Document Detail

Granulocytic sarcoma in children with acute myeloblastic leukemia and t(8;21).
MedLine Citation:
PMID:  9722895     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Granulocytic sarcomas (GS) have been associated with t(8;21). The prognosis of patients with GS is generally regarded as being less favorable than of patients with acute myeloblastic leukemia (AML). GS occurs relatively commonly in Africa and has been reported to affect 10-25% of black children presenting with AML. We sought to establish the incidence of GS in our pediatric population, to determine whether an association with t(8;21) existed, and to report on the outcome of these cases in a single series. PROCEDURE: The records of consecutive pediatric patients treated for de novo AML in Johannesburg between January 1985-December 1995 were reviewed. Fifteen cases of GS among a total of 88 cases of AML presented to the Paediatric Haematology/Oncology Clinics of the Johannesburg and Baragwanath Hospitals. Fourteen (93%) of these patients were black male children. RESULTS: All 9 cases of orbital GS (60%) and almost all cases with concurrent AML M2 had t(8;21). This translocation was present in only 4 n(8.5%) of the remaining 47 AML cases without GS for which cytogenetic data were available. One case presented with a complex chromosomal translocation not previously associated with GS. The median disease-free survival of the GS patients, using conventional chemotherapy treatment protocols, was significantly better than for the patients with AML and no GS (P = 0.0004). CONCLUSIONS: Our data support a strong association between orbital GS, t(8;21), and AML M2 in the pediatric population. This entity occurred virtually exclusively in black male children at presentation. One third of these children who presented with AML had a GS. The favorable prognosis noted in our GS patients on standard induction and intensification therapy without local irradiation conflicts with some previous reports but is consistent with the favorable outcome documented in AML with t(8;21).
R Schwyzer; G G Sherman; R J Cohn; J E Poole; P Willem
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Medical and pediatric oncology     Volume:  31     ISSN:  0098-1532     ISO Abbreviation:  Med. Pediatr. Oncol.     Publication Date:  1998 Sep 
Date Detail:
Created Date:  1998-09-08     Completed Date:  1998-09-08     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7506654     Medline TA:  Med Pediatr Oncol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  144-9     Citation Subset:  IM    
Department of Paediatrics, University of the Witwatersrand, Johannesburg, South Africa.
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MeSH Terms
Acute Disease
African Continental Ancestry Group
Child, Preschool
Chromosomes, Human, Pair 21*
Chromosomes, Human, Pair 8*
Disease-Free Survival
European Continental Ancestry Group
Leukemia, Myeloid / genetics*
Leukemia, Promyelocytic, Acute / genetics*
Orbital Neoplasms / genetics*
Retrospective Studies
South Africa / ethnology
Translocation, Genetic*

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