Korean J Hematol 2007; 42(4):
Published online December 30, 2007
https://doi.org/10.5045/kjh.2007.42.4.397
© The Korean Society of Hematology
조영욱, 지현숙, 장성수, 박찬정, 서을주, 이제환
울산대학교 의과대학 서울아산병원 진단검사의학교실, 내과학교실
Essential thrombocythemia (ET) is a clonalmyeloproliferative disorder that can rarely transform into acute leukemia in 1∼5% of cases. A recent study has found that a significant proportion of leukemic cases from ET were associated with a cytogenetic abnormality (17p deletion). Herein, we report two cases of acute myeloid leukemic transformations harboring a 17p abnormality from a series of 119 ET patients. The first case, a 48-year-old female, developed acute myeloid leukemia with maturation (AML-M2) accompanying myelodysplasia was diagnosed 6.1 years after the initial diagnosis of ET. She was treated with hydroxyurea. Her karyotype showed a monosomy 17. The second case, a 61-year-old male, developed acute megakaryoblastic leukemia (AML-M7) with a very complex hyperdiploidy including addition of 17p13 that developed 6.5 years after the initial diagnosis. He was treated with hydroxyurea and anagrelide. The immunohistochemistry showed p53 overexpression in both cases. Our cases support the specificity of chromosome 17 abnormality and p53 overexpression in acute leukemic transformation from ET.
Keywords Essential thrombocythemia, Acute leukemic transformation, Chromosome 17, p53 overexpression
Korean J Hematol 2007; 42(4): 397-403
Published online December 30, 2007 https://doi.org/10.5045/kjh.2007.42.4.397
Copyright © The Korean Society of Hematology.
조영욱, 지현숙, 장성수, 박찬정, 서을주, 이제환
울산대학교 의과대학 서울아산병원 진단검사의학교실, 내과학교실
Young Uk Cho, Hyun Sook Chi, Seong soo Jang, Chan Jeoung Park, Eul Joo Seo, Je Hwan Lee
Departments of Laboratory Medicine and, Internal Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
Essential thrombocythemia (ET) is a clonalmyeloproliferative disorder that can rarely transform into acute leukemia in 1∼5% of cases. A recent study has found that a significant proportion of leukemic cases from ET were associated with a cytogenetic abnormality (17p deletion). Herein, we report two cases of acute myeloid leukemic transformations harboring a 17p abnormality from a series of 119 ET patients. The first case, a 48-year-old female, developed acute myeloid leukemia with maturation (AML-M2) accompanying myelodysplasia was diagnosed 6.1 years after the initial diagnosis of ET. She was treated with hydroxyurea. Her karyotype showed a monosomy 17. The second case, a 61-year-old male, developed acute megakaryoblastic leukemia (AML-M7) with a very complex hyperdiploidy including addition of 17p13 that developed 6.5 years after the initial diagnosis. He was treated with hydroxyurea and anagrelide. The immunohistochemistry showed p53 overexpression in both cases. Our cases support the specificity of chromosome 17 abnormality and p53 overexpression in acute leukemic transformation from ET.
Keywords: Essential thrombocythemia, Acute leukemic transformation, Chromosome 17, p53 overexpression
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