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Chronic Lymphocytic Leukemia/Small
Lymphocytic Lymphoma (CLL/SLL)
Lymphoma is the most common blood cancer. The two main forms
Treatment is based on the severity of associated symptoms as well
of lymphoma are Hodgkin lymphoma (HL) and non-Hodgkin
as the rate of cancer growth. Since CLL grows slowly, doctors may
lymphoma (NHL). Lymphoma occurs when lymphocytes, a type
decide not to treat it right away, an approach referred to as “watch
of white blood cel , grow abnormal y. The body has two main types
and wait” or “watchful waiting.” Studies have shown that patients
of lymphocytes that can develop into lymphomas: B-lymphocytes
treated early in their disease course, compared to those fol owed
(B-cel s) and T-lymphocytes (T-cel s). Cancerous lymphocytes can
with a “watch and wait” approach, exhibit similar overal survival.
travel to many parts of the body, including the lymph nodes,
The treatment options available for CLL patients include:
spleen, bone marrow, blood or other organs, and can accumulate
is treatment with medication that circulates
throughout the body. Two types of chemotherapy most often
Chronic lymphocytic leukemia (CLL) and smal lymphocytic
lymphoma (SLL) are B-cel NHLs. CLL and SLL are essential y the
• Purine nucleoside analogues: cladribine (Leustatin),
same disease with slightly different manifestations. The only
fludarabine (Fludara) and pentostatin (Nipent)
difference is where the cancer primarily occurs. When the cancer
• Alkylating agents: chlorambucil (Leukeran), cyclophos-
cel s are located mostly in the lymph nodes, the disease is cal ed
phamide (Cytoxan) and bendamustine (Treanda). Pred-
SLL. When most of the cancer cel s are located in the bloodstream
nisone (a steroid) is often given together with chlorambu-
and the bone marrow, the disease is referred to as CLL, although
the lymph nodes and spleen are often involved.
can recognize specific proteins on the
CLL tends to be an indolent (slow-growing) cancer. However,
surface of lymphoma cel s and destroy them. Three commonly
over time, it can progress to a more aggressive type of lymphoma.
Approximately 15,000 new cases of CLL are diagnosed annual y.
• Alemtuzumab (Campath) has been approved for use in
Common signs of disease include a swel ing of the liver and spleen
patients with advanced CLL who are no longer responding
and enlargement of the lymph nodes in the neck, underarm,
stomach or groin. Other symptoms of CLL can include, fatigue, shortness of breath, anemia, bruising, night sweats, weight loss
• Ofatumumab (Arzerra) received approval for the treatment
and frequent infections. Oftentimes, people with CLL have no
of patients with CLL whose disease is refractory to
obvious symptoms of the disease at diagnosis.
fludarabine (Fludara) and alemtuzumab (Campath).
• Rituximab (Rituxan) was approved in combination with
Approximately one-third of al CLL patients wil live for years—and
fludarabine and cyclophosphamide for patients with
even decades—without symptoms. Another one-third wil require
untreated or previously treated CD20-positive CLL.
therapy immediately or wil be symptomatic within three to five years, requiring treatment. Another one-third wil experience
Stem cel transplantation
is a treatment option typical y reserved
intermediate disease progression in which the cancer is dormant
for patients whose CLL does not respond to standard therapies.
and then becomes active, but wil respond to treatment.
Patients, whose disease has transformed into a more aggressive
form, could potential y benefit from a stem cel transplant. In CLL, the patient’s own stem cel s (autologous transplant) are rarely used. Typical y, stem cel s from a donor are used (al ogeneic transplant), most commonly in a reduced intensity al ogeneic transplant (sometimes referred to as
a “mini-al ogeneic transplant”).
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Common combination drug regimens used to treat CLL include:
• R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
• FCR (fludarabine, cyclophosphamide, rituximab)
Website: lymphoma.orgEmail: LRF@lymphoma.org
• BCR (bendamustine, cyclophosphamide, rituximab) • PCR (pentostatin, cyclophosphamide, rituximab)
For some patients, CLL does not return after initial treatment. However, for many patients the
disease does return. For patients who become refractory (disease does not respond to treatment)
offers the fol owing patient education and support programs:
or relapse (disease returns after treatment), secondary therapies may be successful in providing
another remission. Some common single-agent therapies used in the relapsed setting include:
• Chlorambucil (Leukeran) • Ofatumumab (Arzerra)
• Fludarabine (Fludara) • Bendamustine (Treanda)
Some common combination treatment regimens used in the relapsed or refractory setting include:
• R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) • FR (fludarabine, rituximab)
• FCR (fludarabine, cyclophosphamide, rituximab)
• R-CVP (cylcophosphamide, vincristine, prednisone)
Treatments Under Investigation
Many treatments are currently being tested in clinical trials for both newly diagnosed and relapsed/refractory CLL patients. For a listing of treatments under investigation, visit the Lymphoma Research Foundation’s website (lymphoma.org) or order the publication entitled
Understanding CLL/SLL: A Guide for Patients, Survivors and Loved One
. It is critical to remember
that today’s scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, it is important that
patients check with the Lymphoma Research Foundation or their physician for any treatment updates that may have recently emerged.
Participating in Clinical Trials
Clinical trials are crucial in identifying effective drugs and determining optimal doses for
lymphoma patients. Patients interested in participating in a clinical trial should talk to their
physician. Contact the Lymphoma Research Foundation’s Helpline
for an individualized clinical
trial search by cal ing (800) 500-9976 or emailing email@example.com.
Getting the Facts
is published by the Lymphoma Research Foundation (LRF) for the
purpose of informing and educating readers. Because each person’s body and response to
The Lymphoma Research Foundation offers a wide range of resources that address treatment
treatment is different, no individual should
options, the latest research advances and coping with al aspects of lymphoma. The Foundation
self-diagnose or embark upon any course of medical treatment without first consulting
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 lymphoma.org,
responsible for the medical care or treatment of any individual.
e-mail the Helpline
at firstname.lastname@example.org or cal at (800) 500-9976.
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