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Types of Lymphoma > Primary Mediastinal B-cell lymphoma

Last update: 01/03/2014

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Background

"Primary Mediastinal B-cell lymphoma (PMBL/PMBCL) is a less common subtype of diffuse large B-cell lymphoma that arises in the thymus and mainly affects young adults.  

Molecular diagnosis of Primary Mediastinal B Cell Lymphoma has identified it as a clinically favorable subgroup of Diffuse Large B Cell Lymphoma related to Hodgkin Lymphoma 2

Sometimes PMBCL is diagnosed with sclerosis, which means the tumor is mixed with fibrous tissue. "The sclerosis does not indicate the prognosis is worse (or better). A negative gallium scan is a favorable sign. In the medical literature, cure rates for this disease range from 38-88%."  Source: bcm.edu 

See WHO Classification

In the News

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N Engl J Med 2013: Dose-Adjusted EPOCH-Rituximab Therapy in Primary Mediastinal B-Cell Lymphoma http://bit.ly/ZbiSMK 

A "phase 2, prospective study of dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine, prednisone, and rituximab (DA-EPOCH-R) and filgrastim  (a blood cell growth factor) without radiotherapy in 51 patients with untreated primary mediastinal B-cell lymphoma."

DA-EPOCH-R obviated the need for radiotherapy in patients with primary mediastinal B-cell lymphoma compared to retrospective data.

(Funded by the National Cancer Institute; ClinicalTrials.gov number, NCT00001337.)

Incidence

Mediastinal large B-cell lymphoma is a rare form of non-Hodgkin's lymphoma, accounting for about 1 in 50 of all cases,

"In REAL classification PMBL is considered a variant of diffuse large B cell lymphoma." "PMBL accounts for about 2 percent of all non Hodgkins lymphoma." 1

Because of its skewed age distribution, PMBL accounts for a much higher proportion of both younger patients and those undergoing autologous transplantation. Uncertainty remains regarding the management of this disorder."  7

Diagnosis   

To make an accurate diagnosis of lymphoma, a biopsy must be performed by the surgical removal (resection) of a lymph node.   If the lymph nodes within the mediastinum are the only ones affected, the biopsy is likely to be taken during a procedure known as a mediastinoscopy.

A series of tests will then be performed to determine the characteristics of the cells.  If a malignancy is determine, these characteristics will allow your doctors to determine the appropriate treatments to use when needed. 

Gene expression profile in PMBCL

Primary Mediastinal large B cell lymphoma (PMBCL) has been thought of as a special subtype of DLBCL. Its distinct clinical presentation in younger patients with a female predominance has led to the suspicion that it constitutes a unique entity. However, reliable distinction from DLBCL has remained elusive. ...

SOURCE: Improving Diagnosis and Treatment of Lymphomas with Gene Expression Profiling, Joseph M. Connors, M.D.   hematology.org  
 
"Major new insights into the biology of lymphomas have resulted from the use of microarray gene expression technology to elucidate their underlying biology and to identify novel pathways for therapeutic intervention."

Common symptoms

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breathlessness*

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cough or chest pain*

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fatigue

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loss of appetite

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enlarged lymph nodes - painless swelling in the neck, armpit or groin - often in more than one group.

Other symptoms may include night sweats, unexplained high temperatures and weight loss. These are known as B symptoms.

Resources on PMBL

  1. Primary mediastinal B cell lymphoma (PMBL) in two sisters Year: 2002 Abstract No: 2646
  2. Molecular Diagnosis of Primary Mediastinal B Cell Lymphoma Identifies a Clinically Favorable Subgroup of Diffuse Large B Cell Lymphoma Related to Hodgkin Lymphoma  jem.org 9_15_03
  3. Molecular Diagnosis of Primary Mediastinal B Cell Lymphoma Identifies a Clinically Favorable Subgroup of Diffuse Large B Cell Lymphoma Related to Hodgkin Lymphoma  jem.org 9_15_03
  4. Primary Mediastinal Large B Cell Lymphoma: Elucidating Optimal Therapy and Prognostic Factors; an Analysis in 141 Consecutive Patients Treated at Memorial Sloan Kettering from 1980-1999. Session Type: Oral Session  ASH 2004
  5. Lymphomas: Non-Hodgkin Lymphoma  Medscape (free login req.)
  6. About Mediastinal large B-cell lymphoma  Cancerbackup
  7. About Mediastinal large B-cell lymphoma  Emedicine
  8. Technical background on Primary Mediastinal B-Cell Lymphoma asheducationbook.hematologylibrary.org 

Treatment for PMBCL

TOPIC SEARCH ASCO | Medscape | PubMed 

" ... Some patients with early-stage primary mediastinal DLBCL (PMBCL), however, may require involved-field radiation after chemotherapy. 

In a study of 50 patients with untreated PMBCL who received MACOP-B (methotrexate with leucovorin rescue, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin) followed by radiation, 66% had a persistently positive gallium scan after chemotherapy alone, suggesting active disease.136 

However, treatment of PMBCL with other regimens that do not include involved-field radiation, such as ACVBP and DA-EPOCH, have yielded good results in PMBCL.71,135 Furthermore, rituximab has improved the outcome in DLBCL.79 Hence, it is reasonable to limit involved-field radiation to those patients with persistently positive PET scans after such treatments.  

- Medscape  06/07/2006 (source no longer available)

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Primary Mediastinal Large B-Cell Lymphoma theoncologist.alphamedpress.org
Kerry J. Savage
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Outcome of Primary Mediastinal Large B-Cell Lymphoma: Presented at ICML Docguide June 17, 2005

Dose-intensive chemotherapy treatment regimens that do not need to be associated with involved-field radiotherapy (IF-RT) can promote excellent survival rates and low levels of relapse for patients with primary mediastinal large B-cell lymphoma (PMBCL).
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Primary mediastinal large B-cell lymphoma: optimal therapy and prognostic factor analysis in 141 consecutive patients treated at Memorial Sloan Kettering from 1980 to 1999. Br J Haematol. 2005 Sep;130(5):691-9. PMID: 16115124 

We conclude: (i) dose-dense chemotherapy with NHL-15 may be superior to CHOP for PMLBL; (ii) The impact of consolidative radiotherapy requires randomized controlled trials; (iii) The age-adjusted IPI did not predict survival in this analysis; (iv) high-dose chemotherapy/ASCT should be reserved for upfront anthracycline-based therapy failure or in clinical trials for high-risk patients.
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FULL TEXT: Primary mediastinal large B-cell lymphoma (PMLBCL): long-term results from a retrospective multicentre Italian experience in 138 patients treated with CHOP or MACOP-B/VACOP-B. Br J Cancer. 2004 Jan 26;90(2):372-6. PMID: 14735179   full text http://bit.ly/bQom4f 

In our experience, MACOP-B/VACOP-B appears to positively influence OS and EFS in patients affected by PMLBCL, as compared to CHOP. Consolidation IF-RT on mediastinum further improves the outcome of CR patients.
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Combined therapy in the treatment of primary mediastinal B-cell lymphoma: conventional versus escalated chemotherapy.
Ann Hematol. 2002 Jul;81(7):368-73. Epub 2002 Jun 21. PMID: 12185505 

We feel that patients with PMBCL should be treated with more intensive, but not myeloablative chemotherapy, followed by adjuvant radiotherapy to achieve an improvement in outcome in this setting of patients. Patients with pleural or pericardial effusion are considered at high risk for failure with the actual programs of treatment and probably will be considered for experimental therapeutic approaches.
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Induction chemotherapy strategies for primary mediastinal large B-cell lymphoma with SCLEROSIS: a retrospective multinational study on 426 previously untreated patients  http://www.haematologica.org/cgi/reprint/87/12/1258
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Dose-dense and high-dose chemotherapy plus rituximab with autologous stem cell transplantation for primary treatment of diffuse large B-cell lymphoma with a poor prognosis: a phase II multicenter study http://bit.ly/aAmY77
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Primary Diffuse Large B-Cell Lymphoma of the Mediastinum:
Outcome Following High-Dose Chemotherapy and Autologous Hematopoietic Cell Transplantation http://bloodjournal.hematologylibrary.org

Laurie H. Sehn, Joseph H. Antin, Lawrence N. Shulman, Peter Mauch, Anthony Elias, Marshall E. Kadin, and Catherine Wheeler

 

Clinical Trials for PMBCL

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