MDS: beginning to understand

what is MDS???
The Hutchinson Cancer Research Center and the Seattle Cancer Care Alliance say:
"The term "myelodysplastic syndrome" describes diseases of the bone marrow that are generally associated with anemia (with or without low platelet and white blood cell counts) and characterized by a tendency to develop into acute myeloid leukemia. MDS is sometimes referred to as 'preleukemia.'"

http://www.seattlecca.org/aboutscca/pressRoom/archive/
winter2003.htm

they go on to say: "Clinicians and researchers agree that MDS is a disorder of blood- forming (hematopoietic) stem cells and, as such, can be treated by hematopoietic cell transplantation. At present, hematopoietic cell transplantation is probably the only therapy with curative potential for the disease, and it remains the standard of care for those who can tolerate its rigors.

"For some patient groups, the success rate is as high as 70 to 75 percent. However, age and other critical factors disqualify many patients from consideration for transplantation. Some patients with good risk features such as normal chromosomes and low blast-cell counts in the marrow, or patients with what has been termed "sideroblastic" anemia, may have a life expectancy of five to 10 years, or even longer with more conservative management.

"Nevertheless, the need for continuous and often progressively increasing transfusion support is cumbersome, expensive, impairs the quality of life and is associated with long- term side effects.

"Patients with low platelet counts run the risk of hemorrhage, and in those with low white-cell counts, the risk of infection is considerable. Forty to 50 percent of MDS patients die from causes related to these complications."

humm i wonder what the other 50% die of? complications from treatment?

oh here's something nice - they say:

"MDS can evolve slowly and along various paths, and the natural history of this particular disease is especially challenging to understand, according to Dr. Janis Abkowitz, head of the SCCA hematology clinic and a UW professor of medicine. Crucial decisions, such as if and when to do a transplant, rest on a thorough understanding of each patient and the development of his or her disease."

Hutch in Seattle explains some of the subtypes:

"Doctors divide MDS into subtypes based on factors such as:

* Whether you have low levels of RBCs, WBCs, or platelets (or low levels of more than one type of blood cell) in your blood.

* Whether your have blast cells in your blood or your bone marrow, and what percentage of the blood or marrow is made up of these blasts.

* Whether the marrow shows dysplasia in only one type of blood-making cell (unilineage dysplasia) or in more than one type of blood-making cell (multilineage dysplasia)

* Whether there are chromosome abnormalities in the marrow cells and, if so, which types of abnormalities."

" Doctors also look at the surface of MDS cells to see whether the cells express certain antigens. Some of these antigens may cause responses from the patient's own immune system. "

Once we have this info we can classify the disease - of course there are several systems of classification. there's the French, American, English one, there's WHO's system and there's a new one that just measures your risk range.

"According to the World Health Organization (WHO) system of MDS classification:
* Refractory anemia (RA).
* Refractory anemia with ringed sideroblasts (RARS). Ringed sideroblasts are one type of cells that are precursors to RBCs.
* Refractory cytopenia with multilineage dysplasia (RCMD). If a person has multilineage dysplasia and ringed sideroblasts, this is classified as RCMD-RS.
* Refractory anemia with excess blasts-1 (RAEB-1) and refractory anemia with excess blasts-2 (RAEB-2). RAEB-2 is similar to RAEB-1 but with a higher percentage of blasts in the blood and the marrow.
* Myelodysplastic syndrome, unclassified (MDS-U).
* MDS associated with isolated del(5q). The term "del(5q)" means that part of chromosome #5 is missing.

View details of the WHO classification system. http://bloodjournal.hematologylibrary.org/cgi/content/full/100/7/2292 http://www.seattlecca.org/patientsandfamilies/adultCare/clinicalProgs/MyelodysplasticSyndrome/PatientEducation.htm

In Canada they are a bit more bunt when answering the question: What is MDS?

"MDS, or myelodysplastic syndromes, is a group of bone marrow diseases. Normal bone marrow produces three different cell types that help make up the blood - red blood cells, white blood cells, and platelets. In MDS the bone marrow is "injured", that is, loses its ability to produce normal cells of one or more of these types. The diseased cells are called myelodysplastic cells.

"MDS is primarily a disease of the aging, most patients being over 65. MDS does not necessarily shorten life expectancy. The bone marrow's failure to produce normal cells is a gradual process, and the elderly often succumb to other diseases before MDS takes its toll. It is possible, however, that in time the bone marrow will fail completely, making patients unable to fight infection or prevent bleeding.

"There is also the ever present risk of MDS progressing into acute myeloid leukemia (AML), which does not respond well to chemotherapy. AML is a bone marrow malignancy where the marrow is replaced by a population of extremely immature or primitive "blast" or stem cells. Therefore, there is a severe shortage of normal cells. But the majority of patients, roughly 70%, never develop leukemia. In other words, MDS shortens life in some patients but not in others. Eventually 70 to 75% of patients with MDS succumb to either complications or progression to AML." http://www.neutropenia.ca/research/mds.html

The Canadian site also gets quickly to the point concerning the question:
How is MDS Treated?
"Treatment for MDS depends on two main factors: the degree to which blood cell counts are reduced, and the risk of progression to AML.

"Induction Chemotherapy
"In the high-risk group and the intermediate-risk group II, there is a high rate of progression to AML. These patients should consider receiving intravenous chemotherapy. Relatively strong doses of chemotherapy are given to "induce" control of the disease." Unfortunately, the chance of controlling MDS with induction chemotherapy is only about 30%. Even in successful cases, the disease often returns within twelve months. Thus aggressive chemotherapy is given to a minority of MDS patients." http://www.neutropenia.ca/research/mds.html

hey, I ask myself, isn't that the same deal as AML?
well the Canadian site reveals that there's also

"Bone Marrow Transplantation
Bone marrow transplantation is becoming more effective as therapy, particularly for AML. The ultimate goal of transplantation is to cure patients by completely eliminating their myelodysplastic cells. To date, about 500 MDS patients have undergone bone marrow transplantation. Almost all have been under the age of 40. In patients over the age of 55, the risks of transplantation clearly outweigh the benefits, given the high risk that the donated marrow (graft) will reject the patient (host). Because MDS is a disease of the elderly, bone marrow transplantation doesn't represent a viable option for most patients.

In addition to being young enough, candidates for transplants must have a sibling who is the same transplantation type. That is, there must be an HLA match between the two. HLA type - not to be confused with blood type - is determined through a blood test. Unfortunately, children and parents of the patient do not qualify as HLA matches. "

research on other sites reveal this to be a narrow interpretation of options for BMT so keep reading the whole column.

the Canadian site  continues:

"Even in younger patients, bone marrow transplantation is a high-risk procedure. Within the first year there is a 30 to 50% chance of dying from transplant complications, either graft vs. host disease, or damage to the liver or lungs. Patients who survive these complications, however, have a good chance of being cured. There is clearly no right answer to the question of whether patients under the age of 55 who have an HLA match should undergo transplantation. For people without other major medical problems, certainly the risks of life-threatening transplant complications are lower than the risks of delaying, or not receiving, a transplant"

 http://www.neutropenia.ca/research/mds.html

there are many other treatments below these which are the most severe but the only ones likely to result in remission (sometimes optimistically called cure).
Of course i'd rather remission!
next time i'll list some of the approaches -it helps me to understand- and talk about that life expectancy thing.

later
kayla