Two Cases of Acute Leukemic Transformation with a Chromosome 17 Abnormality and p53 Overexpression Evolving from Essential Thrombocythemia.
10.5045/kjh.2007.42.4.397
- Author:
Young Uk CHO
1
;
Hyun Sook CHI
;
Seongsoo JANG
;
Chan Jeoung PARK
;
Eul Joo SEO
;
Je Hwan LEE
Author Information
1. Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea. hschi@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Essential thrombocythemia;
Acute leukemic transformation;
Chromosome 17;
p53 overexpression
- MeSH:
Chromosome Aberrations;
Chromosomes, Human, Pair 17*;
Diagnosis;
Female;
Humans;
Hydroxyurea;
Immunohistochemistry;
Karyotype;
Leukemia;
Leukemia, Megakaryoblastic, Acute;
Leukemia, Myeloid, Acute;
Male;
Middle Aged;
Monosomy;
Sensitivity and Specificity;
Thrombocythemia, Essential*
- From:Korean Journal of Hematology
2007;42(4):397-403
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
