All issues > Volume 47(9); 2004

Case Report

Korean J Pediatr. 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 KR Son¹, Hoon H Kook¹, So Youn SY Kim¹, Hee Jo HJ Back¹, Seok Joo SJ Kim¹, Ha Young HY Noh¹, Mi Jeong MJ Kim¹, Ic Sun IS Choi¹, Shin S Jeong², Jong Hee JH Nam³, Tai Ju TJ 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

Correspondence Hoon H Kook ,Email: hoonkook@chonnam.ac.kr

Abstract

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.

Keywords :Myelodysplastic syndrome, Bone marrow transplantation, Relapse, Granulocytic sarcoma