Chronic Myelogenous Leukemia Presenting in Blast Phase with Nodal, Bilineal Myeloid Sarcoma and T-Lymphoblastic Lymphoma in a Child

• Published in: Pediatric and developmental pathology 2013-03, Vol.16 (2), p.91-96
• Main Authors: Chen, Xueyan, Rutledge, Joe C., Wu, David, Fang, Min, Opheim, Kent E., Xu, Min
• Format: Article
• Language: English
• Subjects: Adolescent; Blast Crisis - genetics; Blast Crisis - pathology; Flow Cytometry; Fusion Proteins, bcr-abl - genetics; Humans; Immunohistochemistry; Immunophenotyping; In Situ Hybridization, Fluorescence; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - genetics; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - pathology; Male; Neoplasms, Multiple Primary - genetics; Neoplasms, Multiple Primary - pathology; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma - genetics; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma - pathology; Sarcoma, Myeloid - genetics; Sarcoma, Myeloid - pathology
• ISSN: 1093-5266; 1615-5742
• DOI: 10.2350/12-07-1230-CR.1

A 14-year-old boy presented with chronic myelogenous leukemia (CML) in blast phase with segregated extramedullary (nodal) myeloid sarcoma and T-lymphoblastic lymphoma. Immunohistochemical stains performed on the lymphadenectomy sample demonstrated T lymphoblasts in the lymph nodes and myeloblasts in the adjacent soft tissue. Fluorescence in situ hybridization performed on paraffin sections confirmed that both T-lymphoblast and myeloblast populations were positive for the t(9;22) BCR/ABL1 translocation. Subsequent flow cytometry analysis of the bone marrow showed expanded populations of abnormal myeloblasts and T lymphoblasts diagnostic of blast phase CML. To the best of our knowledge, bilineal blast phase of CML with segregated extramedullary T lymphoblasts and myeloblasts has not been reported.