Korean Journal of Pediatrics 2004;47(9):1008-1012.
Published online September 15, 2004.
Isolated Intracranial Granulocytic Sarcoma as a Relapse Following Unrelated Bone Marrow Transplantation for Myelodysplastic Syndrome in a 1 Year-Old Infant
Kyung Ran Son1, Hoon Kook1, So Youn Kim1, Hee Jo Back1, Seok Joo Kim1, Ha Young Noh1, Mi Jeong Kim1, Ic Sun Choi1, Shin Jeong2, Jong Hee Nam3, Tai Ju Hwang1
1Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea
2Department of Neurosurgery, Chonnam National University Medical School, Gwangju, Korea
3Department of Pathology, Chonnam National University Medical School, Gwangju, Korea
골수 이형성 증후군 영아에서 비혈연 동종 골수이식 후 단독으로 재발한 두개내 과립구 육종 1례
손경란1, 국훈1, 김소연1, 백희조1, 김석주1, 노하영1, 김미정1, 최익선1, 정신2, 남종희3, 황태주1
1전남대학교 의과대학 소아과학교실
2전남대학교 의과대학 신경외과학교실
3전남대학교 의과대학 병리학교실
Hoon Kook, Email: hoonkook@chonnam.ac.kr
Isolated relapse of myeloid leukemia as a granulocytic sarcoma(GS) following allogeneic bone marrow transplantation(BMT) is very rare manifestation, and usually associated with a poor prognosis. We report a case of isolated intracranial GS in an infant with myelodysplastic syndrome(MDS) following unrelated BMT. A 7 month-old girl was diagnosed with refractory anemia with excess blasts (RAEB). During observation for a couple months several GS developed in the scalp and blast counts in BM increased. Induction chemotherapy resulted in partial remission of BM but GS disappeared. Four months after diagnosis, an unrelated BMT was undertaken. Engraftment was uneventful. On D+160, an intracranial GS of 6.5 cm in size developed. A craniotomy and tumor removal was done. There was no evidence of relapse in BM with complete chimerism. Reinduction chemotherapy using IDA-FLAG resulted in profound neutropenia with pneumonia. She succumbed to respiratory failure despite leukocyte recovery. The optimal management for isolated relapse as GS following BMT should be established.
Key Words: Myelodysplastic syndrome, Bone marrow transplantation, Relapse, Granulocytic sarcoma

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