Part I Waldenstrom’s Macroglobulinemia (WM) also referred as Lymphoplasmacyctic Lymphoma (LPL)

Affects older persons; peak incidence between 6 -7th decades. Tumor cells secrete an M protein (commonly IgM) Mixture of B cells ranging from small lymphocytes to plasmacytic lymphocytes to plasma cells. Involves lymph nodes, bone marrow, and spleen at presentation.

Initially, you will need to learn some basics about Waldenstrom’s macroglobulinemia (WM), sometimes referred to as a lymphoplasmacytic lymphoma (LPL).  Newly diagnosed?  Well striveforgoodhealth.com understands that this is a very difficult time for you. Not only are you, your family and your friends experiencing the gamut of different emotions that come with a cancer diagnosis, but there is so much new information to learn.

Here’s one place to start in getting information on this diagnosis!

Let’s understand first what is effected in the body and answer the questions that probably you are asking yourself  (pt or family or friends).

What is effected in this disease? 

B Lymphocytes are effected; they are a type of white blood cell that makes antibodies. B lymphocytes are part of the immune system and develop from stem cells in the bone marrow. Same with T cells.  T- and B-cells are highly specialized defender cells – different groups of cells are tailored to different germs. When your body is infected with a particular germ, only the T- and B-cells that recognise it will respond. These selected cells then quickly multiply, creating an army of identical cells to fight the infection. Special types of T- and B-cells ‘remember’ the invader, making you immune to a second attack.

What’s so special about the B cells?

With the help of T-cells, B-cells make special Y-shaped proteins called antibodies. Antibodies stick to antigens on the surface of germs, stopping them in their tracks, creating clumps that alert your body to the presence of intruders. Your body then starts to make toxic substances to fight them. Patrolling defender cells called phagocytes engulf and destroy antibody-covered intruders.

With this disease what happens to the B Cells?

When diagnosed with Waldenstrom’s macroglobulinemia (WM) which is a lymphoma or cancer. It affects a type of white blood cell called a B-lymphocyte or B-cell, which normally matures into a plasma cell whose job is to manufacture immunoglobulins (antibodies) to help the body fight infection. In WM, there is a malignant change to the B-cell in the late stages of maturing and it continues to proliferate as a clone of identical cells, primarily in the bone marrow, but also in the lymph nodes and other tissues and organs.  It is known as lymphoplasmacytic lymphoma (LPL) and must be associated with the production of an antibody protein or immunoglobulin known as IgM in order for WM to be diagnosed.

What’s the difference of WM versus LPL?  Are they the same disease? 

Sometimes WM and LPL (lymphoplasmacytic lymphoma) are used interchangeably although WM is really an LPL. However, WM comprises about 90 – 95% of all LPL patients.

The cancer cells of LPL have the appearance of both B-lymphocytes and plasma cells, hence the term lymphoplasmacytic.  LPL cells can secrete immunoglobulin antibodies (IgM, IgA, IgG, IgE or IgD), but those who secrete IgM are called WM.  Currently, the clinical term in cases of LPL with circulating monoclonal IgM is WM.  LPL is the term that describes the appearance of the bone marrow or lymph node used by pathologists.
My doctor said WM was a rare disease. How rare is it?
What does that mean for me?
WM is a rare cancer seen only in approximately three to five per million people per year with about 1500 new cases diagnosed in the US each year. This disorder is age-dependent and is quite rare under the age of 40 (less than 1 percent
of patients).  Typically, patients present between the ages of 60 and 70. For reasons that are unclear, WM is almost twice as common in men as in women and is more common in Caucasians than other ethnic groups. There is a familial predisposition to WM, with most studies suggesting that approximately 20-25% of patients have a first degree relative with WM or other B cell disorders. WM is a rare disease and as such it does not command much support for research dollars because there are few financial incentives for pharmaceutical companies; as much as I hate to document this.
Is there a cure for WM?
 
No, although quality of life and survival for WM patients are continuing to improve because of better treatments.
How long do I have left to live?
Although WM is incurable, in most cases it can be effectively treated to provide a good quality of life for many years.
In most patients, WM is a fairly indolent, chronic disease.

The median survival has varied in studies, from 5 years to 10

years. Median survival suggests that half of all patients survive 5 to 10 years. Another way to answer this question is to look at the 5
-year survival rate. The 5-year survival rate tells you what percentage of people live at least 5 years after the
cancer is found. Percent is how many out of 100. The 5
-year survival rate for people with Waldenstrom’s
macroglobulinemia is about 75%.
However, it is important to note that survival rates vary based on a number of individual factors including the patient’s age and whether the patient has other medical problems.
It is also important to remember that statistics on the survival rates for people with WM are an estimate.
As newer agents and treatments that
are more effective and less toxic become available, the life expectancy will continue to increase. The main causes of
death because of WM include disease progression, transformation to high-grade lymphoma, or complications of
therapy. However, because of the advanced age of patients with WM, many will die of unrelated causes.

 

 

 

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