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
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
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Primary mediastinal B cell lymphoma (PMBL) in two sisters
Year: 2002 Abstract No: 2646
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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
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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
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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
Lymphomas: Non-Hodgkin Lymphoma Medscape
(free login req.)
About Mediastinal large B-cell lymphoma Cancerbackup
About Mediastinal large B-cell lymphoma Emedicine
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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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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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Clinical Trials for PMBCL
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