Research is one of the mainstays of the MD Anderson Cancer Center Madrid. The center increases its research activity every year and to date, more than 500 patients have taken part in some sort of clinical trial.
Myelodysplastic Syndrome
DESCRIPTION AND TYPES
Myelodysplastic syndrome, or MDS, is a group of diseases in which the bone marrow doesn’t produce enough healthy blood cells. Red blood cells, white blood cells and platelets originate in the marrow and move into the bloodstream when they mature. In MDS, the marrow produces too many immature cells (blasts).
These blasts die in the marrow or soon after entering the bloodstream, resulting in too few healthy blood cells and low patient blood counts.
In its mildest form, myelodysplastic syndrome may be only anemia, low platelets or low white blood count, but higher-risk types can progress to acute myeloid leukemia (AML). Each year in Spain 3.000 new cases of MDS are diagnosed.
Ninety percent of myelodysplastic syndrome patients are over the age of 60, and 10-20% of diagnosed cases will progress to AML.
Symptoms of Myelodysplastic Syndrome
There are multiple symptoms of myelodysplastic syndrome, many of them non-specific. Signs may include:
- Easy bruising
- Fatigue
- Weight loss
- Petechiae (tiny red spots just under the skin)
- Fever
- Frequent infections
- Weakness
- Shortness of breath
Having one or more of the symptoms listed above does not necessarily mean you have myelodysplastic syndrome However, it is important to discuss any symptoms with your doctor, since they may indicate other health problems.
Types of Myelodysplastic Syndrome
There are many different types of myelodysplastic syndrome. Over the last 30 years, at least three classification systems have been created in an effort to categorize the various disease types. MD Anderson uses the International Prognostic Scoring System (IPSS), a numerical score to determine a patient's risk for myelodysplastic syndrome:
|
Risk Level |
IPSS Score |
|
Low risk |
0 |
|
Intermediate risk 1 |
0.5 – 1 |
|
Intermediate risk 2 |
1.5 - 2 |
|
High risk |
≥2.5 |
The following factors are used to calculate the IPSS score:
|
|
0.0 |
0.5 |
1.0 |
1.5 |
2.0 |
|
% Marrow Blasts |
<5 |
5-10 |
|
11-20 |
21-30 |
|
Cytogenetics |
Normal, -Y |
Other |
-7, del(7)q |
|
|
|
Cytopenias:
|
0/1 |
2/3 |
|
|
|
The IPSS score is also used by oncologists to predict age-related survival rates and progression to acute myelogenous leukemia in MDS patients.
|
|
No. of Patients |
Low |
Int-1 |
Int-2 |
High |
|
A. Median Survival (yr) |
|||||
|
Total Patients (%) |
816 |
267 (33) |
314 (88) |
176 (22) |
59 (7) |
|
Median Survival (yr) |
|
5.7 |
3.5 |
1.2 |
0.4 |
|
Age (yr) |
|
|
|
|
|
|
<60 |
205 (25) |
11.8 |
5.2 |
1.8 |
0.3 |
|
>60 |
611 |
4.8 |
2.7 |
1.1 |
0.5 |
|
<70 |
445 (54) |
9.0 |
4.4 |
1.3 |
0.4 |
|
>70 |
271 |
3.9 |
2.4 |
1.2 |
0.4 |
|
B. 25% AML Evolution (yr) |
|||||
|
Total Patients (%) |
759 |
235 (51) |
295 (39) |
171 (22) |
58 (8) |
|
Median Survival (yr) |
|
9.4 |
3.3 |
1.1 |
0.2 |
|
Age (yr) |
|
|
|
|
|
|
<60 |
187 (25) |
>9.4 (NR) |
6.9 |
0.7 |
0.2 |
|
>60 |
572 |
9.4 |
2.7 |
1.3 |
0.2 |
|
<70 |
414 (55) |
>9.4 (NR) |
5.5 |
1.0 |
0.2 |
|
>70 |
345 |
>5.8 (NR) |
2.2 |
1.4 |
0.4 |
NR = not reached
While recognizing the usefulness of the IPSS, we developed a new prognostic model specifically for the Low and Int-1 subset of MDS patients, below. This is based on an analysis published in Leukemia, 2008; 22: 538-543.
|
Adverse Factor |
Assigned |
|
Unfavorable cytogeneticsa |
1 |
|
Age > 60 years |
2 |
|
Hgb <10 g/dl |
1 |
|
Plt <50 K/ul |
2 |
|
Plt 50–200 K/ul |
1 |
|
BM blasts >4% |
1 |
|
aIn this analysis, diploid and 5q only were favorable cytogenetic, all others were considered as unfavorable cytogenetics. |
|
|
Abbreviations: BM, bone marrow; Hgb, hemoglobin; Plt, platelets. |
|
Estimated survival outcomes within each score range and proposed risk categories
|
Score |
No. of patients |
Median |
Four-year survival (%) |
Category |
|
0 |
11 |
NR |
78 |
1 |
|
1 |
58 |
83 |
82 |
1 |
|
2 |
113 |
51 |
51 |
1 |
|
3 |
185 |
36 |
40 |
2 |
|
4 |
223 |
22 |
27 |
2 |
|
5 |
166 |
14 |
9 |
3 |
|
6 |
86 |
16 |
7 |
3 |
|
7 |
13 |
9 |
NA |
3 |
Abbreviations: NA, not assessable; NR, not reached. |
||||
...AND MD ANDERSON
PREVENTION
Causes & Risk Factors
Most cases of myelodysplastic syndrome have no known cause, but some factors have been determined to increase the risk. Advancing age is perhaps the most common risk factor, since it rarely occurs in people under the age of 60. Other risk factors include smoking, long-term exposure to benzene and prior treatment with chemotherapy or radiation.
TREAT
Treatment
Over the last few years, the care of patients with myelodysplastic syndrome and the understanding of this disease have improved significantly. This has resulted in the development of new therapies.
Lower risk myelodysplastic syndrome patients are treated initially for the specific complications of the disease, such as anemia and low blood counts. If there are indications for more aggressive therapy, strategies that are now considered standard of care include the hypomethylating agents (5-azacitidine and decitabine) and lenalidomide.
Higher risk myelodysplastic syndrome patients usually need more aggressive therapy but much depends on the age and condition of the patient. Younger patients with high risk disease are considered for front-line chemotherapy approaches followed perhaps by allogeneic stem cell transplantation. For older patients, who constitute the majority, intensive chemotherapy is rarely considered. Instead, our strategy focuses on development of active and safe treatments for newly diagnosed patients as well as those who have failed the standard of care based therapies.
Myelodysplastic syndrome is one of the main areas of research of the Department of Leukemia. This is demonstrated by the large number of clinical trials specific for patients with myelodysplastic syndrome and by the extensive basic and translational research exemplified by our NIH funded MDS P01 grant, now in its fourth year, to increase our understanding of the disease. Approximately 300 patients with myelodysplastic syndrome are evaluated in our center per year by a group of physicians completely dedicated to the care of these patients. This care is provided in close collaboration with the referring physicians, a key aspect for the long-term follow up of these patients who will require extensive care during the course of their myelodysplastic syndrome.
SUPPORT
Cancer is a journey that no one needs to take alone. There are many forms of support to help you through every stage: diagnosis, treatment and survivorship. Whether you meet with other cancer survivors like yourself, use complementary therapies or individual coping mechanisms, support is available. Listed below are just some of the ways to find help and hope.
Support Groups
Getting together with other cancer patients in a support group is a valuable coping tool. Support groups are usually focused on a single disease or topic, such as breast cancer survivors or people coping with life-changing side effects from their cancer or cancer therapy. These groups allow participants to meet others like themselves and seek strength from each other. Most major cities and cancer hospitals offer support groups that meet weekly or monthly. There are also dozens of online support Web sites or message boards for those who may not have access to a traditional meeting.
Complementary Therapies
Complementary therapies are used in conjunction with cancer treatment, in an effort to reduce treatment side effects, ease depression and anxiety and help cancer patients take their mind off the negative aspects of their situation. Complementary therapies may include mind-body exercises like yoga, Tai Chi and Qi gong; visualization or guided imagery; using art or music as therapy and self-expression and traditional Eastern medicine such as acupuncture.
Physical Activity
Staying physically active as much as possible during cancer treatment has many positive benefits. Physical activity stimulates the release of endorphins, a hormone that helps elevate mood, as well as decreasing feelings of fatigue.
Exercises for cancer patients can range from simple stretches done in the bed or chair, to more active pursuits such as walking or light gardening work. However, it’s important not to push yourself too hard. Check with your doctor before attempting any physical activity to make sure you are up to it.
Journaling/Blogging
Many people find it helpful to keep a journal of their cancer treatment experience. It may be as simple as recording symptoms and side effects into a notebook, or may include personal emotions and opinions about what they may be going through. Journals can be private, like a diary, or shared with loved ones and even strangers.
Increasingly, people are turning to the Internet to share their cancer journey with the world at large and to seek out others with similar experiences. Many cancer patients have begun their own Web log, or “blog” to publicize their battle with cancer. Twitter, a mini-blogging technology that limits posts to 140 characters, has also proven to be a helpful tool for cancer patients to keep friends updated and reach out to others.
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