MCL Primer
Post Date: 04/17/16
Hello everyone: Although I've posted much of this information in the content of this blog, I thought it appropriate to Re-Cap and Re-Post the brief Mantle Cell Lymphoma (MCL) 'primer' with some of the updates in treatments since I began this journey in 2010.
For those who'd like a quick-link/reference (Introduction) to Lymphomas, here is a link to the City of Hope website:
http://healthlibrary.cityofhope.org/34,17643-1
http://www.cityofhope.org/clinical-program/lymphoma
and another link to a good glossary of the terminology posted by the Lymphoma Association in the UK:
http://www.lymphomas.org.uk/
Glossary
http://www.lymphomas.org.uk/about-lymphoma/what-lymphoma/glossary
Here we go with re recap:
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Mantle
Cell Lymphoma
Overview
Lymphoma is the most common blood cancer. The two main forms of
lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). Lymphoma occurs
when cells of the immune system called lymphocytes, a type of white blood cell,
grow and multiply uncontrollably. Cancerous lymphocytes can travel to many
parts of the body, including the lymph nodes, spleen, bone marrow, blood, or
other organs, and form a mass called a tumor. The body has two main types
of lymphocytes that can develop into lymphomas: B-lymphocytes (B-cells) and
T-lymphocytes (T-cells).
Mantle
cell lymphoma (MCL) is a rare, B-cell NHL that most often affects men over the
age of 60. The disease may be aggressive (fast growing) but it can also behave
in a more indolent (slow growing) fashion in some patients. MCL comprises about
five percent of all NHLs. The disease is called "mantle cell
lymphoma" because the tumor cells originally come from the "mantle
zone" of the lymph node. MCL is usually diagnosed as a late-stage disease
that has typically spread to the gastrointestinal tract and bone marrow.
A
diagnosis of MCL requires taking a small sample of tumor tissue, called a
biopsy, and looking at the cells under a microscope. A blood test may also be
necessary to measure the white blood cell count and certain proteins, which
help to diagnose MCL. Other tests, such as a bone marrow biopsy and a computed
axial tomography (CAT) scan may be used to confirm a diagnosis and to determine
what areas of the body are involved by the cancer.
Overproduction
of a protein called Cyclin D1 is found in more than 90 percent of patients with
MCL. Identification of excess Cyclin D1 from a biopsy is considered a very
sensitive tool for diagnosing MCL. One-quarter to one-half of patients with MCL
also have higher than normal levels of certain proteins that circulate in the
blood, such as lactate dehydrogenase (LDH) and beta-2 microglobulin. Measuring
these and other proteins can help doctors determine how aggressive an
individual patient's MCL is and may guide therapy decisions.
Symptoms
Common
signs and symptoms of NHL include swelling of the lymph nodes (which is often
but not always painless), fever, night sweats, unexplained weight loss and lack
of energy. While most people who have these complaints will not have NHL,
anyone with persistent symptoms should be seen by a physician to make sure that
lymphoma is not present.
Risk Factors
The
causes of NHL remain unknown, however, risk for developing lymphoma may be
higher in individuals who:
·
have a family history of NHL (though no hereditary pattern has
been well established)
·
are affected with an autoimmune disease
·
have received an organ transplant
·
have been exposed to chemicals such as pesticides, fertilizers or
organic solvents for a long period
·
have been infected with viruses such as Epstein-Barr, human
T-lymphotropic virus type 1 (HTLV-1), HIV/AIDS, hepatitis C or certain
bacteria, such as H-pylori
Stages
Non-Hodgkin
lymphoma is divided into four stages based on how far the disease has spread.
·
Stage I (early disease): the cancer is found only in a single lymph node OR in one organ or
area outside the lymph node.
·
Stage II (locally advanced
disease): the
cancer is found in two or more lymph node regions on one side of the diaphragm.
·
Stage III (advanced disease): the cancer involves lymph nodes both above and below the
diaphragm.
·
Stage IV (widespread disease): the cancer is found in several parts of one or more organs or
tissues (in addition to the lymph nodes). Or, it is in the liver, blood or bone
marrow.
Treatment Options
The type
of treatment selected for a patient with MCL depends on multiple factors,
including the stage of disease, the age of the patient, and the patient's
overall health. For the subset of patients who do not yet have symptoms and who
have a relatively small amount of slow growing disease, "watchful
waiting" and monitoring the disease for progression may be an acceptable
option. MCL is usually diagnosed once it has spread throughout the body, and
the majority of these patients will require treatment. Initial treatment
approaches for aggressive MCL in younger patients include combination
chemotherapy, typically in combination with the monoclonal antibody rituximab
(Rituxan), as first-line treatment, followed by autologous stem cell transplant
(in which patients receive their own stem cells).
HyperCVAD (cyclophosphamide,
vincristine, doxorubicin, dexamethasone alternating with methotrexate and cytarabine)
plus rituximab are recommended as aggressive induction therapy and are
associated with durable remissions in newly diagnosed patients For older
patients, chemotherapy followed by a prolonged course of rituximab alone, known
as maintenance, is often recommended. A common chemotherapeutic treatment
approach used to treat MCL is called R-CHOP, which combines rituximab,
cyclophosphamide, doxorubicin, vincristine, and prednisone. Bendamustine
(Treanda) in combination with rituximab is another common first-line treatment
option. Several additional intensified chemotherapy combinations are also used
in combination with rituximab, particularly in younger patients.
Although
high-dose chemotherapy followed by allogeneic (in which patients receive stem
cells from a donor) stem cell transplantation is very intensive and causes
various side effects, it may increase response times for selected younger
patients.
Bortezomib
(Velcade) is approved by the U.S. Food and Drug Administration for the
treatment of MCL patients who have received at least one prior therapy. In
October 2014, bortezomib for injection was approved for previously untreated
patients with MCL. Bortezomib is the first treatment in the United States to be
approved for use in previously untreated patients with MCL; for more
information, please visit: Bortezomib (Velcade)
On June
5, 2013, the U.S. Food and Drug Administration (FDA) approved lenalidomide
(Revlimid) for the treatment of patients with mantle cell lymphoma (MCL) whose
disease has relapsed or progressed after two prior therapies.
In
November 2013, ibrutinib (Imbruvica) was approved to treat patients with Mantle
Cell lymphoma who have received at least one prior therapy. For complete
details about this approval, please visit: http://www.fda.gov/drugs/informationondrugs/approveddrugs/ucm374857.htm.
Treatment options may change as new treatments are discovered and current
treatments are improved. Therefore, it is important for patients to discuss
current treatment options with their physician.
Treatments Under Investigation
Many new
drugs used alone or in combination are being studied in clinical trials for
MCL, including idelalisib (GS-1101, formerly CAL-101), vorinostat (Zolinza),
ofatumumab (Arzerra), everolimus (Afinitor), panobinostat, and temsirolimus
(Torisel).
Clinical Trials
Clinical
trials are crucial for identifying effective drugs and combinations for
lymphoma patients. Because the optimal initial treatment of MCL is not clear
and is such a rare disease, clinical trials are very important and will
identify the best treatment options in this disease. Patients interested in
participating in a clinical trial should talk to their physician or contact
LRF's Helpline for an individualized clinical trial search by calling (800)
500-9976 or emailinghelpline@lymphoma.org.
Follow Up
Patients
in remission should have regular visits with a physician who is familiar with
their medical history as well as with the treatments they have received.
Medical tests (such as blood tests and CAT scans) may be required at various
times during remission to evaluate the need for additional treatment.
Some
treatments can cause long-term effects or late effects, which can vary based on
duration and frequency of treatments, age, gender, and the overall health of
each patient at the time of treatment. The doctor will check for these effects
during follow-up care.
Survivors
and their caregivers are encouraged to keep copies of all medical records and
test results as well as information on the types, amounts, and duration of all
treatments received. This documentation will be important for keeping track of
any effects resulting from treatment or potential disease recurrences. For
further information, please review our fact sheet on survivorship issues.
Support
A
lymphoma diagnosis often triggers a range of feelings and concerns. In
addition, cancer treatment can cause physical discomfort. Support groups and
online message boards can help patients connect with other people who have
lymphoma. One-to-one peer support programs, such as LRF's Lymphoma Support Network,
match lymphoma survivors (or caregivers) with volunteers who have gone through
similar experiences.
Resources
LRF
offers a wide range of resources that address treatment options, the latest
research advances and ways to cope with all aspects of lymphoma. LRF also
provides many educational activities, from in-person meetings to
teleconferences and webcasts. For more information about any of these
resources, visit the website at www.lymphoma.org or www.FocusOnMCL.org.
You can also contact the Helpline at (800) 500-9976 or helpline@lymphoma.org.
Lymphoma Helpline
Professional
staff members are available to answer your questions and provide individual
support to you and your loved ones. Contact our Helpline, available Monday
through Friday from 8:00am - 5:00pm Pacific Standard Time (PST). Call (800)
500-9976 or e-mail helpline@lymphoma.org.
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·
Mantle Cell Lymphoma – Peter Martin, MD (2015) View webcast
