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KMID : 0367419750160040063
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Journal of Korean Pediatric Society
1975 Volume.16 No. 4 p.63 ~ p.68
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A Case of Di Guglielmo Syndrome Transformed to Myelomonocytic Leukemia
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桂姬順/Park, Hee Soon
徐弘俊/金仲植/朴台圭/李謹洙/Seo, Hong Joon/Park, Tae Kyu/Kim, Joong Sik/Lee, Keun Soo
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Abstract
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Di Guglielmo syndrome has been known as one of the myeloproliferative syndrome, namely a varient form of neoplastic disease.
It is reported that the majority of cases appear in the adults and male is more susceptible than female.
In recent few years, chromosomal aberrations such as aneuploidy, polyploidy, ph(_1) 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 tranformed to myeloblastic leukemia terminally. But Lie-Injo etc. reported that a case of Di Guglielmo 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 brusing 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 chowed excess hypoploid metaphases of various mode (78% of all metaphases) and chromosomal brekage were noted in 0.07/cell.
7 months after conservetive treatment, phipheral blood and bone marrow findings showed markedly increased myelomonocytic cells, which represent a case of Di Guglielmo syndrome transformed to acute myleomonocytic leukemia.
This paper presents the first case of Di Guglielmo syndrome transformed to acute myleomonocytic leukemia, at least among the cases in pediatric age.
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