Myelodysplastic Syndromes

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Myelodysplastic Syndromes
Myelodysplastic syndromes are a group of diseases (clonal hematopoietic disorders) in which the
bone marrow does not produce enough healthy blood cells.
A person with a myelodysplastic syndrome does not create enough red blood cells, white blood
cells, or platelets. The immature blood cells die early and do not function normally. This is
considered as a premalignant condition in some patients that will progress to leukemia.
Myelodysplastic syndromes are either primary (no known cause) or secondary (as a result of
aggressive cancer treatment complications).
Clinically, many different forms of myelodysplastic syndromes exist, including the following.
Refractory anemia
Patient is anemic and has too few red blood cells, but a normal amount of white blood cells and
platelets. This form has a low prevalence of progression to acute leukemia.
Refractory anemia with ringed sideroblasts
Patient is anemic and has too few red blood cells. The red blood cells have too much iron, but the
number of white blood cells and platelets is normal. This form has a low prevalence of
progression to acute leukemia.
Refractory anemia with excess blasts
Patient has too few red blood cells and may have anemia. The individual has a normal number of
blasts (immature blood cells), with possible changes to the white blood cells and platelets. This
may progress to acute myeloid leukemia.
Refractory anemia with excess blasts in transformation
Patient has too few red blood cells, white blood cells, and platelets, and is anemic with too many
blasts in the blood—known as acute myeloid leukemia.
Refractory cytopenia with multilineage dysplasia
Patient has too few of at least two types of blood cells. Some individuals also will have excess
iron. This can progress to acute leukemia.
Myelodysplastic syndrome associated with isolated del(5q) chromosome
abnormality
Patient has too few red blood cells and anemia, with a specific change in the chromosome.
Unclassifiable myelodysplastic syndrome
Patient has too few of one type of cell in the blood, and a normal number of blasts in the bone
marrow and blood. The disease is not one of the other myelodysplastic snydromes.
Risk factors for development
The following are risk factors for developing myelodysplastic syndromes:
 Male
 Caucasian
 Older than 60 years of age
 Past treatment with chemotherapy or radiation
 Previous autologous bone marrow transplant
 Exposure to some chemicals, including tobacco smoke, pesticides, and solvents
 Exposure to heavy metals, including mercury or lead
 Sometimes preceded by a few years, an unexplained macrocytic anemia with no evidence of
megaloblastic anemia and a mild thrombocytopenia or neutropenia
Signs and symptoms
The following are signs and symptoms of myelodysplastic syndromes:
 Shortness of breath
 Fatigue
 Pallor
 Easy bruising or bleeding
 Petichiae
 Fever
 Frequent infections
 Nose and gum bleeding
 Hemoptysis, hematuria, or blood in stools (may occur)
Treatment and medications
Transfusion of the cells that are missing and treatment of infections are the main treatments.
Life-threatening infections, especially fungal varieties, require granulocytes with antifungal
agents. Some patients will develop an enlarged spleen, which may lead to spontaneous rupture
and intra-abdominal exsanguination. Unfortunately, splenectomy is dangerous and fraught with
complications for these patients.
Transfusing packed red blood cells (RBCs) for severe or symptomatic anemia only helps the
patient for 24 weeks, the life span of RBCs. Some patients receiving RBC transfusion will
require iron chelation therapy to prevent tissue damage. Platelet transfusion is useful to stop
bleeding in patients with thrombocytopenia, but the life span for transfused platelets is only 37
days.
Medications used for these conditions include retinoids, hematopoietic growth factors,
antithymocyte globulin, demethylation agents, and immunomodulators. Cytotoxic chemotherapy
is used in patients with increasing myeloblasts and those who have progressed to acute leukemia.
The usual combination treatment is cytarabine-anthracycline. Bone marrow transplantation is
used in patients with poor prognosis or with advanced myelodysplastic syndromes who are 55
years of age or younger and who have an available donor. However, most patients are elderly,
and only a few young patients have myelodysplastic syndromes, so the use of bone marrow
transplantation is limited.
Stem cell transplantation is becoming more common for patients with myelodysplastic
syndromes and usually is combined with high-dose chemotherapy. This is the only potential cure
for myelodysplastic syndromes, but it is only used in people who are considered high risk, who
have a matched stem-cell donor, and whose life expectancy without successful treatment merits
the risks associated with the treatment itself. Clinical trials are ongoing to look at reducedintensity stem-cell transplantation, which may prove useful in older and/or sicker patients.
Nutrition and supplements
Here are some facts you should know about nutrition and supplements in regard to
myelodysplastic syndromes.
Medications: Many medications commonly prescribed for myelodysplastic syndromes have
important nutritional consequences. Speak to a registered dietitian to learn more about this
important topic.
Herbal supplements: Many herbal supplements are dangerous to people with myelodysplastic
syndromes, including ginseng, St. John’s wort, garlic, Ginkgo biloba, and astragalus.
A neutropenic diet: If your neutrophil count is very low, your doctor might prescribe a
neutropenic diet to reduce your chances of developing a bacterial infection. A registered dietitian
can provide you with information about this diet.
Vitamin B12 and folic acid: Your doctor may prescribe vitamin B12 and folic acid supplements.
Iron: You will most likely need to have your iron levels checked frequently, because levels may
become either too high or too low, depending on your unique medical status.
References and recommended readings
Aplastic Anemia & MDS International Foundation. Frequently asked questions. Available at:
http://www.aamds.org/aplastic/disease_information/qa_library/alternative_therapy/. Accessed
February 19, 2012.
Besa EC. Myelodysplastic syndrome. Available at:
http://emedicine.medscape.com/article/207347-overview. Accessed February 19, 2012.
Leukemia & Lymphoma Society®. Myelodysplastic syndromes. Available at:
http://www.leukemia-lymphoma.org/all_page.adp?item_id=55442. Accessed February 19, 2012.
National Cancer Institute. Myelodysplastic syndromes treatment (PDQ®). Available at:
http://www.cancer.gov/cancertopics/pdq/treatment/myelodysplastic/Patient. Accessed February
19, 2012.
Review Date 2/12
O-0510
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