HLA-DR antigen-positive acute promyelocytic leukemia

Acute promyelocytic leukemia (APL) with t(15;17)(q22;q21)/PML-RARα is a subtype of acute myeloid leukemia (AML) with distinct morphologic and immunophenotypic characteristics. It is a highly aggressive disease that requires rapid diagnosis and early intervention. In addition to morphologic evaluation, flow cytometry has been widely used to facilitate prompt diagnosis of this disease. Compared with other types of AML, APL typically displays a triad of absent or weak CD34, absent HLA-DR, and positive CD117. HLA-DR positive APL is extremely rare and its clinical and pathological features have not been reported. A total of 45 cases of APL with t(15,17)/PML-RARα were diagnosed at Harbor-UCLA Medical Center from year 2006 to 2015. Among them, only two cases were positive for HLA-DR by flow cytometry immunophenotyping. Here we describe the clinical, morphologic, immunophenotypic, and cytogenetic features of these two cases.