Myelodysplastic Syndrome
Myelodysplastic syndromes have historically been subjected to incomplete definitions and biologic understanding of disease.1,2 With the better understanding of this disease by morphology, cytogenetic evaluation and molecular testing it is now easier to categorize this disease. Myelodysplastic syndrome could not be described as a distinct syndrome until the first half of the 20th century when bone marrow biopsies were started in routine. Still, early suggestive reports can be found in the medical literature: a 1907 report by Luzzatto of megaloblastic “pseudo-aplastic anemia,” 3, could have been MDS cases.
In the mid-1920s, Di Guglielmo in Naples described a group of marrow disorders associated with bizarrely shaped erythrocytes and low peripheral
cell counts , which in some cases ultimately proved fatal.4 For many years thereafter, a heterogeneous group of marrow disorders associated with anemia and erythroid dyplasia were labelled “Di Guglielmo syndrome” by hematologists. The first MDS-specific term, “refractory anemia,” was used in the 1930s to describe patients who had unexplained anemia as a consequence of hypoproliferative marrows and who failed to respond to treatment with the available hematinics: iron salts and the liver extract found by Minot and Murphy in the early 1920s to cure pernicious anemia.5
In 1942, Chevallier and colleagues in France discussed syndromes they labeled as “odo-leukemias.”6 Similarly in 1949, Hamilton-Paterson in London used the term preleukaemic anemia to describe patients with refractory anemia antecedent to AML.7 In 1953, Block and coworkers in Chicago expanded the concept to include cytopenias of all lineages and described cases that closely fit with our current concepts of a clonal myeloid disorder prior to evolution to overt AML.8 In 1956, Björkman inMalmö, Sweden described four cases of idiopathic refractory sideroblastic anemia, one of which terminated in AML.9
In 1970, Dreyfus proposed the designation “les anémies réfractaires avec excès de myeloblasts” (i.e., refractory anemia with excess myeloblasts) and in 1976 he and colleagues proposed a preliminary classification of these syndromes.10 In 1975, at a conference held in Paris, Bessis and Bernard used the term hematopoietic dysplasia, later shortened to myelodysplasia, for the group of disorders having a more indolent course than AML. In the year following the Paris conference, a group of seven hematopathologists from France, the United States, and England—the “French-American-British (FAB) Co-Operative Group”- proposed the term “dysmyelopoietic syndrome” as an alternative to preleukemia. A few years later, “dysmyelopoietic syndrome” was revised to become the “myelodysplastic syndrome(s)” still used today.11 The 1982 FAB classification proved influential and was the basis of subsequent classifications of MDS and related disorders by the World Health Organization (WHO). WHO classification is now considered as a standard system now to prognosticate this disorder according to morphological, cytogenetic and molecular basis.
In the mid-1920s, Di Guglielmo in Naples described a group of marrow disorders associated with bizarrely shaped erythrocytes and low peripheral
cell counts , which in some cases ultimately proved fatal.4 For many years thereafter, a heterogeneous group of marrow disorders associated with anemia and erythroid dyplasia were labelled “Di Guglielmo syndrome” by hematologists. The first MDS-specific term, “refractory anemia,” was used in the 1930s to describe patients who had unexplained anemia as a consequence of hypoproliferative marrows and who failed to respond to treatment with the available hematinics: iron salts and the liver extract found by Minot and Murphy in the early 1920s to cure pernicious anemia.5
In 1942, Chevallier and colleagues in France discussed syndromes they labeled as “odo-leukemias.”6 Similarly in 1949, Hamilton-Paterson in London used the term preleukaemic anemia to describe patients with refractory anemia antecedent to AML.7 In 1953, Block and coworkers in Chicago expanded the concept to include cytopenias of all lineages and described cases that closely fit with our current concepts of a clonal myeloid disorder prior to evolution to overt AML.8 In 1956, Björkman inMalmö, Sweden described four cases of idiopathic refractory sideroblastic anemia, one of which terminated in AML.9
In 1970, Dreyfus proposed the designation “les anémies réfractaires avec excès de myeloblasts” (i.e., refractory anemia with excess myeloblasts) and in 1976 he and colleagues proposed a preliminary classification of these syndromes.10 In 1975, at a conference held in Paris, Bessis and Bernard used the term hematopoietic dysplasia, later shortened to myelodysplasia, for the group of disorders having a more indolent course than AML. In the year following the Paris conference, a group of seven hematopathologists from France, the United States, and England—the “French-American-British (FAB) Co-Operative Group”- proposed the term “dysmyelopoietic syndrome” as an alternative to preleukemia. A few years later, “dysmyelopoietic syndrome” was revised to become the “myelodysplastic syndrome(s)” still used today.11 The 1982 FAB classification proved influential and was the basis of subsequent classifications of MDS and related disorders by the World Health Organization (WHO). WHO classification is now considered as a standard system now to prognosticate this disorder according to morphological, cytogenetic and molecular basis.