Document Detail

HLA-DRnegative, CD34negative Hypergranular Acute Myeloid Leukemia With Trisomy 6 and del(5)(q22q33): Case Report and Review of the Literature.
MedLine Citation:
PMID:  21768886     Owner:  NLM     Status:  Publisher    
We report a unique pediatric case of hypergranular acute myeloid leukemia with myelodysplasia-related changes. The patient presented with moderate leukocytosis with neutrophilia with left-shift maturation and dysplasia, anemia, and multiple sclerotic bone lesions. The bone marrow was hypercellular with a predominance of myeloblast cells and/or abnormal promyelocytes with hypergranular cytoplasm. Flow cytometric immunophenotyping showed that the leukemic cells were positive for CD13, CD33, and myeloperoxidase, and negative for HLA-DR and CD34. Morphology and immunophenotyping were highly suggestive of acute promyelocytic leukemia. The classic t(15;17) or other RARα rearrangements were not detected by cytogenetic or molecular assays, ruling out acute promyelocytic leukemia. Standard cytogenetic analysis showed that the karyotype of the predominant clone was 47,XY,+6 with evidence of clonal evolution to 47,XY,+6,del(5)(q22q33). A literature and database review showed that trisomy 6 is a rare occurrence in hematological malignancies and, to our knowledge, has never been reported in association with del(5)(q22q33) in a child presenting with hypergranular acute myeloid leukemia with myelodysplasia-related changes. We present a current review of the literature and summarize the clinical features of 57 cases of trisomy 6 as the primary chromosomal abnormality in hematological disease.
Bob Argiropoulos; Brian Clifford; Susan Crocker; Elizabeth Sinclair-Bourque; Elizabeth McCready; Jean McGowan-Jordan; Donna L Johnston; Ruth Padmore
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-7-15
Journal Detail:
Title:  Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology     Volume:  -     ISSN:  1536-3678     ISO Abbreviation:  -     Publication Date:  2011 Jul 
Date Detail:
Created Date:  2011-7-19     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
*Department of Genetics, Cytogenetics Laboratory ‡Division of Hematology/Oncology, Children's Hospital of Eastern Ontario †Department of Pediatrics, University of Ottawa §Division of Hematopathology and Transfusion Medicine, The Ottawa Hospital, Ottawa, ON, Canada.
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