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What is Myelodysplastic Syndrome?

I sometimes watch ‘Autopsy: The Last Hours of…’ on REELZ. Recently it featured Larry Hagman of “I Dream of Jeannie” and “Dallas” fame. Mr. Hagman died from Acute Myeloid Leukemia (AML) that had progressed from something called Myelodysplastic Syndrome (MDS).  1/3 of Myelodysplastic Syndrome cases morph into Acute Myeloid Leukemia which is a rapidly growing bone marrow cancer. MDS was previously referred to as a pre-leukemia or called Smoldering Leukemia. Because 2/3 of MDS does not develop into cancer it was thought to have a low malignant potential. As more has become known about MDS it is now considered a type of cancer.

WHAT IS IT?

Myelodysplastic Syndrome is a group of disorders caused by poorly developed blood cells that are not able to function properly. Something has gone wrong in the bone marrow, the spongy stuff found inside most bones. Bone marrow is comprised of blood-forming cells, fat cells and supporting tissue. A small part of the blood-forming cells are known as stem-cells, the building blocks for new cell growth. Dysplastic means ‘abnormal’ and this condition causes these blood-forming cells to become defective which affects red blood cells; the oxygen carriers, white blood cells; the infection fighters and platelets; the clotting mechanism. Defective cells do not live as long as normal cells, leaving the patient ripe for all kinds of issues.

SYMPTOMS

  • Fatigue and shortness of breath
  • Paleness and frequent infections and fever
  • Easy or unusual bleeding and bruising (Petechia)
  • Loss of appetite and weight loss
  • Bone pain

Unfortunately, any or all of these can signify any number of health issues, all of which should merit a visit to your health care professional for a blood test. Too few RBCs brings on anemia and the resulting symptoms. Too few WBC’s may present as leukopenia or neutropenia, often accompanied by frequent or severe infections. Too few platelets cause easy bruising and bleeding seen first as nosebleeds or bleeding gums. As with any cancer, early detection is a key component to its treatment.

CAUSES/RISK FACTORS

When blood cells don’t mature properly, die in the marrow or shortly after entering the blood stream you eventually end up with more immature or defective cells than healthy cells. Most MDS variants have no known cause for this cell behavior but most often is liked to some type of cell mutation.

  • Old Age/Sex: MDS is most commonly found in men in their 70’s and 80’s. It rarely occurs under age 50.
  • Previous cancer treatments: people who have been treated with certain chemotherapy drugs have a higher risk. Combining chemo with radiation therapy elevates risk as do stem-cell transplants. This type of MDS is called ‘Treatment Related MDS’ and only accounts for a small percentage of cases.
  • Genetic Syndromes: Fanconi Anemia, Diamond-Blackfan Anemia or Familial Platelet Disorder.
  • Familial Myelodysplastic Syndrome: a gene mutation that runs families.
  • Environmental Exposures: High dose radiation exposure or long-term workplace exposure to Benzene or other chemicals prevalent in the petroleum or rubber industries.

TREATMENT/MANAGEMENT

There is no known cure for Myelodysplastic Syndrome. Treatment and management become one and the same. The goal is to ease symptoms, offer comfort, slow progression and prevent complications. Options are based on age, overall health, personal preferences, type of MDS (there are 6 recognized by the World Health Organization), prognostics and personal outlook and attitude. Management includes overall healthy behaviors like proper diet and rest, medications designed to boost blood cell production and blood transfusions. Bone marrow or stem-cell transplant offer the only chance at an actual cure but carry a very high risk for serious complications or unintended consequences.

The Myelodysplastic Syndrome Foundation offers up these fun-facts (their words, not mine) regarding this often unrecognized, underdiagnosed and rare group of bone-marrow failure disorder.

  • 33 to 55 people are diagnosed each day
  • 60,000 to 170,000 sufferers currently in the US with 87,000 new cases diagnosed yearly worldwide
  • 75% are men over 60
  • 30% progress to Acute Myeloid Leukemia (AML)
  • Survivability is up to 6 years for low-risk patients down to 5 months for high-risk patients

Patient and family resources are available on the MDS Foundation website that offer information about MDS, bone marrow, clinical trials and specialized treatment centers. They also have a book ‘Building Blocks of Hope” which can be downloaded or available as a hard copy.

Protein-Packed foods to Boost Bone Marrow:

3 ounces of beef, chicken, turkey, pork and lamb: 21 grams

3 ounces of tuna fish: 21 grams

One egg: 6 grams

One-half cup lentils: 9 grams

One-half cup black, kidney and navy beans: 8 grams

3 ounces of tofu: 9 grams

1 ounce of nuts: 4 to 6 grams

5 ounces of nonfat Greek yogurt: 12 to 18 grams

One-half cup cottage cheese: 14 grams

One-third cup quinoa: 6 grams

The goal is to consume 46-56 grams of protein per day and the list above breaks it down into manageable quantities. These foods have the added benefit of coming packed with other nutrients needed to strengthen bone marrow.

“Nothing in life is to be feared, it is only to be understood. Now is the time to understand more so that we can fear less.” Marie Curie

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