The myelodysplastic syndrome (SMD) represents a heterogeneous group of acquired and clonal hematologic disorders which affect the hemocytopoietic Stem Cell. They are characterized morphologically and clinically by ineffective hematopoiesis, progressive peripheral cytopenia, dysplasia in one or more cellular lineages, hypercellular and dysplasic bone marrow with variable percentage of blasts, in most cases; and evolutionary trend to acuted leukaemia. The transformation process in MDS is a multistep process causing the accumulation of genetic lesions involving genes that govern the mechanisms of proliferation and differentiation of hematopoietic precursors. In 1982, the French-American-British Hematology group (FAB) classified the SMD according to morphologic criteria in peripheral blood and bone marrow. Controversies and disagreements forced the WHO in 1999 to review the classification and to propose a new one. The WHO classification system introduces the importance not only of the morphologic characteristics, but also the relevance of clinical and cytogenetic evaluation, and the use of the inmunophenotype and the biological information to be able to define the different entities. The WHO classification correlates better with the prognosis, the therapeutic response, and the progression than the FAB classification. The International Prognostic Scoring System (IPSS) provides an improved method for evaluating prognosis in MDS. This classification system should prove useful for more precise design and analysis of therapeutic trials in this disease. With regard to treatment, present-day hope is placed in gene therapy. The development of new drugs directed against specific targets is the hope for the future.
机构:
Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Ludwig Boltzmann Cluster Oncol, Vienna, AustriaMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Valent, Peter
Orazi, Attilio
论文数: 0引用数: 0
h-index: 0
机构:
New York Presbyterian Hosp, Weill Cornell Med Ctr, New York, NY USAMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Orazi, Attilio
Buesche, Guntram
论文数: 0引用数: 0
h-index: 0
机构:
Hannover Med Sch, Inst Pathol, D-3000 Hannover, GermanyMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Buesche, Guntram
论文数: 引用数:
h-index:
机构:
Schmitt-Graeff, Annette
George, Tracy I.
论文数: 0引用数: 0
h-index: 0
机构:
Stanford Univ, Dept Pathol, Sch Med, Stanford, CA 94305 USAMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
George, Tracy I.
Sotlar, Karl
论文数: 0引用数: 0
h-index: 0
机构:
Univ Munich, Inst Pathol, D-80539 Munich, GermanyMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Sotlar, Karl
Streubel, Berthold
论文数: 0引用数: 0
h-index: 0
机构:
Med Univ Vienna, Inst Pathol, Vienna, AustriaMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Streubel, Berthold
Beham-Schmid, Christine
论文数: 0引用数: 0
h-index: 0
机构:
Med Univ Graz, Inst Pathol, Graz, AustriaMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Beham-Schmid, Christine
Cerny-Reiterer, Sabine
论文数: 0引用数: 0
h-index: 0
机构:
Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, AustriaMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Cerny-Reiterer, Sabine
Krieger, Otto
论文数: 0引用数: 0
h-index: 0
机构:
Elisabethinen Hosp Linz, Dept Internal Med 1, Linz, AustriaMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Krieger, Otto
van de Loosdrecht, Arjan
论文数: 0引用数: 0
h-index: 0
机构:
Vrije Univ Amsterdam Med Ctr, Amsterdam, NetherlandsMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
van de Loosdrecht, Arjan
Kern, Wolfgang
论文数: 0引用数: 0
h-index: 0
机构:
MLL Munich Leukemia Lab, Munich, GermanyMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Kern, Wolfgang
Ogata, Kiyoyuki
论文数: 0引用数: 0
h-index: 0
机构:
Nippon Med Sch, Dept Med, Div Hematol, Tokyo 113, JapanMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Ogata, Kiyoyuki
Wimazal, Friedrich
论文数: 0引用数: 0
h-index: 0
机构:
Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Med Univ Vienna, Dept Obstet & Gynaecol, Vienna, AustriaMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Wimazal, Friedrich
Csomor, Judit
论文数: 0引用数: 0
h-index: 0
机构:
Semmelweis Univ, Inst Pathol, Budapest, HungaryMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Csomor, Judit
Varkonyi, Judit
论文数: 0引用数: 0
h-index: 0
机构:
Semmelweis Univ, Dept Hematol, Budapest, HungaryMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Varkonyi, Judit
Sperr, Wolfgang R.
论文数: 0引用数: 0
h-index: 0
机构:
Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, AustriaMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Sperr, Wolfgang R.
Werner, Martin
论文数: 0引用数: 0
h-index: 0
机构:
Univ Freiburg, Dept Pathol, D-7800 Freiburg, GermanyMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Werner, Martin
Kreipe, Hans
论文数: 0引用数: 0
h-index: 0
机构:
Hannover Med Sch, Inst Pathol, D-3000 Hannover, GermanyMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria
Kreipe, Hans
Horny, Hans-Peter
论文数: 0引用数: 0
h-index: 0
机构:
Inst Pathol, Ansbach, GermanyMed Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria