All issues > Volume 16(4); 1973

Case Report

J Korean Pediatr Soc. 1973;16(4):335-340. Published online April 30, 1973.

A Case of Di Guglielmo Syndrome Transformed to Myelomonocytic Leukemia( associated with chromosomal abnormalities)

Hee Soon Kye¹, Hong Joon Seo¹, Tae Kyu Park¹, Joong Sik Kim¹, Keun Soo Lee¹

¹Department of Pediatrics, National Medical Center

Abstract

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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