| Myelodysplastic syndrome: pathophysiology and differential diagnosis |
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Yeo-Kyeoung Kim, Hyeoung-Joon Kim |
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| 골수형성이상증후군의 병태생리와 감별진단 |
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김여경 &#; 김형준, Hyeoung-Joon Kim |
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| Abstract |
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Myelodysplastic syndrome (MDS) refers to a group of clonal disorders affect both hematopoietic stem cells and
progenitor cells within the erythrocytic, granulocytic, and megakaryocytic lineages. Ineffective hematopoiesis, the major
manifestation of MDS, arises from a complex interaction between hematopoietic progenitors and the microenvironment,
resulting in premature apoptotic death of progenitors and their maturing progeny. Development and progression of MDS
is suggested to be a multistep alteration to hematopoietic stem cells. Although the molecular pathogenesis of MDS has
not been clearly elucidated, a model of MDS pathogenesis has been proposed whereby a normal hematopoietic stem cell
acquires successive genetic abnormalities that ultimately lead to malignant transformation and clonal expansion. Early
mutations in stem cells may cause differentiation arrest and apoptosis leading to dysplasia and cytopenia, wherease
subsequent defects affecting myeloid cell proliferation may cause the clonal expansion of aberrant cells and leukemic
transformation. The heterogeneity in the clinical and morphologic features in MDS reflects the diversity and complexity
of the underlying genetic defects. Some of these different molecular alterations have been described. However, since the
developing targeted therapeutic advances in MDS will likely depend on a full comprehension of underlying molecular
mechanisms, it may be required to make further progress in understanding the exact pathomechanisms of this disease.
(Korean J Med 76:115-120, 2009) |
| Key Words:
Myelodysplastic syndrome; Pathophysiology; Diagnosis |
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