Should I get a second opinion? If so when?»
It is quite customary for newly diagnosed WM patients to get a second opinion from a recognized WM expert, or at least from a hematologist-oncologist who is familiar with treatment of WM. WM is a rare disease and as a result many general oncologists have had little experience dealing with many WM patients over the course of their careers. Not only can a newly diagnosed patient get a second opinion, but a WM patient at any stage of their illness can and should get a second opinion should there be any need for further clinical evaluation. Second opinions are also important with regard to bone marrow biopsy interpretations for much the same reasons as outlined above and because WM is also a difficult pathological diagnosis to make in many circumstances. Local support groups are excellent sources for the WM patient looking for a second opinion locally; many WM patients will travel fair distances to seek second opinions from world- recognized WM experts.
Should I get the shingles vaccine? »
The shingles vaccine is a live attenuated (weakened) virus vaccine, and immunocompromised people (which include those with blood cancers such as WM) should not receive live virus vaccines. If you are a WM patient on certain chemotherapy regimens such as fludarabine or bortezomib, have undergone stem cell transplantation, or have had shingles previously, you should consider a prophylactic anti-viral medication such as acyclovir to help prevent shingles.
Should I get a flu shot? Which one? »
Flu shots are appropriate and even recommended for people with WM. The shot is a killed virus vaccine and is therefore safe to use. There is some question as to whether immunocompromised people (which include those with blood cancers such as WM) can mount an effective immune response following vaccination; however, any immune response is better than none, especially given the mortality rate of seasonal flu in the elderly. Nasal mist vaccinations such as FluMist are live virus vaccines and are not recommended for immunocompromised people.
How often should I see a doctor?»
A WM patient should see the hematologist – oncologist on a periodic basis depending on the severity of the disease. Watch and Wait patients do not need to see the oncologist as often as a WM patient who is actively receiving treatment. Watch and Wait patients typically see the oncologist every 3-6 months depending on disease burden, as do WM patients who are not receiving treatment. WM patients in treatment obviously see the oncologist far more frequently than patients not receiving treatment; WM patients receiving treatment see their oncologist as often as on a weekly basis depending on the treatment regimen and the patient’s health status. Some treatments can cause side effects such as low blood counts or IgM flare, which need to be recognized early and managed appropriately.
It is important for WM patients, regardless of their disease status, to also regularly see their family physician/ internist/GP on a regular basis irrespective of oncologist visits in order to keep abreast of other preventative and chronic health issues besides WM.
What does "low grade" lymphoma mean?»
Low grade lymphoma, or “indolent lymphoma” as it is often referred to, is any slow growing lymphoma that tends to grow and spread slowly, as opposed to intermediate or high-grade lymphoma which tends to grow and spread more quickly. Low grade lymphomas tend to progress very slowly and may not cause any major complications for a long period of time. Not all low grade lymphoma patients progress at the same rate or exhibit similar complications from the disease as there is variability between patients who have the same diagnosis.
WM is considered a low grade, or indolent lymphoma, as it is typically slow growing in most cases.
How do I find a good doctor for a second opinion? »
Because WM is so rare, some private practice oncologists may never see a WM patient, while others may see only a few. Generally speaking, large medical universities/teaching hospitals see more WM patients and have staff physicians more experienced with WM. The IWMF website has a list of several of the better-known experts at http://www.iwmf.com/about-wm/finding-a-doctor.aspx. Also, you may want to contact your local support group leader (http://www.iwmf.com/services/support-groups.aspx) who may have experience to share regarding doctors in your area.
Where do I find "state of the art" treatment for WM?»
The most important factor related to “state of the art” treatment for a WM patient is the knowledge base of the hematologist- oncologist who is responsible for the treatment of the patient in question (see “Should I get a second opinion”). Oncologists who have a great deal of experience and expertise treating WM patients are usually located at large teaching university hospitals. Many WM patients are treated by their local oncologists under the guidance of WM experts from other institutions. It is often not necessary to travel great distances to receive treatment at “centers of excellence” unless the patient wishes to do so.
WM experts follow the most recent International Workshop on Waldenstr?m’s Macroglobulinemia Consensus Panel recommendations on treatment for WM. The IWMF publishes a regularly updated booklet “Treatment Options” that includes the most up-to-date treatment recommendations for WM as outlined by the WM experts worldwide. The IWMF “Treatment Options” booklet can be obtained upon request from the IWMF office by calling 941-927-4963 or by visiting the IWMF website at www.iwmf.com.
What is a full remission? »
In WM and other indolent lymphomas, the preferred terminology is response rather than remission. A complete response is categorized by the 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.
What is partial remission? »
In WM and other indolent lymphomas, the preferred terminology is response rather than remission. A partial response is categorized by a >50% reduction of serum monoclonal IgM concentration by protein electrophoresis and a >50% decrease in adenopathy or organomegaly on physical examination or on CT scan; and no new symptoms or signs of active disease.
How can I get more information to / for my doctor? »
What is the best way to select an oncologist who is familiar with WM? »
Given the rare incidence of WM (0.5 cases per 100,000 people per year) most independent private practice hematologist - oncologists may see only a few WM patients throughout their careers. As a general rule, large university teaching hospitals see many more WM patients and have clinicians who are more familiar with the diagnosis and management of WM. You should ask your hematologist-oncologist how much experience he/she has had with treating WM patients and whether he/she is willing to consult with a renowned WM expert in the management of your disease. The IWMF website has a short list of several of the better-known experts at http://www.iwmf.com/about-wm/finding-a-doctor.aspx.
Are there any biomarkers that distinguish between subtypes of WM? »
WM is a disease with diverse manifestations. Although patients may appear to fall into general categories (high IgM vs. low IgM, enlarged lymph nodes vs. no enlarged lymph nodes, peripheral neuropathy vs. no neuropathy, for instance), no formal subtypes of WM (with biomarkers) have been designated as such in the medical literature.
What causes WM? Is there an environmental cause? »
At this time, we cannot say definitely what causes WM. Various studies have suggested predisposing risk factors such as IgM MGUS (monoclonal gammopathy of undetermined significance), familial history of WM or other B-cell disorders, chronic infectious diseases such as hepatitis or AIDS, exposure to certain solvents, dyes, and pesticides, and increasing age (WM is primarily a disease of the elderly).
How long can I expect to live after being diagnosed with WM?»
The older literature quotes an average life expectancy of 5-7 years, but that statistic is outdated. A significant percentage of patients may remain on watch and wait (smoldering WM) for years before requiring treatment. Newer and less toxic treatments such as Rituxan are probably prolonging life expectancy for many WM patients. Dr. Irene Ghobrial of the Dana-Farber Cancer Institute reviewed the records of 337 patients with newly diagnosed symptomatic WM between 1960 and 2001 seen at the Mayo Clinic in Rochester, MN. The disease-specific survival was found to be 11.6 years. Hopefully, even newer studies will confirm that longer survival is occurring.
Why am I on watch and wait and not being treated?»
Patients without significant symptoms affecting quality of life should not be treated. At the current time, there is no treatment that will cure WM, but it is an indolent (slow-growing) cancer and generally does not require immediate treatment in order to save life. Unfortunately, many current therapies have toxic side effects, and treating asymptomatic patients with these therapies may potentially have an adverse effect on quality of life and health that is worse than the disease itself.
How do I get a packet of information about WM?»
Is there a familial predisposition to WM? Do I have to worry about my kids getting it?»
There is a familial predisposition to WM, with most studies suggesting that approximately 20% of patients have a history of the disease or related B-cell disorders in their families. At this time, there is no way of predicting which, if any, family members of a WM patient will ultimately develop WM, although those with IgM MGUS (monoclonal gammopathy of undetermined significance) are at greater risk. However, WM is primarily a disease of older people and a significant percentage of diagnosed patients may go for years before requiring treatment; therefore, one shouldn’t worry excessively about this possibility.
How does WM differ from CLL and multiple myeloma?»
WM cells share some characteristics with both CLL (chronic lymphocytic leukemia) and multiple myeloma cells. CLL is a cancer of immature B-lymphocytes, while multiple myeloma is a cancer of plasma cells, which are the final step in B-lymphocyte maturation. Under the microscope, the cancerous cells of WM have an intermediate appearance between B-lymphocytes and plasma cells and are called lymphoplasmacytic cells. There are surface markers on the cells of WM, CLL, and multiple myeloma that help a pathologist to distinguish among them. In general, the surface marker pattern of CLL is CD19+/CD20+/CD5+/CD23+. WM cells are usually CD19+/CD20+/CD5-/CD23-. Multiple myeloma cells are typically CD19-/CD20-/CD38+/CD138+. The cancerous cells of WM and CLL secrete monoclonal IgM, although CLL cells secrete lower levels than WM cells. Most multiple myeloma cells secrete monoclonal IgG or IgA, although there is a rare IgM-secreting variant. Because all three diseases are cancers of the immune system, infections are a major concern with all three, as are anemia and other blood disorders due to crowding of the bone marrow with abnormal cells. All three diseases can smolder – meaning that some patients remain asymptomatic for long periods of time and not require treatment. Enlarged lymph nodes and an abnormally high number of circulating B-lymphocytes are more common with CLL than WM. Bone lesions and kidney problems are much more common in multiple myeloma than in WM or CLL.
What’s the difference between lymphoma and leukemia?»
Lymphoma is a cancer of specific white blood cells called B-lymphocytes or T-lymphocytes, which are cells of the lymphatic system and protect the body from infection; frequently (but not always) many types of lymphoma result in enlarged lymph nodes. Leukemia is also a cancer of the white blood cells, but these abnormal cells may be neutrophils, monocytes, or other white blood cells as well as lymphocytes; typically these abnormal cells are very immature and circulate in large numbers in the bloodstream.
Who was Waldenstrom? What does “macroglobulinemia” mean anyway?»
Dr. Jan Waldenstr?m (1906-1996) was a Swedish physician who in 1944 first described two patients with symptoms of what is now known as Waldenstrom’s macroglobulinemia. “Macroglobulinemia” is a compound word—“macro” meaning large and “globulinemia” meaning protein in the blood. In the case of WM, the large protein in the blood is IgM, produced by the cancerous cells.
What’s the difference between WM and LPL? Are they the same disease? »
Sometimes WM and LPL (lymphoplasmacytic lymphoma) are used synonymously although WM is really a subset (type) of LPL. LPL is also an indolent, non-Hodgkin’s lymphoma characterized by the way the cells look under a microscope and flow cytometry parameters (see “What is WM”). These cells are called lymphoplasmacytic because they have the characteristics of both lymphocytes and plasma cells. LPL cells can secrete IgM, IgA, or IgG, but if they secrete IgM then the disease is more properly called WM. WM is by far the most common type of LPL.
What is BTK?»
BTK stands for Bruton’s tyrosine kinase. It is an important enzyme in the development and activation of B-cells. When B-cells are exposed to antigens, they are activated through cell pathways that include BTK. If BTK is over-expressed, as it is in WM, it increases the proliferation of WM cells and promotes their survival. BTK is the target of a new oral drug called ibrutinib (trade name Imbruvica), which is being used as treatment for WM.
What is MYD88 and what is this important MYD88 mutation I've heard about in WM patients?»
MYD88 is a protein coded by a gene called myeloid differentiation primary response 88. When B-cells are exposed to antigens, MYD88 initiates several downstream cell pathways that result in the expression of factors critical to the development and activation of B-cells. A single specific mutation in the MYD88 gene is designated MYD88 L265P, and this mutation was discovered to have a wider prevalence in WM (approximately 90% of patients) than in most other kinds of blood cancers.
What is the significance of the MYD88 L265P mutation in WM?»
Its significance is still not completely understood. Although it is prevalent in WM (approximately 90% of patients), at this point we do not believe it causes the disease. However, it does appear to play an important role in the proliferation and survival of WM cells by leading to over-expression of proteins such as BTK that are involved in B-cell development and activation. Because of its prevalence in WM, its presence or absence may become useful as part of the diagnostic workup of patients with suspected WM or related diseases.
Are there other gene mutations important in WM?»
Researchers are looking at several other gene mutations found in significant percentages of WM patients. Such work is still very preliminary, but at least one mutation in the gene CXCR4 appears to be an adverse factor in the prognosis of WM and may lead to the proliferation of WM cells in tissues outside the bone marrow. The IWMF is currently sponsoring research to study CXCR4.
What’s a support group and what happens there?»
A support group is an association of WM patients, caregivers, and friends who are geographically close and who meet periodically to support each other as they cope with WM. Each support group has one or more leaders who organize meetings and communicate with the various group members. Support groups can do a variety of things – they can invite speakers, watch audio-visual presentations, have picnics or fundraisers, or just informally discuss topics of interest. The IWMF has support groups in the U.S. and around the world. For a list of support groups, go to http://www.iwmf.com/services/support-groups.aspx.
Where are IWMF support groups? »
The IWMF has support groups across the U.S, Canada, Europe, Australia and New Zealand. New support groups are being developed all the time in other countries. You can access the most current list of support groups at http://www.iwmf.com/services/support-groups.aspx.
What do I do if I’m not near a support group but want to talk to someone?»
The IWMF maintains a Lifeline list, which is a group of people who have volunteered to speak with patients and caregivers about a variety of topics and have provided their telephone numbers and e-mail addresses. This list can be found at http://www.iwmf.com/services/lifeline.aspx.
How do I join the IWMF? »
You can go to our website at http://www.iwmf.com/join/index.aspx and join either online by making a donation or by printing a form that you can fill out and mail along with your donation to the IWMF office at 6144 Clark Center Ave., Sarasota, FL 34238. You can also call our office at 941-927-4963.
How can I raise money for the IWMF? »
Where else can I find information about WM? »
An exhaustive list is beyond the scope of these FAQs, but a good place to start is the IWMF website at http://www.iwmf.com. You can call our office at 941-927-4963 and request a free Info Pak and/or order a set of DVDs with presentations from our most recent annual Educational Forum for patients. Speaking of the Educational Forum, it is held each spring at a different location in the U.S. and is a great way for the newly diagnosed WM patient and caregiver(s) to learn more about WM. We also offer IWMF-Talk, which is an e-mail discussion list where all matters regarding WM are discussed among patients and caregivers; online; you can join IWMF-Talk by going to http://www.iwmf.com/docs/subscribe_instructions.pdf. Support groups are another good way to learn about WM, and you can locate the most current list of support groups at http://www.iwmf.com/services/support-groups.aspx.
Is there someone I or my spouse/caregiver can talk to? »
The IWMF maintains a Lifeline list, which is a group of people who have volunteered to speak with patients and caregivers about a variety of topics and have provided their telephone numbers and e-mail addresses. This list can be found at http://www.iwmf.com/services/lifeline.aspx. Caregivers are encouraged to attend IWMF support group meetings where they can also find information and support. You can access the most current list of support groups at http://www.iwmf.com/services/support-groups.aspx. At our annual Educational Forum we offer a breakout session for caregivers to share their experiences and support for each other.
How do I include the IWMF in my Will?»
There are various ways to donate to the IWMF through a Will. These include bequests, charitable remainder trusts, gift annuities, life insurance designations, life estates, or charitable lead trusts. To read more about these go to http://www.iwmf.com/donate/gifts-for-research-only.aspx. If you are interested, contact the IWMF office at 941-927-4963, and the office staff will put you in touch with an adviser who can assist you.
What's an Ed Forum like and should I attend? »
The IWMF's annual Educational Forum is a unique opportunity for patients and caregivers to learn about our disease from specialists in Waldenstrom’s macroglobulinemia (WM) who are involved in many areas of clinical practice and research. Held in a different part of the United States every year, the “Ed Forum” offers something for everyone, no matter what your experience or level of knowledge.
Presentations aimed at the layperson address symptoms and complications of the disease, current treatment options, new therapies on the horizon, and recent research findings that may someday lead to a cure. Breakout sessions permit in-depth exploration of specialized topics and opportunities for patients and caregivers to share their experiences in a safe and supportive environment.
Every Ed Forum is different, but “Early Bird” sessions typically begin on Friday morning and presentations continue through Saturday, culminating on Sunday morning with a popular “Ask the Doctor” session where patients can have their questions answered by a panel of experts.
Attending an Ed Forum in person is the best way to benefit from the program, but most sessions are videotaped and recorded on a set of DVDs for the benefit of those who cannot attend or those who simply want to reinforce their learning experience. For information regarding DVD's, contact the IWMF Office at . For more informaiton about the Ed Forum, click here.
Testimonials about the Ed Forum »
Expressions of attendees about the Ed Forum experience (for more information about the Ed Forum, click here)
What is it like to live with WM? »
Each WM patient is unique in the way the disease or various treatments will affect his/her quality of life. Multiple factors can affect one’s ability to deal with WM, including age, fitness level, lifestyle, psychological health, co-existing health problems, work situation, and financial situation. Obviously, a strong support system of family and friends is important for dealing with the ups and downs of a chronic disease. The goals of care should encompass medical and non-medical strategies that will help the WM patient achieve a good quality of life for an extended period of time – fortunately, newer and safer therapies are enabling many WM patients to reach that goal. By and large, most people manage to live with WM very well, as anyone who has attended one of our annual Educational Forums can attest to.
Are there any foods that are beneficial or harmful to eat while on the various therapies? »
In general, you should try to eat a healthy, well balanced diet rich in whole grains, fruits, and vegetables. This applies to patients at any stage of their disease, whether undergoing treatment or not. Avoid hot or spicy foods if you have mouth sores, dry mouth, or gastrointestinal problems while on treatment. It may be necessary during treatment to take dietary supplements if nausea is causing decreased food intake, but this should be done with the advice of your physician. Patients with neutropenia (greatly decreased neutrophils, a type of white blood cell) due to treatment should avoid raw fruits, raw vegetables, and raw seafood during the period of low blood counts. Studies have suggested that grapefruit intake should be avoided during certain chemotherapies, and you should consult with your physician or pharmacist if you regularly eat grapefruit or drink grapefruit juice. Other studies have reported that green tea may inhibit the anti-cancer properties of bortezomib (Velcade) and should be avoided while on this therapy.
How do we deal with telling family and friends? »
Generally speaking, if or how one tells family and friends about the WM cancer diagnosis remains a very personal and individual decision. Certain work or family situations may preclude sharing this information. However, as a general rule it’s best to be open and honest with family and friends about what to expect. Many people hear “cancer” and cringe, some may panic, but in this case they shouldn’t feel despondent WM is usually a disease which is very slow-growing, and most people live with it for many years. Many of the newer treatments being developed for WM aim to make it a chronic but manageable disease. We believe one day a cure can be found. A strong support system of knowledgeable family and friends is important for a patient dealing with the ups and downs of a chronic disease like WM. Once the initial shock has worn off, encourage family and friends who are interested in learning more about WM to contact the IWMF at http://www.iwmf.com or call 941-927-4963 for more information. Sharing one’s concerns and fears with other WM patients who have “walked in your shoes” is a wonderfully cathartic way to ease one’s anxieties about the WM diagnosis.
Will I be able to keep working?»
There is no definite answer, as individual patients have different disease symptoms and different responses to treatment. While on watch and wait and largely asymptomatic, most WM patients are able to continue working. When treatment is necessary, certain therapies have a greater tendency to cause temporary fatigue and other issues affecting the ability to work than others. Your physician can give you a more realistic expectation of how a particular treatment will impact your work status.
What should I do to protect my immune system?»
Wash your hands frequently and avoid touching your hands to your face, especially during cold and flu season. Keep up to date on your flu and pneumonia vaccinations. Eat a healthy, well balanced diet and get the proper amount of sleep. Avoid close contact with people who are exhibiting obvious symptoms of colds, flu, or other diseases. Be sure to wash raw fruits and vegetables before eating and make sure that meat is cooked to the proper temperature. Research studies have also shown that moderate exercise is beneficial to the immune system. These are all common sense things that everyone should do, no matter their state of health.
Are there any alternative medical treatments for WM?»
No. There are complementary therapies, such as yoga, tai chi, acupuncture, massage, etc., that can improve your quality of life as a cancer patient, but these should be done in addition to conventional treatments, not in place of them. If you are considering dietary supplements, you should consult your physician first. You can read more about complementary and alternative medicine at http://www.iwmf.com/surviving/complementary.aspx.
Do I need to tell my employer, co-workers, and friends that I have WM?»
This is a very individual decision for you to make based on such things as how your disease is affecting your daily life, how well those around you can cope with this knowledge, and how much your emotional and psychological well-being is impacted by having others know about your condition.
Are people with WM eligible for disability benefits?»
What is happening in my body while I watch and wait? How long do most patients stay on watch and wait? If I have a disease, shouldn't I be treating it, rather than being on watch and wait?»
WM is a variable disease in the way it affects people. Many patients can remain on watch and wait for a number of years with very stable disease, while others may see a slow progression (increasing IgM, increasing anemia, enlarging lymph nodes, increasing neuropathy) over time, to the point where they need to begin treatment. Patients without significant symptoms affecting quality of life should not be treated. At the current time, there is no treatment that will cure WM, but it is an indolent (slow-growing) cancer and generally does not require immediate treatment in order to save life. Unfortunately, many current therapies have toxic side effects, and treating asymptomatic patients with these therapies may potentially have an adverse effect on quality of life and health that is worse than the disease itself.
Is it important for me to become knowledgeable about the disease and treatments? Isn't that what my doctor is for?»
You should become a partner with your physician. You know your body best and you cannot expect your physician to be able to ascertain during a brief office visit just how you are feeling and how you are being affected by your WM. You should be aware of the signs and symptoms to expect from worsening disease, and you should have some familiarity with WM terminology and the laboratory tests commonly used to monitor your disease. If you are undergoing treatment, you should educate yourself about possible side effects so that you can alert your physician to problems before they worsen. Unless your physician is a WM expert, he or she typically deals with many different types of cancer (most of which are not WM) and being knowledgeable about your disease can be of help to your physician.
What kind of skin problems are related to WM? »
Skin problems are relatively rare with WM. Sometimes, the lymphoplasmacytic cells of WM can infiltrate the skin or the IgM secreted by WM cells can deposit in the skin. Symptoms can include skin thickening, nodules, or rashes. If you have these symptoms, you should see a dermatologist to rule out other causes for skin problems. Occasionally, people with WM may have thrombocytopenia (low platelets) or their high IgM may cause bleeding problems in the skin, leading to bruising, purpura (small red or purple areas), or petechiae (tiny spots). Some patients may experience increased susceptibility to insect bite reactions (mediated through the mast cells of lymphoma patients).
What symptoms will I see in the future? What are warning signs? »
Patients develop symptoms because of either IgM secretion or infiltration of WM cells in the bone marrow, spleen, lymph nodes, or other tissues.
- Patients may develop peripheral neuropathy (numbness, pain, or tingling of the fingers and toes) or see it worsen over time.
- Excessive IgM secretion may cause hyperviscosity (thickness of the blood) leading to bleeding of the nose, gums, and gastrointestinal tract, dizziness, mental confusion, balance problems, or loss of vision due to bleeding in the retinal blood vessels.
- IgM may also attack the platelets or red blood cells, causing bleeding problems or anemia.
Bone marrow infiltration can crowd out the normal blood-forming cells, leading to worsening anemia or other low blood counts, while the spleen or lymph nodes may increase in size and become uncomfortable.
There are instances of WM cells forming solid tumors in various areas, including the breast, spine, gastrointestinal tract, and extremities, and if you have unusual pain, bleeding, or lumps you should consult your physician.
Constitutional symptoms can include fever, night sweats, loss of appetite, loss of weight, and fatigue.
Because WM is a highly variable disease, most patients will experience only a few of these symptoms, and their severity will vary.
Patients and their physicians should also be aware of relatively rare conditions such as Bing-Neel syndrome or transformation. Bing-Neel syndrome is involvement of the central nervous system caused by the presence of WM cells or monoclonal IgM directed against the conduction cables of the brain and spinal cord. The symptoms of Bing-Neel syndrome can include headaches, personality and cognitive changes, visual hallucinations, speech impairment, seizures, gait changes, and alterations of right hand function. Rarely, loss of hearing may occur. These symptoms can be present even when bone marrow involvement by WM is stable.
Transformation occurs when some of the WM cells acquire more mutations over time, resulting in the development of a more aggressive lymphoma, such as diffuse large B-cell lymphoma; aggressive lymphoma requires prompt and specific treatment or it can rapidly be fatal. Painless swelling in the neck, armpit, or groin, caused by enlarged lymph nodes, is sometimes one of the first signs of aggressive lymphoma, and other symptoms may include night sweats, high fevers, weight loss, or extreme lethargy. These symptoms can mimic other conditions, including WM. The definitive way to diagnose an aggressive lymphoma is from biopsy.
What is the cause of night sweats in WM?»
Drenching night sweating is one of the so-called “B symptoms” associated with lymphomas. We do not have a definitive answer as to the cause, but one possible mechanism is that the both the progression of lymphoma and the body’s way of fighting infection have some things in common—both may lead to the mobilization of immune cells and proteins called cytokines, and these immune activities may account for fever, muscle aches, and night sweats.
How can WM affect my eyes?»
WM can affect the eyes in several ways. The IWMF quarterly newsletter, the Torch, has an excellent article on this subject, called “Waldenstrom and The Eye,” written by Dr. Maureen Hanley, a doctor of optometry. Go to http://www.iwmf.com/docs/Torch%20%20Jan%202011.pdf.
Which measurement is more reliable/valuable - IgM or SV (serum viscosity)? »
In general, the IgM measurement is more reliable and is one of the more important parameters used in determining a WM patient’s disease status (although it is not the only one). Some WM patients never develop high serum viscosity but have other symptoms associated with their disease (anemia, peripheral neuropathy, etc.). However, the SV measurement can be important for those patients who tend toward high IgM levels and need to monitor their serum viscosity as one way to determine if they need treatment. Note also that there is lab variability in IgM and SV measurements. If possible, try and use the same lab every time.
Are IgG and IgA levels an important measurement to follow too?»
WM patients tend to have low levels of both these immunoglobulins. If a patient has recurrent infections (of the sinuses, for example), then low IgG and IgA levels may be responsible and possible treatment could include IVIg (intravenous immunoglobulin) infusions. If a WM patient is not experiencing recurrent infections, this measurement is probably less important.
How do I interpret the numbers on my blood test results?»
Your blood test results are compared to results for an average patient population tested in each laboratory, enabling the laboratory to develop a reference range of normal values – this reference range thus varies slightly among different laboratories. For this reason, it is desirable to have all testing performed by the same laboratory so that accurate comparisons can be made with your results over a period of time. Any test results will be flagged if they are abnormally low or abnormally high. You should try to become knowledgeable about your blood tests and look for patterns or trends in your numbers, rather than focusing on a particular test result. If you have any abnormal results, you should discuss these with your physician – some abnormal results may be more concerning than others. See also the IWMF publication on this topic at: http://www.iwmf.com/docs/Blood_Tests.pdf
What are the key numbers in my blood testing?»
One very important principle to remember is that WM patients are not treated solely on the basis of blood test values.
Generally speaking, the most important blood tests to monitor are IgM, hemoglobin, and platelets.
The IgM value will tend to increase as the disease progresses, and an IgM value exceeding 3.0 g/dL is a risk factor for the development of hyperviscosity (excessive blood thickening).
For those patients with high IgM, serum viscosity (SV) testing may be included – an SV in excess of 3.0 cp should also be closely monitored.
Those with lower than normal hemoglobin have anemia, which tends to worsen as the disease progresses. While mild anemia can be tolerated, a hemoglobin value less than 10 g/dL may be an indication for treatment if the anemia is affecting quality of life.
Low platelets (< 100,000) may be another issue indicating a need for treatment if bleeding problems are present.
Because of the variability of disease manifestations in WM patients, these numbers are only approximate – some patients may be symptomatic at lower or higher levels than those stated. Patients with special issues, such as WM-related kidney disease or enlarged lymph nodes for instance, may want to monitor their disease progression with additional tests. A bone marrow biopsy may be a consideration, but is usually only performed upon initial diagnosis or when there are worrisome changes in blood tests and symptoms.
Will plasmapheresis have an effect on the Rituxan I have just taken? If so, how long should I wait before having plasmapheresis after having received Rituxan?»
Yes it will.
When one undergoes plasmapheresis, the plasma portion of the blood (containing the IgM) is separated from the cellular portion of the blood and discarded. During and after a Rituxan infusion, the Rituxan circulates throughout the body in the plasma portion of the blood and binds to both the WM and normal B-lymphocytes, leading to their destruction.
Because Rituxan is an IgG-type antibody, its half-life (the period of time that it will decrease by half in the body) should be approximately 22 days. However, when one receives plasmapheresis shortly after Rituxan, the discarded plasma will also contain free, unbound Rituxan, leaving a greatly reduced amount still available.
The longer one can delay plasmapheresis after Rituxan, the more effective the Rituxan will be. Obviously, if a patient is experiencing a dangerous IgM “flare” following Rituxan, then plasmapheresis may be necessary to preserve health or life.
IgM “flare” is a temporary increase in IgM that is particularly likely to occur with Rituxan therapy and can worsen symptoms. If your oncologist is concerned about the possibility of IgM “flare,” the better protocol would be to proactively perform plasmapheresis before Rituxan therapy.
Will plasmapheresis have an effect on the IVIg I have just received?»
Yes, plasmapheresis will reduce the amount of the IVIg one has just received. When one undergoes plasmapheresis, the plasma portion of the blood (containing the IgM) is separated from the cellular portion of the blood and discarded. During and after an IVIg infusion, the IVIg circulates throughout the body in the plasma portion of the blood, although some of it will go into the tissue space. Typically, IgG has a half-life (the period of time that it will decrease by half in the body) of approximately 22 days. However, when one receives plasmapheresis shortly after IVIg, the discarded plasma will also contain IVIg. The amount of IVIg reduction with each plasmapheresis treatment will vary but will typically be around 50%, leaving a greatly reduced amount still available.
How do I learn about the availability of drug trials?»
When should I get treatment?»
Patients should be treated when they become symptomatic, not on the basis of blood test numbers alone. To some extent, the decision to begin treatment is dependent on a particular patient’s tolerance of symptoms and how they are affecting quality of life. Patients develop symptoms because of either IgM secretion or infiltration of WM cells in the bone marrow, spleen, or lymph nodes. Patients may develop peripheral neuropathy (numbness, pain, or tingling of the fingers and toes) or see it worsen over time. Excessive IgM secretion may cause hyperviscosity (thickness of the blood) leading to bleeding of the nose, gums, and gastrointestinal tract, dizziness, mental confusion, balance problems, or loss of vision due to bleeding in the retinal blood vessels. IgM may also attack the platelets or red blood cells, causing bleeding problems or anemia. Bone marrow infiltration can crowd out the normal blood-forming cells, leading to worsening anemia or other low blood counts, while the spleen or lymph nodes may increase in size and become uncomfortable. There are instances of WM cells forming solid tumors in various areas, including the breast, spine, gastrointestinal tract, and extremities, and if you have unusual pain, bleeding, or lumps you should consult your physician. Constitutional symptoms can include fever, night sweats, loss of appetite, loss of weight, and fatigue. Because WM is a highly variable disease, most patients will experience only a few of these symptoms, and their severity will vary. The IgM level in and of itself is not usually the sole indication for treatment; however, high IgM levels can lead to elevated serum viscosity levels, which in turn, should be attended to.
What can I expect from treatment?»
The goal of treatment is to reduce or relieve the severity of the symptoms you had when you began treatment and to maintain that state for an extended period of time. There is currently no treatment that is a cure for WM. While you are undergoing treatment and for a while afterward, you may experience temporary symptoms related to treatment toxicities. Some of these may occur during an infusion and may be alleviated by certain pre-medications. Others may remain throughout the course of the therapy and for a short while afterward. These include fatigue, nausea, hair loss, weight loss, low blood counts, and fever, to name a few. Treatment-related toxicities vary according to the specific type of treatment, and you should consult your physician to determine exactly what to expect.
Are there any treatments that target the MYD88 mutation in WM patients?»
There are no treatments that target the MYD88 L265P mutation. However, there are drugs that target some of the downstream proteins in the MYD88 pathway. Ibrutinib (trade name Imbruvica) is an oral therapy developed to inhibit Bruton’s tyrosine kinase (BTK). Other BTK inhibitors are also in development, as are drugs that inhibit IRAK-1 and IRAK-4, which are additional proteins in the downstream pathway of MYD88.
If I am a relatively young patient, should I consider a stem cell transplant early in my disease or wait until later?»
If you are a relatively young patient, an autologous stem cell transplant (one that uses your own stem cells) is an option. In the past, transplant was considered a “last-ditch” treatment, but this philosophy has changed. Autologous transplantation has become a much safer option now than in the past and is achieving good responses. It appears that patients have a better chance at improved survival and quality of life if they don’t wait until they have been heavily treated before transplant. Current thinking is that younger patients may want to consider transplant after one or two treatment regimens. The autologous transplant is a second-line recommendation for relapsed/refractory WM and is not suggested for first-line treatment at this time. Another option to consider, if possible, is to collect stem cells and store them for a “rainy day” transplant. At this point, allogeneic transplant (one that uses donor stem cells) is still considered an experimental procedure for WM.
What are my out-of-pocket expenses for participating in clinical trials?»
There is no hard-and-fast rule on clinical trial expenses. Clinical trials will cover the cost of the treatment and the testing associated with follow up to monitor your response. Travel expenses and other costs are usually not covered. However, the cost will mostly depend on your country’s health care/health insurance system. Clinical trials have a coordinator, research nurse, or social worker who can assist you in determining what you will need to pay during a specific trial.
How do various treatments for WM affect my immune system?»
Most treatments for WM have some harmful, though usually temporary, effects on the immune system because, after all, WM is a cancer of the immune system. These treatments, while harming the cancer cells, can also cause collateral damage to the normal immune system cells. Typical side effects include low white blood cell counts (neutrophils and/or lymphocytes), which create a greater risk of infection. During treatment, one should be especially vigilant to wash hands frequently, avoid others with symptoms of diseases such as colds or flu, and take recommended prophylactic medications (such as acyclovir to prevent shingles). You should consult your physician for specific immune system side effects related to your particular treatment.
What if my treatment doesn't work?»
You should allow adequate time for your treatment to work. Just because you don’t see immediate results doesn’t mean you have treatment failure. For example, Rituxan, one of the most common WM therapies, may not achieve its best result for 6-8 months following the last treatment. Fortunately, since WM is usually slow-growing, it’s frequently not necessary to achieve immediate results in order to save life. Treatment options are increasing all the time, and if your treatment has truly failed, you and your physician should be able to determine an alternate course of therapy, perhaps with the advice of a WM expert. More information on treatment options can be found at http://www.iwmf.com/treatment/treatment-options.aspx.
What if I don't get treatment? What could I expect to happen?»
If you are asymptomatic, you should not be treated. However, you should be mindful of your health condition at all times, and consult with your doctor regarding treatment for things such as below normal hemoglobin levels, above normal serum viscosity levels, etc. If you are symptomatic to the point that treatment is recommended but you refuse it, typically any symptoms you have will become more severe, even life-threatening, with time although it may be impossible to predict how long it will take for this to occur. While it is usually slow growing, WM is a cancer – it can have a significant negative impact on quality of life and ultimately cause death.