Did you know?

The IWMF raised more than five million dollars for WM medical research

Did you know?

The IWMF raised more than 60 support groups in North America and nearly 20 others world-wide

Did you know?

The IWMF has a current worldwide membership of nearly 4,000

Did you know?

IWMF-TALK is a wonderful place to share information and learn from other survivors

Did you know?

Each year 1,500 new cases of Waldenstrom's macroglobulinemia are diagnosed in the USA

Did you know?

Volunteers at the IWMF Telephone Lifeline are waiting to share their experiences with you

Did you know?

You can order DVD recordings of past WM Educational Forums online or by mail

Did you know?

All IWMF programs and services are funded by WM survivors, caregivers, friends, and family

Undergoing Treatment

Watch and Wait »

 

Watch and Wait is a difficult concept to comprehend when a patient is first diagnosed with Waldenstrom’s macroglobulinemia (WM). The term Watch and Wait (W&W) has also affectionately been coined “Wait and Worry” or “Watch and Worry."   Conventional thinking in cancer treatment dictates either surgical removal of the cancer or aggressive treatment with chemotherapy or radiation. From an emotional point of view, knowing that you are taking action to eliminate the cancer from your body can be a comforting thought.

 

WM is different in that it is an “indolent” lymphoma. The definition for the term indolent from Webster’s dictionary is “slow to develop or heal." This is a good description for the symptoms exhibited by many, but not all, WM patients. The key to utilizing the W&W approach to treatment is determined by the relative progression of the disease and the severity of the patient’s symptoms at diagnosis. When a patient is first diagnosed it is very important to begin the process of recording laboratory values and symptoms to establish a baseline for the patient. As additional laboratory tests are performed and patient symptoms are monitored, a pattern will become evident which will indicate a patient’s rate of disease progression. In many WM cases, there will be very little change in a patient’s status from visit to visit. It is for these patients that a strategy of W&W may be an option. This strategy of “non-treatment” may last for months and indeed years, depending on the patient and how his or her symptoms progress. There are many patients in the WM community who have used this approach and have not needed treatment immediately after diagnosis.

 

The advantage to this strategy is that the patient avoids the use of treatments which could have side effects and associated toxicities until treatment is absolutely necessary. The question that confronts the patient is: “By delaying treatment am I allowing the cancer to become more difficult to treat in the future or am I allowing my symptoms to progress to the point that they cannot be easily managed?” This question can only be answered by you and your oncologist after a thorough review of your individual test results and physical exam findings.
 

Undergoing Treatment »

 

Once a decision to proceed with treatment has been made, it is vitally important that patients familiarize themselves with the type of treatment chosen, potential side effects of treatment, possible interactions with other medications or nutritional supplements.  Patients also need to decide where the treatment will be provided and who will be responsible for administering the treatment. Knowing these facts ahead of time helps remove much of the anxiety surrounding the treatment decision.

 

Many treatments related to Waldenström’s macroglobulinemia (WM) are administered on an outpatient basis: either at home, in a doctor’s office, or at an outpatient clinic. Some treatments may require overnight hospitalization, although this is rare in WM. Treatment may be given in many forms: oral medications, intramuscular or subcutaneous injections, or intravenous therapy. Laboratory tests are typically done prior to the start of each treatment to confirm a patient’s status before or during therapy. Some treatments may require preparatory medications taken the day before or on the day of treatment. An example of this would be the administration of (cortico)steroids and antihistamines prior to treatment with rituximab (Rituxan/Mabthera).

 

As noted above, laboratory tests may be done periodically during the course of treatment to check on a patient’s response to therapy and/or to monitor side effects related to treatment. These tests will enable your oncologist to adjust therapy as necessary in order to maximize efficacy while minimizing side effects.


Treatment of WM may take several weeks to months, depending on the course of therapy chosen. It is not unusual for patients to have a round of therapy and then wait a week or a month before another cycle of treatment. Oncologists like to follow certain standard protocols of treatment but will often make adjustments as required depending on the patient’s response to therapy.


Results from treatment are monitored by periodic laboratory tests and physical exams by the oncologist. Responses to different types of treatment vary in the amount of time required to see effects and may take several weeks to months to appear. This poses a particular challenge to WM patients as they are usually accustomed to seeing rapid results from treatment for other types of illnesses. In some instances treatment can actually cause a temporary worsening of the laboratory results before improvement is seen. This is most notably seen with a phenomenon known as “Rituxan flare” which is a transient increase in the levels of IgM after treatment with Rituxan, followed by a decrease to pretreatment levels and lower over time. For this reason, a WM patient must be monitored closely and not rush into further treatment without allowing for an adequate period of time for the current treatment to show its efficacy. Patience is an important and often under-utilized virtue when dealing with treatments related to WM.

 

The website chemocare.com is sponsored by the Scott Hamilton CARE Initiative at the Cleveland Clinic Taussig Cancer Center. It lists the most commonly used chemotherapy drugs, along with information about how these drugs are administered and their potential side effects.
 

Post-Treatment »

 

After a patient finishes treatment, a waiting period begins. There are many thoughts that may arise in a patient’s mind with regard to his or her treatment. Common questions include: “How soon will it work?” “When will I feel better?” “Will I have any side effects?” “When can I resume my normal routine?”  The answers to these questions vary from patient to patient; therefore some general guidance is offered below.


There are various treatment regimens for Waldenstrom‘s macroglobulinemia (WM) and post treatment monitoring may differ depending on the treatment selected. The patient should understand and consider this as it applies to his or her specific situation. As always, it is most important to consult with your oncologist before, during, and after treatment.


Most chemotherapeutic treatments come with side effects which alter the normal composition of blood components. In most cases chemotherapy is not started on a WM patient unless there are one or more of the following: anemia, abnormal platelets, abnormal white blood cell counts, elevated IgM, low IgG, enlarged lymph nodes or organs, or constitutional symptoms which require treatment.

 

The effects resulting from chemotherapy treatment are measured against the pre-treatment laboratory values. This points once again to the importance of following the trends of laboratory tests from the time that a patient is diagnosed with WM. It may take weeks to months before a change is seen in lab values.

 

It is normal practice for the oncologist to see the patient more often post treatment to ensure that the patient is monitored for adverse effects of treatment. Should a treatment be successful, there will be an incremental improvement in lab values over time. The oncologist will review the progress toward a normal state.

 

On occasion a patient’s lab values will worsen post treatment. This is primarily due to the general toxicity of some chemotherapeutic agents which damage normal cells as well as cancer cells. Anemia and/or decreased immunity are possible and are treated with blood transfusions or with red blood cell stimulating agents (hematopoietic drugs) such as erythropoietin, or colony stimulating factors for white blood cells such as Neulasta or Neupogen. It should be noted that these treatments are used to prevent the patient from becoming too anemic or immuno-compromised to function normally. Physicians may also elect to prophylactically treat patients with antibiotics and/or anti-fungals during the post-treatment period.


Another known possibility is the appearance of shingles (herpes zoster virus infection) during or after treatment. Some oncologists will put their patients on prophylactic antiviral therapy to prevent a shingles outbreak. It is thought that the immune system is weakened from chemotherapy, which allows the herpes zoster virus to reactivate and result in an outbreak of shingles. The use of antiviral therapy can be started at the first signs of shingles but requires the patient and the oncologist to be alert to the first signs of an outbreak. For antiviral therapy to be most effective, the outbreak must be caught early and antiviral drug therapy started immediately.


The majority of patients will be monitored closely post treatment with lab work and office visits for the oncologist to evaluate the patient’s progress. With close monitoring and awareness by the patient of his or her condition, the post treatment period should be uneventful. It is important to be patient after treatment to allow adequate time for the treatment to take effect before moving on to another course of therapy.


Communication between patient and physician is very important during the post treatment period. At each follow-up visit be sure to discuss any new symptoms that interfere with your daily life such as fatigue, pain, neuropathy, trouble sleeping, sexual problems, weight gain or loss, emotional problems such as anxiety or depression, etc. Eating a healthy diet and starting a slow and gradual exercise program under the supervision of a physician will assist the patient with an improved sense of well being.


A valuable publication can be found at the link below:


“Facing Forward: Life After Cancer Treatment” found at www.cancer.gov/cancertopics/life-after-treatment.pdf.
 

WM Transformation »

 

Since WM is an indolent (slow-growing) lymphoma, it is not uncommon for patients to have it for many years. In a small percentage of patients, having WM (or other indolent lymphomas) for a long time may increase their susceptibility to developing a transformation of their disease into a more aggressive lymphoma, such as diffuse large B-cell lymphoma. This appears to be a result of natural B-cell clonal evolution; that is, over time the cancerous B-cells may acquire additional mutations that cause them to change histologically, meaning a change in their microscopic morphology and disease markers. The percentage of patients who undergo this transformation has been variously cited as approximately 6-13% , depending on the study. It has also been suggested that treatment with nucleoside analogs such as fludarabine and cladribine (2CdA) may contribute to the development of transformation. Additional studies are needed to confirm this observation.


Signs of transformation to a more aggressive lymphoma include worsening constitutional symptoms (fever, weight loss, night sweats), rapidly enlarging lymph nodes, liver, and spleen, signs of disease outside the bone marrow, and profound cytopenias (reduction in the number of blood cells).


There is also evidence that prolonged treatment with alkylating agents such as chlorambucil and with nucleoside analogs can cause myelodysplastic syndrome and/or acute myelogenous leukemia. Myelodysplastic syndrome was once known as “pre-leukemia” and is characterized by inefficient and disorderly production of myeloid blood cells such as neutrophils or monocytes. Such situations likely occur because these drugs can cause DNA damage, leading to the development of mutations that can potentially become cancerous. While it is important for physicians to recognize these possibilities, it does not mean that such treatments should not be given. Physicians and patients should weigh the risks vs. benefits of these particular agents.
 

Response (Remission) Types »

 

Waldenstrom’s macroglobulinemia (WM) is an interesting disease in that it may remain “smoldering” for many years and not require treatment. Following treatment it is not uncommon for patients to stabilize at a moderate state of disease as evidenced by relatively stable lab values: not getting markedly worse or markedly better. Some patients will have a profound improvement in their lab values and their physical status but may find it short lived and require additional treatment(s). Yet other patients will receive treatment and may transiently worsen for a brief period of time (e.g. Rituxan flare) only to be followed by a very gradual improvement in their lab values over several months to years with a prolonged time before re-treatment is needed.


The period after treatment when a WM patient has experienced some improvement in lab values and physical symptoms is commonly called a “remission," although the preferred terminology is a “response.” Remission usually implies an absence of disease markers; however, in WM, complete absence of such markers is very unusual. It was felt that uniform criteria should be defined in order to objectively evaluate the types of response to treatment. Therefore, the Third International Workshop on Waldenstrom’s Macroglobulinemia (IWWM3) set forth the following responses:


Progressive disease occurs when there is an increase in serum monoclonal IgM of greater than 25% (confirmed by a second measurement), progression of clinically significant disease findings including cytopenias (reduction in number of blood cells), bulky lymph nodes or enlarged organs, or significant symptoms (fever, night sweats, weight loss, hyperviscosity, neuropathy, symptomatic cryoglobulinemia).


Stable disease is categorized by a reduction in serum monoclonal IgM of less than 25% or an increase of less than 25%, without progression of lymph node or organ enlargement, cytopenias, or clinically significant disease symptoms.


A minor response is a reduction in serum monoclonal IgM equal to or greater than 25%, but less than 50%, and no new symptoms or signs of active disease.


A partial response is a reduction in serum monoclonal IgM equal to or greater than 50%, a 50% decrease in lymph node or organ enlargement on physical examination or on CT scan, and no new symptoms or signs of active disease.


A complete response
is categorized by a disappearance of serum and urine monoclonal IgM, absence of malignant cells in the bone marrow, resolution of enlarged lymph nodes or organs confirmed by CT scan, and no signs or symptoms of disease. Reconfirmation is required six weeks later.


Not evaluable or delayed response occurs in cases where there is insufficient time or data for a determination of response to treatment. A delayed response may be seen after purine analog or monoclonal antibody therapy; therefore, patients should be followed for at least three months after treatment initiation to be considered unresponsive to therapy. Sometimes the best response is exhibited several months after treatment discontinuation. Occasionally in the research literature one may see the term “very good partial response.” Certain researchers have coined this term to mean a reduction in serum monoclonal IgM of 90%. One may also see the term “objective response,” which is a statistical term combining the number of complete and partial responses.


In all cases the continued monitoring of a patient‘s physical status and lab results by the oncologist will indicate what type of response a patient is experiencing.
 

Treatment Options Following Relapse »

 

When a patient who has finished treatment notices that his laboratory values and physical symptoms are beginning to trend in a deteriorating direction, the patient and his oncologist are confronted with choosing the next appropriate course of action, be it continued periodic monitoring or selection of the next type of therapy. Signs of a deteriorating condition are: decreasing hemoglobin/hematocrit, decreasing platelets, increasing IgM, increasing peripheral neuropathy, fatigue, fever and night sweats, high serum viscosity, vision problems, enlarging lymph nodes or organs, etc. Even though laboratory values and physical symptoms may be worsening, treatment does not necessarily have to begin immediately. The severity of the symptoms, overall health condition, and quality of life will factor into the decision of when to begin re-treatment. The dilemma is: what treatment does one choose? If the patient has had good results with a prior therapy that led to a significant period of response, then a repeat treatment with the same drug therapy may be an appropriate consideration. If a prior therapy was not very effective or the response period was short, a different type of therapy may be indicated.


Fortunately, the choices of treatments for relapsed patients are many and are continually increasing. Over the years there has been a multitude of treatments developed for WM, largely based on treatments for other B-cell lymphomas. There is also the important possibility to participate in a clinical trial. The choice of treatment plan is made with the oncologist carefully weighing the patient’s physical status along with the mechanism of action of the drugs considered and their ability to be used with other drugs to maximize their effects on WM cells. Stem cell transplants are also to be considered after careful evaluation of the patient’s lab values, treatment history, and physiologic age.


It is most important for a patient to be engaged with his oncologist during this treatment selection process.