| A Case of Di Guglielmo Syndrome Transformed to Myelomonocytic Leukemia( associated with chromosomal abnormalities) |
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Hee Soon Kye, Hong Joon Seo, Tae Kyu Park, Joong Sik Kim, Keun Soo Lee |
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Department of Pediatrics, National Medical Center |
| 單核球性 白血病으로 移行된 Di Guglielmo 症候群 1例 一染色體異當을 同伴한一 |
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桂姬順, 徐弘俊, 朴台圭, 金仲植, 李謹洙 |
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國立醫療院 小兒科 |
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| Abstract |
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Di Guglielmo syndrome has been known as one :of the myeloproliferative syndrome, namely a variant form of neoplastic disease. It is reported that the majority of cases appear iri the adults and male is more susceptible than female. In recent few years, chromosomal aberrations such as aneuploidy, polyploidy, phi chromosome etc.
are reported, although the degree and the mode of involvement were variable. And also, it is known that the majority of cases are transformed to myeloblastic leukemia terminally. But Lie-In jo etc. reported that a case of Di Gugliolmo syndrome was transformed to acute monoblastic leukemia, probably after lightening injury. In Korea, Kim et al reported a case of this syndrome transformed to monocytic leukemia. This 9 years old female patient who was visited to NMC because of purpura, easy bruising and pallor, showed characteristic findings such as macrocytic and megaloblastic erythroid cells with bizzare nuclei and mitotic figures, progressive anemia, striking erythroid hyperplasia of bone marrow and gradual development indicative of Di Guglielmo syndrome at first admission. Phipheral blood lymphocyte culture showed excess hypoploid metaphases of various mode (78% of all metaphases) and chromosomal breakage were noted in 0. 07/cell. 7 months after conservative treatment, phipheral blood and bone marrow findings showed markedly increased myelomonocytic cells, which represent a cas; of Di Guglielmo syndrome transformed to acute myelomonocytic leukemia. This paper presents the first case of Di Guglielmo syndrome transformed to myelomonocytic leukemia, at least snnong the cases reported in pediatric age. |
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