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Myelodysplasia

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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Obituaries
From Art in America, 9/1/04

Lygia Pape, 77, prominent Brazilian avant-gardist, died May 3 in Rio de Janeiro of myelodysplasia, a blood disease. She worked in a variety of mediums, including artist's books, painting, printmaking, sculpture, installation and dance. In the 1950s, Pape became involved in Concretist, a movement based on the austere geometric style of such European artists as Mondrian and Malevich. At the beginning of the decade, she was producing a series of woodblock prints in which geometric forms blend with the natural wood grain. In 1953, she helped found the Concretist group La Frente, and in the later '50s, she co-founded the innovative Neo-Concretist movement with Hello Oiticica and Lygia Clark. The newer style sought to retain the formal qualities of its predecessor while involving viewers in interactive experiences. In the mid-'60s, she worked with the filmmaking group Cinema Novo. Her work was recently included in "Body and Soul," a comprehensive survey of 400 years of Brazilian art mounted by the Guggenheim Museum, and the 2003 Venice Biennale.

Nigel Greenwood, 62, art dealer, died Apr. 14 of cancer in London. In the 1970s and '80s, his gallery was an important venue in London for a wide range of international avant-garde art. Among the artists he exhibited were Keith Milow, David Tremlett, John Walker, Christopher LeBrun, Ian McKeever, Richard Tuttle, Bernd and Hilla Becher, Gilbert & George and Ed Ruscha. He closed his gallery in 1992, but continued to work as a private dealer and art adviser.

Beatrice Riese, 86, artist and collector, died Apr. 2 in Manhattan of cancer. Born in the Netherlands and raised in Germany, she moved to the U.S. with her parents in 1940. In New York, she studied with Will Barnet and Clyfford Still, and made a living as a textile designer. Her abstract paintings and drawings are characterized by gridded geometric forms. She also served as president of American Abstract Artists for 14 years.

Elise Asher, 92, painter and poet, died Mar. 7 in Manhattan of complications from a broken hip. Known for her abstracted nature scenes and works incorporating poetry--her own and that of others, including her husband, Stanley Kunitz--she had her first solo show in 1953 at Tanager Gallery in New York and published her first book of poetry, The Meandering Absolute, in 1955. In 2000, a retrospective of her work was held at the Fine Arts Work Center in Provincetown, where she lived part of the year, and another is on view at June Kelly Gallery in New York, Sept. 7-Oct. 5.

Noah Sylvester Purifoy, 86, assemblage artist, died Mar. 5 in a fire at his home in Joshua Tree, Calif. Sculptures in his 1968 traveling exhibition, "66 Signs of Neon," were constructed with debris from the 1965 Watts riots. He was co-founder of the Watts Towers Arts Center in 1964, and served on the California Arts Council from 1976 to 1987.

--"Artworld" is compiled by Stephanie Cash and David Ebony

COPYRIGHT 2004 Brant Publications, Inc.
COPYRIGHT 2004 Gale Group

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