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Best Pract Res Clin Haematol. 2003 Sep;16(3):369-85.

Expression of cell-surface antigens in acute promyelocytic leukaemia.

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  • 1New York Medical College, Valhalla, NY, USA.


Acute promyelocytic leukaemia (APL) with M3 (or M3v) morphology is the only AML subtype to date for which morphology and immunophenotype agree. In other words, FAB M3 is interchangeable with a unique marker profile. More precisely, we have finally recognized a surrogate marker profile for leukaemia derived from the (15;17) translocation and expressing PML/RARalpha transcripts. To present this as a new development may come as a surprise to many. After all, the antigen expression pattern of AML-M3 was well recognized for many years: absence or weak expression of HLA-DR, CD117, CD15, CD11b and CD34 in the context of a myeloid phenotype (CD33 and CD13 expression) and frequently associated with moderate to high side-scatter appearance upon flow cytometric evaluation, depending upon the degree of granularity of the leukaemic cells. While partially correct, this established APL phenotype is both flawed and limited in its ability to distinguish APL from other AML subtypes, such as natural-killer-cell AML. Given the availability of phenotype-specific therapy for APL, such as all-trans retinoic acid or arsenic trioxide, failing to diagnose APL or misdiagnosing a case of AML with an APL-like phenotype will result in serious clinical consequences. Faced with this dilemma, we have recently performed a comprehensive immunophenotypic analysis of APL patients entered on Eastern Cooperative Oncology Group trials. Our results give diagnostic power to only three antigens, HLA-DR, CD11a and CD18, all of which are characteristically expressed at low levels by APL cells. Despite some significant antigenic differences (e.g. in CD34 expression), this surrogate marker profile for t(15;17) APL applies to both the M3 and the M3v FAB phenotypes and to all three isoforms of the PML/RARalpha transcript.

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