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Myelodysplastic syndromes

The myelodysplastic syndromes (MDS, formerly known as "preleukemia") are a diverse collection of haematological conditions united by ineffective production of blood cells and varying risks of transformation to acute myelogenous leukemia. Anemia requiring chronic blood transfusion is frequently present. more...

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Signs and symptoms

Abnormalities include:

  • neutropenia, anemia and thrombocytopenia (low cell counts of white & red blood cells and platelets, respectively)
  • abnormal granules in cells, abnormal nuclear shape and size
  • chromosomal abnormalities, including chromosomal translocations.

Symptoms of myelodysplastic conditions:

  • Anemia - chronic tiredness, shortness of breath, chilled sensation, sometimes chest pain
  • Neutropenia (low white cell count) - increased susceptibility to infection
  • Thrombocytopenia (low platelet count) - increased susceptibility to bleeding

All these conditions have an increased risk of developing acute leukaemia, which is notoriously resistant to treatment ("secondary leukaemia").

Diagnosis

Investigation:

  • Full blood count and examination of blood film
  • Bone marrow aspirate and biopsy with examination by an experience hematopathologist
  • Cytogenetics or chromosomal studies. This is performed on the bone marrow aspirate.

Pathophysiology

MDS is due to genetic defects in the multi-potent blood stem cell of the bone marrow. Most of these are not yet described. Differentiation of the abnormal cells is impaired. Clonal expansion of the abnormal cells lead to production of abnormal cells and decreased production of normal bone marrow products.

Bleeding (due to lack of platelets) or infection (due to lack of white blood cells) is common in the majority of MDS patients. In about 25-35% of patients there is a further genetic mutation in one of the abnormal blood stem cells which eventually results in acute leukemia. The progression of MDS to leukemia is a good example of the multi-step theory of carcinogenesis in which a series of mutations occur in an initially normal cell and transform it into a cancer cell.

Types and classification

French-American-British (FAB) classification

In 1974 and 1975 a group of pathologists from France, the United States, and Britain met and deliberated and derived the first widely used classification of these diseases. This French-American-British (FAB) classification was published in 1976 and revised in 1982. Cases were classified into 5 categories: (ICD-O codes are provided where available)

  • (M9980/3) Refractory anemia (RA) - characterized by less than 5% primitive blood cells (myeloblasts) in the bone marrow and pathological abnormalities primarily seen in red cell precursors;
  • (M9982/3) Refractory anemia with ringed sideroblasts (RARS) - also characterized by less than 5% myeloblasts in the bone marrow, but distinguished by the presence of 15% or greater red cell precursors in the marrow being abnormal iron-stuffed cells called "ringed sideroblasts";
  • (M9983/3) Refractory anemia with excess blasts (RAEB) - characterized by 5-19% myeloblasts in the marrow;
  • (M9984/3) Refractory anemia with excess blasts in transformation (RAEB-T) - characterized by 20-29% myeloblasts in the marrow (30% blasts is defined as acute myeloid leukemia);
  • (M9945/3) Chronic myelomonocytic leukaemia (CMML) - not to be confused with chronic myelogenous leukemia or CML - characterized by less than 20% myeloblasts in the bone marrow and greater than 1000 * 109/uL monocytes (a type of white blood cell) circulating in the peripheral blood.

A table comparing these is available from the Cleveland Clinic.

Read more at Wikipedia.org


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2nd Annual MDS Foundation Charity Golf Tournament Attracts PGA Professionals and Other Big Name Sports Figures; Proceeds Support Innovative Research in
From Business Wire, 7/26/05

CROSSWICKS, N.J. -- The second annual MDS Charity Golf Tournament will be held on Monday, August 1, 2005 at Olde York Country Club in Chesterfield, New Jersey. Proceeds from this event support Young Investigator Research Grants which are awarded to Fellows in Hematology undertaking innovative research in MDS.

Celebrity guests include Bruce Fleisher, Senior PGA Professional, winner of the 2001 US Senior Open, Senior PGA Professionals Jay Siegel, Jim Thorpe, Bob Toski, Bob Murphy and Bobby Wadkins. Other celebrity guests will include Joe Thiesman, former Washington Redskins Quarterback, Jim Palmer, three-time Cy Young Award Winner and Baltimore Oriole Baseball Hall of Famer, and Mike Schmidt, former Philadelphia Philly's 3rd base player, winner of the Homerun Crown 8 times and Baseball Hall of Famer.

This year's event will begin with a cocktail reception on Sunday night, July 31st, and continue with a full day of activities on Monday August 1st, including a Breakfast Question and Answer Session with the PGA Pros and Celebrity Guests, Golf Clinic with small group instruction led by the PGA Pros, 18 holes of golf, Meals, Contests and Prizes for Putting, Longest Drive and more.

For sponsorship information, registration or to make a donation, please contact Audrey Hassan, Patient Liaison, MDS Foundation at 609-298-1035 or toll-free at 1-800-637-0839.

About MDS

Myelodysplastic Syndromes (MDS) is a group of diseases in which the bone marrow fails to produce blood cells in the normal manner and often progresses to acute myeloid leukemia. MDS is the most common of all the hematological malignancies and currently affects tens of thousands of people worldwide.

About the MDS Foundation

The Myelodysplastic Syndromes Foundation, Inc. is an international organization serving the educational, research and support needs of clinicians and patients in the area of MDS since 1994. The MDS Foundation is a publicly supported organization, exempt from Federal Income Tax under Section 501(c)(3) of the Internal Revenue Code. Offices are located at 36 Front Street, Crosswicks, New Jersey. Mailing address is The MDS Foundation, P.O. Box 353, Crosswicks, New Jersey, 08515

COPYRIGHT 2005 Business Wire
COPYRIGHT 2005 Gale Group

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