes malignant it's maturation stage 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), an increasingly by the genetic expression.

 

Indolent Lymphomas

CLL/SLL
Chronic Lymphocytic Lymphoma  

Marginal Zone:
MALTBALT  / Cutaneous
Extranodal / Eye / Splenic

Follicular b-cell
 most common indolent type 

Waldenstrom's
Macroglobulinemia

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

 

What's New

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Hematopathology Approaches to Diagnosis and Prognosis of Indolent B-Cell Lymphomas  asheducationbook.org 2005 

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
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Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma 
CLL/SLL

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Follicular lymphoma
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follicular small cleaved cell

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follicular mixed small cleaved and large cell 

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Diffuse small cleaved cell

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Hairy-cell leukemia  

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Lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia

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Marginal zone - MALT (extranodal)

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Marginal zone - Nodal 

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Splenic lymphoma with villous lymphocytes 
(splenic marginal zone lymphoma)  

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Waldenström’s Macroglobulinemia - Lymphoplasmacytic lymphoma

T-cell types
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Mycosis fungoides/Sézary syndrome

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T-Cell CLL

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

Indolent Lymphomas
Treatment Resources

Treatment is often deferred until the patient becomes symptomatic. The 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
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Indolent, Stage I and Contiguous Stage II Adult Non-Hodgkin’s Lymphoma  Cancer.gov
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Indolent, Noncontiguous Stage II/III/IV Adult Non-Hodgkin’s Lymphoma   Cancer.gov
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Indolent, Recurrent Adult Non-Hodgkin’s Lymphoma  Cancer.gov
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Low grade Lymphoma  asheducationbook.org /2004 full text 
Related Articles:
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Related resources: Chemotherapy | Refractory disease | Targeted and Immune-based | Transplants
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Treatment approaches, overview for indolent and aggressive  MSKCC
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Early stage localized disease?  See Radiotherapy
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Lymphoma Diagnosis and Treatment: CHOP, MALT, PET, and More  Medscape (free login, req.)
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Lymphomas: Lessons in Overcoming Indolence,  Levine  Medscape
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Factors that determine treatment and timing
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Follicular Lymphoma, Treatment Policy - Dr. Louise Bordeleau  PDF | PDF-Help
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Strategy for treating indolent NHL - FDA Flow chart
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The Indolent Lymphomas - COMPREHENSIVE REVIEW,  MEDICAL ONCOLOGY: A - Ali W. Bseiso, MD Peter McLaughlin, MD
  • Treatment overview - Best Practices of Medicine for NHL & HD  Merck Medicus
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    Treatment Sequencing of Therapies for Low-Grade Lymphomas  cancercare.org 
    Presenter: Peter Rosen, MD, Professor of Medicine, UCLA School of Medicine, Department of Hematology and Oncology, Los Angeles, CA.
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    Watch & Wait background and treatment consideration for pts with indolent NHL

    Aggressive Lymphomas

    Related PubMed abstracts - 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 
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    AIDS-associated lymphoma
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    Large noncleaved cell lymphomas

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    Large cell immunblastic, plasmacytoid

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    Small noncleaved cell

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

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    Primary Mediastinal large B-cell

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    Diffuse large cell lymphoma
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    diffuse mixed cell

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    diffuse large cell

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

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    Central nervous system (CNS) lymphoma

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    Large Cell Immunoblastic

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    Lymphoblastic lymphoma 

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    Mantle-cell lymphoma
    (Sometimes behaves indolently)

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    Post-transplantation Lymphoproliferative disorder

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    T-cell types - Also see T-cell subtypes
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    Adult T-cell leukemia/lymphoma

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    Angioimmunoblastic

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    Anaplastic large cell (T-cell/null cell)

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    Lymphoblastic lymphoma/leukemia   

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    Precursor T-cell

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    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
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    Adult wide spread, stage III/IV - standard of care  Cancer.gov
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    Adult, localized, stage I/II - standard of care  Cancer.gov
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    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:
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    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
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    Antibody Therapy in Aggressive Lymphomas  asheducationbook.hematologylibrary.org 
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    Therapeutic approaches according to REAL/WHO classification  ncbi.nlm.nih.gov/ 
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    Prophylactic intrathecal methotrexate and hydrocortisone reduces central nervous system recurrence and improves survival in aggressive non-Hodgkin lymphoma  http://cat.inist.fr/
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    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.
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    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."
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    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
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    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
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    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
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    Intensive Treatment More Effective Than CHOP for aggressive NHL  CancerConsultants.com 3/04
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    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 
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    Treating Aggressive Non-Hodgkin's Lymphoma -  Non-Hodgkin's Lymphoma: Where Do We Stand Today? - John D. Hainsworth, MD - Medscape  2003 (free login req.)
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    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
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    Follicular grade 3 lymphoma - related abstracts
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    Related resources: Chemotherapy | Refractory disease | Targeted and Immune-based | Transplants
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    Treatment approaches, overview for indolent and aggressive  MSKCC
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    High-Dose Therapy for Follicular Lymphoma  cancernetwork  
    Arnold Freedman, MD, Jonathan W. Friedberg, MD , and John Gribben, MD, PhD
    Department of Medicine, Harvard Medical School, Dana-Farber Cancer Institute, Boston, Massachusetts
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    Lymphoma Diagnosis and Treatment: CHOP, MALT, PET, and More  Medscape (free login, req.)
  • Treatment overview - Best Practices of Medicine for NHL & HD  Merck Medicus

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    Disclaimer:  The information 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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