A Case of Monosomy 7 Syndrome Transformed into Acute Myelocytic Leukemia / 대한소아혈액종양학회지
Korean Journal of Pediatric Hematology-Oncology
;
: 352-357, 1999.
Article
in Korean
| WPRIM
| ID: wpr-201406
ABSTRACT
Monosomy 7 syndrome is a rare myeloproliferative disorder of children, and has a clinical presentation similar to JCML. Both syndromes present in children younger than 2 years of age. Pallor, lymphaenopathy, hepatosplenomegaly, recurrent infection, facial rash and petechial bleeding are frequently present. The hematologic picture is characterized by leukocytosis with monocytosis, anemia, thrombocytopenia. It is important to distinguish these two disorders because of their different natural courses. The course of JCML is brief, with most patients dying within 9 months. Patients with monosomy 7 syndrome often present initially with repeated bacterial infections and develop AML after a latent period of 3~6 years. We report a case of monosomy 7 syndrome with typical clinical course in a 3-year-old male patient. The patient experienced a malignant transformation into AML. To our knowledge, this is the first case report of malignant transformation in monosomy 7 syndrome of children in Korea.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pallor
/
Thrombocytopenia
/
Bacterial Infections
/
Myelodysplastic Syndromes
/
Leukemia, Myeloid, Acute
/
Exanthema
/
Hemorrhage
/
Anemia
/
Korea
/
Leukocytosis
Limits:
Child
/
Child, preschool
/
Humans
/
Male
Country/Region as subject:
Asia
Language:
Korean
Journal:
Korean Journal of Pediatric Hematology-Oncology
Year:
1999
Type:
Article
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