@article{oai:niigata-u.repo.nii.ac.jp:00022182, author = {石塚, 利江 and 小田, 良彦 and 高井, 和江 and 真田, 雅好 and 岡崎, 悦夫}, issue = {7}, journal = {新潟医学会雑誌, 新潟医学会雑誌}, month = {Jul}, note = {Transient abnormal myelopoiesis (TAM) with Down Syndrome is characterized by increased blast cells and resembles acute congenital leukemia. This disorder is unique in the point that the hematological abnormality is self-limiting. However, the association between TAM and the subsequent development of acute leukemia is not rully understood. We studied 8 patients of TAM with Down syndrome hospitalized during the recent 7 years. Clinical features of all patients were mostly compatible with the criteria proposed by Nagao, et al. Three had non immune hydorps fetalis (NIHF), two heart anomalies, one pulmonary leukostasis, two liver dysfunction, and two pancytopenia. Two died of NIHF after blast cells had disappeared and the other 6 are alive and healthy, in whom blast cells had disappeared by three months of age. In 6 patients, immunological studies revealed that more than 40% of blast cells were reactive to either platelet peroxidase (PPO) or antiplatelet glycoprotein (Gp) IIb/IIIa complex monoclonal antibodies. In the other two, those studies were not performed because the blast cells had disappeared within 1 week. Chromosomal analysis of blast cells showed all trisomy 21 and no marker chromosome. Colony assays of blood and marrow cells in methylcellulose were performed when diagnosis of TAM was made. CFU-C from blood was increased in three of the four cases. CFU-C from bone marrow was higher than normal in three of the five. These findings suggest that TAM represents a selective expansion of chromosome 21 trisomic blast cells which showed positive reaction to either PPO or Gp IIb/IIIa. The blast cells in TAM expressed megakaryocyte markers.}, pages = {593--597}, title = {Down症候群の新生児にみられたtransient abnormal myelopoiesis(TAM)8例の臨床像(第20回新潟造血器腫瘍研究会十周年記念シンポジウム : 造血器腫瘍十年の歩み)}, volume = {104}, year = {1990} }