In the office today, four separate breast cancer patients asked about the case of ABC Co-anchor Robin Roberts who announced she has Myelodysplastic Syndrome. Thus, we will take a moment to explain this illness as well as its relationship to cancer and chemotherapy.
Perhaps it is because of its almost unpronounceable name that this dangerous disease, which affects more than 10,000 new patients each year, is almost unknown. Still, every adult hematology and oncology office is full of such patients, because they usually survive for years. The trick to remembering Myelodysplastic (my – low – dis – plastic) Syndrome (also called Myelodyplasia) is the abbreviation MDS. Every nurse and doctor, who treats such patients, will know about what you are talking. It may also give you that slight edge of an expert in the know.
Our bone marrow is a miraculous machine whose purpose is to produce white cells, red cells and platelets. It functions like a combination nursery and assembly line. The original mother cells, known as stem or progenitor cells, produce infant blood cells. These cells go threw a maturation process, which takes days. The final perfect cell is released into the blood when it has grown up and is ready for the real world of the body.
MDS means the miraculous marrow machine is malfunctioning. The cause is genetic injury to one of the mother stem cells. This damage to the mother cells chromosomes results in abnormal baby cells that do not function, fall apart or simply die. While there are certain genetic birth injuries that increase the risk of MDS (Down’s, Neurofibromatosis, Xeroderma pigmentosum) the damage to the gene usually happens during a person’s life. The longer one lives the more chance there is to get MDS. Most MDS patients are over 60 and the highest risk group is more than 80 years old.
Exposures in life such as benzene, smoking and radiation increase the risk. Obesity patients have more MDS. Certain chemotherapies can cause MDS. The chance of chemotherapy resulting in MDS is less than one percent (5 cases per 10,000 treated patients), but that is double the risk without chemotherapy. The longer and more intensive the chemotherapy, the greater the risk.
As the bone marrow begins to make mistakes, there is a slow drop in blood cells in the circulation. Which cells drop depends on what mother cell is injured and what is the injury. Patients may become anemic as their red blood cells drop. This may make them tired and need transfusions. If the white blood cells fall, than the patient may get recurrent infections, especially in the skin (cellulitis). 25% of MDS patients have significant drops in their platelets, which can lead to bleeding. Each case is a little different and which combination of cells is affected is individual.
Studying the patient’s blood under the microscope and doing a bone marrow biopsy determine the specific type of MDS. Not only does the hematologist look at the cells in the marrow and blood, he tests for the type of genetic injury. The treatment and prognosis are determined with this information. Many cases of MDS are so mild that they require no initial therapy and can be watched for years. Some are more severe and require immediate and aggressive intervention.
For patients who are stable and have mild to moderate symptoms, the basic approach is one of support. The doctor will optimize the patient’s general health, make certain the patient has adequate nutritional stores to make blood and monitor the condition. As the disease progresses, which may take years, patients may receive medicine to stimulate their bone marrow and often begin to get transfusions as needed. Since this condition often affects the elderly, more complex treatments may not be possible.
For patients with aggressive MDS that are younger and healthier there are chemotherapies. While this may not seem to make sense, the idea is to suppress the abnormal stem cells so that normal stem cells can take over. These treatments can have significant side effects as the patient is receiving drugs which suppress the bone marrow, when they are already having bone marrow malfunction. At the extreme, it is possible in a few selected patients to wipe out the patients bone marrow with very high dose chemotherapy and transplant new bone marrow. This is semi-experimental and done only in specialized institutions.
Eventually, the bone marrow of each patient gradually fails. About 15% of MDS patients progress to true leukemia (which is why many experts classify MDS as a pre-leukemia or even as a cancer). Despite transfusions and aggressive medical care, this is for most patients a fatal disease. How fast this occurs depends on the type of MDS (which stem cells are damaged). Most patients survive for two to six years, some more. A few deteriorate more quickly.
Myelodysplastic Syndrome is an uncommon, but not rare condition. For most patients there is hope but not yet a cure. There is a great deal of research going on around MDS and the literature is rapidly expanding. Each patient should inquire whether he or she is a candidate for an experimental trial. However, compassionate, comprehensive and continuous medical care, can improve not only the quality of life, but it’s length.
As published in Sunrise Rounds.
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