Transient abnormal myelopoiesis in Down's syndrome.
Recent data have elucidated the pathogenesis of transient abnormal myelopoiesis (TAM) to a great extent. TAM is a monoclonal disorder which resolves spontaneously and the target cell in this disorder is a multipotent stem cell which is capable of differentiating into megakaryocytes. The pathogeneses of TAM/AMKL (acute megakaryoblastic leukemia) appears to be closely associated with abnormal quality and quantity of a gene located on chromosome 21. AMKL developing after the regression of TAM appears to come from the same clone as the TAM, which apparently experiences some kind of genetic alterations. It seems that the gene responsible for TAM will soon be cloned in the near future. However, the mechanism of spontaneous regression of TAM has as yet not been clarified. The expanding clone in the transient physiological immunodeficient state, during the perinatal period, might be eliminated with the maturation of more mature immunosurveillance. Alternatively, the TAM clone might be destined to undergo spontaneous death, which is called "programmed cell death" (apoptosis). The mechanism of this phenomenon awaits further elucidation.
Kurahashi
H
H
Department of Pediatrics, Osaka University Hospital, Fukushima, Japan.
Hara
J
J
Yumura-Yagi
K
K
Tawa
A
A
Kawa-Ha
K
K
D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002891:Chromosomes, Human, Pair 21; D004314:Down Syndrome / Q000097:blood / Q000150:complications*; D006412:Hematopoietic Stem Cells / Q000473:pathology; D006801:Humans; D016130:Immunophenotyping; D007938:Leukemia / Q000151:congenital; D007947:Leukemia, Megakaryoblastic, Acute / Q000209:etiology; D008533:Megakaryocytes / Q000473:pathology; D009196:Myeloproliferative Disorders / Q000209:etiology* / Q000235:genetics / Q000473:pathology