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Int J Clin Exp Pathol 2010;3(1):75-86

Original Article
Diagnosis of biphenotypic acute leukemia: a paradigmatic approach

Xianfeng Frank Zhao, Ivana Gojo, Teresa York, Yi Ning, Maria R. Baer

Departments of Pathology, Medicine and Pediatrics, University of Maryland School of Medicine, and Hematological Malignancies Program,
Marlene and Stewart Greenebaum Cancer Center, Baltimore, MD 21201

Received July 31, 2009; accepted September 23, 2009; Available online October 10, 2009

Abstract: Biphenotypic acute leukemia (BAL), or acute leukemia with a single population of blasts coexpressing markers of two different
lineages, is a rare clinical entity.  To define BAL, a scoring system was proposed by the European Group of Immunological Markers for
Leukemias (EGIL) in 1995.  However, increasing evidence suggests that this system has limitations, as acknowledged by the 2008 World
Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissues.  Although substantially improved in relation to
the EGIL, the new WHO Classification is still not optimal for guiding the clinical management of patients with BAL.  We propose a new
paradigmatic approach to defining BAL based on recent clinical studies of BAL and advances in immunologic marker definition and
cytogenetics, and applied our new approach to 8 cases of “BAL” among a cohort of 742 new acute leukemias in our Cancer Center.  By our new
criteria, 6 cases were reclassified as acute lymphoblastic leukemia (ALL), while only 2 were still classified as BAL.  Our approach is also
supported by analyses of the BAL cases previously reported by other institutions. (IJCEP907009).

Key words: Biphenotypic acute leukemia; AML; ALL; EGIL; classification

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Address all correspondence to:
X. Frank Zhao, MD PhD
Department of Pathology
University of Maryland School of Medicine
10 S. Pine Street, MSTF 711B
Baltimore, MD 21201
Tel: (410)-328-5555
Fax: (410)-328-5508
E-mail: xzhao@umm.edu