Clinical manifestations, pathologic features, and diagnosis of acute promyelocytic leukemia in adults
- Richard A Larson, MD
Richard A Larson, MD
- Editor-in-Chief — Hematology
- Section Editor — Leukemia
- Professor of Medicine
- University of Chicago Pritzker School of Medicine
- Sandeep Gurbuxani, MBBS, PhD
Sandeep Gurbuxani, MBBS, PhD
- Assistant Professor, Section of Hematopathology, Department of Pathology
- University of Chicago
Acute myeloid leukemia (AML) refers to a group of hematopoietic neoplasms involving cells committed to the myeloid lineage. Acute promyelocytic leukemia (APL) is a biologically and clinically distinct variant of AML. APL was classified as AML-M3 in the older French-American-British (FAB) classification system and is currently classified as acute promyelocytic leukemia with t(15;17)(q24.1;q21.2);PML-RARA in the World Health Organization classification system . (See "Classification of acute myeloid leukemia".)
APL represents a medical emergency with a high rate of early mortality, often due to hemorrhage from a characteristic coagulopathy. It is critical to start treatment with a differentiation agent (eg, all-trans retinoic acid) without delay as soon as the diagnosis is suspected based upon cytologic criteria, and even before definitive cytogenetic or molecular confirmation of the diagnosis has been made.
The clinical features, diagnosis, and prognosis of APL in adults will be reviewed here. The following other topics are covered separately:
●Molecular biology of APL (see "Molecular biology of acute promyelocytic leukemia")
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- CLINICAL FEATURES
- General features
- Coagulopathy and APL
- PATHOLOGIC FEATURES
- - Hypergranular form
- - Microgranular variant
- Molecular genetics
- Variant RARA translocations
- - Conventional karyotype
- - Fluorescence in situ hybridization
- - Reverse transcriptase polymerase chain reaction
- - Anti-PML monoclonal antibodies