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KMID : 0387820020090010030
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Clinical Pediatric Hematology-Oncology
2002 Volume.9 No. 1 p.30 ~ p.37
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Minimally Differentiated Acute Myeloid Leukemia (AML-M0) in Children: Clinico-biological Characteristics of 4 Cases
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Kim So-Youn
Kook Hoon
Yang Sung-Jin
Cho Duck
Kim Hyun-Jung
Choi Ik-Sun
Ryang Dong-Wook
Hwang Tai-Ju
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Abstract
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Purpose:AML M0, a newly defined entity of AML, is a rare subtype with dismal outcome. We investigated the clinico-biologic characteristics of 4 AML M0 cases in childhood.
Methods:We reviewed the medical records of 4 AML M0 patients diagnosed at the Pediatric Department of Chonnam National University Hospital from Jan. 1995 to Dec. 2001. We analyzed the clinical, cytologic, cytochemical, immunologic and cytogenetic findings. Details on the treatments and their results were also described.
Results:AML M0 accounted for 5.4% (4/75) of newly diagnosed AML. Two of them were less than 2 years of age. One case was secondary leukemia following autologous transplantation for ALL L3. Morphologically, 3 cases displayed myeloblasts. All cases were having less than 3% of the blasts positive for myeloperoxidase (MPO) or Sudan black B (SBB) by light microscopy. Early precursor markers, such as CD34, HLA-DR, and CD7 were expressed in 100%, 100%, and 50% of cases, respectively. CD13 and/or CD33 were positive in all cases. Karyotypic abnormalities were demonstrated in 3: 2 involving chromosome 7 and one with complex abnormality. Three patients died from relapse and treatment related complications with the median survival duration of 4 mo (range 1-7 mo).
Conclusion:AML M0 should be considered in leukemia cases negative for MPO/SBB, negative for lymphoid specific markers, and positive for myeloid markers. As those patients may have low possibility of getting remission as well as high risk of early relapse, more effective treatment strategies, such as stem cell transplantations, should be developed.
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KEYWORD
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AML MO, Cytochemistry:Clinical characteristics, Immunophenotype, Cyto-genetics
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