Medical College of Wisconsin, USA
Gabriela Gheorghe is board certified in hematopathology in addition to pediatric pathology and anatomic and clinical pathology. Currently, she is working as a Hematopathology Expert at the Children’s Hospital of Wisconsin. A graduate of University of Medicine and Pharmacy Timisoara, Romania,she is a Director of Hematology and Flow Cytometry at The CHW.She has published several papers in reputed journals and is an active member of the Society for Pediatric Pathology and Children’s Oncology Group. She is involved in pathology residents, pediatric hematology/oncology fellows and hematopathology fellows teaching.
The revised 2008 WHO classification of myeloid neoplasms and acute leukemias provides an up to date classification system that is based on peer reviewed, recent articles. Acute myeloid leukemia not otherwise specified (AML, NOS) is a category that will continue to diminish as more cytogenetic subgroups are being recognized. In recent years, new data has emerged, particularly in the adult population, with regards to a new entity that has the potential to be recognized by the next WHO classification system as a distinctive type of leukemia with recurrent cytogenetic abnormality: leukemia with (8;16) translocation. It appears that the pediatric AML with t (8;16), in particular the congenital type might be different from the adult counterpart. Pediatric leukemias with t (8;16) appear to have unique clinical, morphological and immunophenotypic features. Although an aggressive clinical course (including DIC) is common, spontaneous remissions have been described. This type of leukemia may present as congenital leukemia (half of reported cases). Morphologically monocytic/monoblastic differentiation with or without blast hemophagocytosis is often present. By flow cytometry, blasts often show high side scatter and are positive for CD56 and negative for CD34 and CD117. MPO/NSE dual positivity by cytochemistry makes classification a challenging process. The unique gene expression signatures have recently been described in pediatric and adult cases and include overexpression of HOXA genes, a potential target for monitoring minimal disease and possibly a therapeutic target. Literature in the pediatric population is limited to case reports and very small series. We believe that this entity should be included in the upcoming WHO classification of Hematopoietic Tumors and that pediatric AML with t (8;16) is a more heterogeneous entity as compared to the adult type.
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