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Lymphomas- staging, management, treatment

STAGING OF LYMPHOMAS

Ann Arbor staging classification scheme is a common one for Lymphomas. It is based on the results of imaging studies and related tests that reveal the extent of the cancer involvement.

HL is often described as being “bulky” or “nonbulky.” Nonbulky means the tumor is small; bulky means the tumor is large. Nonbulky disease has a better prognosis than bulky disease.

NHL is a complicated set of diseases with a complex classification system. In fact, the classification system is continuously evolving as we learn more about these cancers. The newest classification system takes into account not only the microscopic appearance of the lymphoma but also its location in the body and genetic and molecular features.

Grade is also an important component of the NHL classification.

  1. Low grade: These are often called “indolent” lymphomas because they grow slowly. Low-grade lymphomas are often widespread when discovered, but because they grow slowly, they usually do not require immediate treatment unless organ function is compromised. They are rarely cured and can transform over time to a combination of indolent and aggressive types.
  2. Intermediate grade: These are rapidly growing (aggressive) lymphomas that usually require immediate treatment, but they are often curable.
  3. High grade: These are very rapidly growing and aggressive lymphomas that require immediate, intensive treatment and are much less often curable.

The “staging,” or evaluation of extent of disease, for both HL and NHL, are similar.

  1. Stage I (early disease): Lymphoma is located in a single lymph node region or in one area or organ outside the lymph node.
  2. Stage II (locally advanced disease): Lymphoma is located in two or more lymph node regions all located on the same side of the diaphragm or in one lymph node region and a nearby tissue or organ.
  3. Stage III (advanced disease): Lymphoma affects two or more lymph node regions, or one lymph node region and one organ, on opposite sides of the diaphragm.
  4. Stage IV (widespread or disseminated disease): Lymphoma is outside the lymph nodes and spleen and has spread to another area or organ such as the bone marrow, bone, or central nervous system.

Both HL and NHL are further classified with letters.

  1. An “A” or “B” designation indicates whether the person with lymphoma had symptoms such as fevers and/or weight loss at the time of diagnosis. “A” indicates no such symptoms, and “B” indicates symptoms.
  2. An “E” designation indicates that the tumor spread directly from a lymph node into an organ or that a single organ outside the lymphatic system is affected with no apparent lymphatic involvement. If the spleen is involved an “S” designation is added.

MANAGEMENT OF LYMPHOMAS

Treatment is based on the actual classification of disease, the stage of disease, prior treatment (if any), and the patient’s ability to tolerate therapy. If the disease is not an aggressive form and is truly localized, radiation alone may be the treatment of choice. With aggressive types of NHL, aggressive combinations of chemotherapeutic agents are given even in early stages. More intermediate forms are commonly treated with combination chemotherapy and radiation therapy for stage I and II disease. The biologic agent interferon has been approved for the treatment of follicular low grade lymphomas, and an antibody to CD20, rituximab (Rituxan), has been effective in achieving partial responses in patients with recurrent low-grade lymphoma. Studies of this agent in combination with conventional chemotherapy have demonstrated an improvement in survival as well. Central nervous system involvement is also common with some aggressive forms of NHL; in this situation, cranial radiation or intrathecal chemotherapy is used in addition to systemic chemotherapy. Treatment after relapse is controversial. BMT or PBSCT may be considered for patients younger than 60 years of age.

Lymphoma Treatment

The vast majority of cancer patients receive ongoing care from oncologists but may in fact be referred to more than one oncologist should there be any question about the disease. Patients are always encouraged to gain second opinions if the situation so warrants this approach. In addition to one’s primary-care physician, family members or friends may offer information. Also, many communities, medical societies, and cancer centers offer telephone or Internet referral services.

Once one settles in with an oncologist, there is ample time to ask questions and discuss treatment regimens.

  1. The doctor will present each type of treatment, discuss the pros and cons, and make recommendations based on published treatment guidelines and his or her own experience.
  2. Treatment for lymphoma depends on the type and stage. Factors such as age, overall health, and whether one has already been treated for lymphoma before are included in the treatment decision-making process.
  3. The decision of which treatment to pursue is made with the doctor (with input from other members of the care team) and family members, but the decision is ultimately the patient’s.

As in many cancers, lymphoma is most likely to be cured if it is diagnosed early and treated promptly.

  1. The most widely used therapies are combinations of chemotherapy and radiation therapy.
  2. Biological therapy, which takes advantage of the body’s innate cancer-fighting ability, is used in some cases.

The goal of medical therapy in lymphoma is complete remission. This means that all signs of the disease have disappeared after treatment. Remission is not the same as cure. In remission, one may still have lymphoma cells in the body, but they are undetectable and cause no symptoms.

  1. When in remission, the lymphoma may come back. This is called recurrence.
  2. The duration of remission depends on the type, stage, and grade of the lymphoma. A remission may last a few months, a few years, or may continue throughout one’s life.
  3. Remission that lasts a long time is called durable remission, and this is the goal of therapy.
  4. The duration of remission is a good indicator of the aggressiveness of the lymphoma and of the prognosis. A longer remission generally indicates a better prognosis.
  5. Remission can also be partial. This means that the tumor shrinks after treatment to less than half its size before treatment.

The following terms are used to describe the lymphoma’s response to treatment:

  1. Improvement: The lymphoma shrinks but is still greater than half its original size.
  2. Stable disease: The lymphoma stays the same.
  3. Progression: The lymphoma worsens during treatment.
  4. Refractory disease: The lymphoma is resistant to treatment.

The following terms to refer to therapy:

  1. Induction therapy is designed to induce a remission.
  2. If this treatment does not induce a complete remission, new or different therapy will be initiated. This is usually referred to as salvage therapy.
  3. Once in remission, one may be given yet another treatment to prevent recurrence. This is called maintenance therapy.

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