Scott Seaman, founder of the Chicago Blood Cancer Foundation, also writes about cancer for national online news site The Examiner. A survivor of non-Hodgkin Lymphoma, Scott is dedicated to supporting all blood cancers and his latest article is about CML, including the upcoming Virtual Patient Summit the National CML Society https://www.facebook...ionalcmlsociety is hosting on 9/22 for US patients in observance of CML Awareness Day. http://www.examiner....and-information
Chronic Myeloid Leukemia (CML): awareness and information
August 24, 2012 By: Scott Seaman
The days are growing shorter and a hint of fall is in the air as the beautiful summer of 2012 draws near an end. Thankfully, most patients diagnosed with a once fatal form of leukemia known as Chronic Myeloid Leukemia (CML) will not see their days on earth grow shorter on account of their disease. Today, the future is much brighter for patients diagnosed with CML than it would have been just 12 years ago.
The tremendous progress was neither fortuitous nor guaranteed like the changing of the seasons. Instead, the progress made in CML is a function of long-standing investment in cogent medical research, tremendous work of talented researchers spanning decades, and the dedication of patient advocates. As September 22 is CML Awareness Day, this is an appropriate time to examine CML.
What is CML? Leukemia is the second most common category of blood cancer and CML is the rarest of the four major forms of leukemia. Leukemia involving the type of marrow cell that forms special white blood cells known as lymphocytes is called "lymphocytic" or "lymphoblastic" leukemia. Where the cell changes take place in a type of marrow cell that normally goes on to form red blood cells, some types of white blood cells, and platelets, the leukemia is called "myeloid."
Chronic myeloid leukemia (CML) is known by several other names, including chronic myelogenous leukemia, chronic myelocytic leukemia, and chronic granulocytic leukemia. Call it want you want, CML results from a change or mutation in the DNA of a single bone marrow cell. As a chronic leukemia, it typically progresses slowly and permits the growth of greater numbers of (immature) irregular cells. While a normal white blood cell count is in the 5,000 to 10,000 range, in CML patients the count is typically between 50,000 and 500,000 at the time of diagnosis. Left unchecked, this cell overgrowth ultimately will lead to the patient's death.
Symptoms and diagnosis: Although most cancers have "stages," CML has phases: chronic phase; accelerated phase; and blast crisis. Most patients, approximately 80 percent, are diagnosed in the chronic phase. As with many cancers, an early diagnosis (i.e., during the chronic phase) is important.
People with CML may not have any symptoms at the time of their diagnosis. Often the diagnosis is made in connection with a regular checkup or a medical examination for another condition. Some people with CML, however, are symptomatic. Potential symptoms may include fatigue, shortness of breath while doing everyday activities, an enlarged spleen, or being anemic.
In most cases, blood and marrow cells are examined to make a CML diagnosis. Various laboratory tests are used to examine the blood and marrow cells. With CML, the hemoglobin concentration is decreased and the white cell count is increased, often to very high levels. The number of platelets may be increased or decreased, depending on the severity of the person's disease. Bone marrow samples are examined to confirm the blood test findings and to determine if a chromosomal abnormality known as the "Philadelphia chromosome" exists.
By the numbers: Nearly 30,000 people in America are living with CML and it was estimated that more than 5,100 people were diagnosed with CML in 2011. The number of people living with CML has grown dramatically since 2001 due to improved treatments. CML can strike someone at any age - even children are diagnosed with CML. But the rate of incidence increases with age, from about less than one in 100,000 people up to age 40, about two in 100,000 people at 55, to about nine in 100,000 people at 80 and older. Most diagnoses occur in people between the ages of 50 to 69.
Translocation and the Philadelphia chromosome: Thanks to committed scientists and research that dates back to 1960, we understand the specific abnormality resulting in CML. The abnormal cell growth is caused by a change in the person's chromosomes. Human cells normally have 23 pairs of chromosomes. In CML patients, sections of chromosome 9 and chromosome 22 switch places, changing both chromosomes. The altered 22 chromosome, also known as the Philadelphia chromosome, gives the CML patient's body new instructions which result in both the over production of white blood cells and development of other immature blood cells which are incapable of full development. Left unchecked, these actions will result in the person's death. This "translocation" of chromosome 9 and chromosome 22 is found only in the CML cells and in a portion of patients with acute lymphoblastic leukemia.
Lifesaving treatments: An understanding of the translocation of the chromosomes is not merely a footnote of interest to scientists. This discovery served as the foundation for effective new treatments. The National CML Society tells us that, prior to 2001, traditional therapies such as chemotherapy were used, and the average life expectancy after diagnosis was three to five years.
Now, according to the National CML Society, most patients today can expect to live a normal lifespan as long as they have access to treatment and adhere to the prescribed treatment. The reason for this huge reversal in survival fortune for CML patients is a new form of targeted drug therapy that has been available to patients since 2001. This category of drugs, known as tyrosine kinase inhibitors, interferes with the signal activity that results in the proliferation of cancer cells. More specifically, these drugs target the BCR-ABL protein associated with the Philadelphia chromosome and, like many of today's targeted therapies, leave healthy cells alone. These treatments have transformed this previously fatal leukemia into a manageable chronic disease for most patients.
The oldest of these treatments is imatinib mesylate (Gleevec), which has been the standard initial therapy for chronic phase CML since 2001. Studies have shown that Gleevec can keep the chronic phase of CML under control for at least 10 years, the length of the observation period since this drug's approval. Not all patients succeed with this drug. Some CML patients experience side effects of Gleevec that are significant enough to require them to discontinue the drug. In some patients the disease becomes resistant to Gleevec.
In 2010, two additional oral drug therapies - dasatinib (Sprycel) and nilotinib (Tasigna) - were approved for newly diagnosed chronic phase CML patients. Neither Sprycel nor Tasigna has been shown to result in longer survival at this point. Some findings suggest that these drugs may produce faster complete cytogenetic and molecular response, which might prove to be associated with better long-term outcomes. Additionally, these drugs provide options for some patients who cannot take Gleevec. Two additional tyrosine kinase inhibitors, bosutinib and ponatinib, are now being used in clinical trials and are expected to become available for CML patients in the future.
Those who do not respond to this type of treatment may be treated by a bone marrow or stem cell transplant, a potentially curative treatment for the disease. Older treatments are sometimes used on patients and important clinical trials are ongoing.
Special CML Awareness Day Event: The National CML Society is hosting a free educational webinar that will be streamed live on the internet called "Living Well with CML: A Virtual Patient Summit" on September 22, 2012 from 1:00 pm - 6.30 pm EST, Noon to 5:30 pm CST. Participants include: Greg Stephens, Executive Director of the National CML Society; basketball hall-of-famer Kareem Abdul-Jabbar, who was diagnosed with CML in 2009; CML patient advocate extraordinaire Pat Elliott; and numerous healthcare professionals.
Greg Stephens founded the National CML Society, originally called Carolyn's Hope, in memory of his mother Carolyn who lost her life to the disease. Greg points out "awareness activities enable those impacted by CML to connect with experts and each other to join in solidarity as a community dealing with cancer in new ways that are not found in more traditional cancer support services." He adds, "these events educate the patient's family, friends and others in the patient's life, as well as the public at large, so they can better understand this form of cancer treatment which is now impacting many other types of cancer, with even more projected in the future."
Pat Elliott, a health journalist, has worked hard with Greg to put this event together and we give her the last word on the subject. Knowing this Examiner's penchant for using war analogies in talking about cancer, Pat points out that the CML community is focused on "living well with CML" and "living with cancer" and not on "battling cancer." Greg and Pat are tremendous champions for people with CML. If you are impacted by CML, you should join them on September 22.