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Previous Volume 361:1872-1885 November 5, 2009 Number 19 Next

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According to the 2008 World Health Organization (WHO) classification system for hematologic cancers, the primary myelodysplastic syndromes are one of five major categories of myeloid neoplasms (Table 1).¹ The main feature of myeloid neoplasms is stem-cell–derived clonal myelopoiesis with altered proliferation and differentiation. The phenotypic diversity of these neoplasms has been ascribed to different patterns of dysregulated signal transduction caused by transforming mutations that affect the hematopoietic stem cell. There is increasing evidence that haploinsufficiency, epigenetic changes, and abnormalities in cytokines, the immune system, and bone marrow stroma all contribute to the development of the myelodysplastic syndromes. In . . . [Full Text of this Article]

Population at Risk

Pathological Features

Classification

Diagnosis

Karyotypic Abnormalities

Pathogenesis

Clonality and the Stem Cell

Mutations

Mouse Models

The 5q Minus Syndrome, Del(5q), and Haploinsufficiency

Global Gene-Expression Studies

Single-Nucleotide Polymorphisms

Ineffective Hematopoiesis, Accelerated Apoptosis, and Bone Marrow Stroma

Epigenetic Changes

Altered Immune Responses

Leukemic Transformation

Prognostic Scoring

Treatment

Conclusions

Source Information

From the Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN (A.T.); and the Department of Pathology, University of Chicago, Chicago (J.W.V.).

Address reprint requests to Dr. Tefferi at the Division of Hematology, Department of Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55905, or at tefferi.ayalew@mayo.edu.

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