Acute promyelocytic leukemia: Clinical and morphologic features and prognostic factors

Giuseppe Awisati, Francesco Lo Coco, Franco Mandelli

Research output: Contribution to journalArticlepeer-review

Abstract

The most impressive clinical feature of acute promyelocytic leukemia (APL) at diagnosis is the presence in 80% to 90% of patients of a severe hemorrhagic syndrome. Recent data favor a fibrinolytic/proteolytic process rather than a disseminated intravascular coagulation as the mechanism mainly responsible for the hemorrhagic diathesis in APL. Morphologically, two main cytologic variants have been identified: the classical hypergranular APL (M3), which represents the great majority of all APL, and the microgranular variant (M3v), which accounts for about 15% to 20% of all APL. A rare basophilic variant has also been described. With regard to prognosis, it has markedly changed from that of a rapidly fatal acute leukemia to that of a highly curable disease. This revolutionary progress was mainly due to the introduction during the 1990s of all-trans retinoic acid (ATRA) for the treatment of this disease. After the introduction of ATRA, in addition to clinical features such as hyperleukocytosis (white blood cell count > 10 × 109/L) or thrombocytopenia (platelet count <10 × 103/L) at presentation, immunophenotype markers and polymerase chain reaction status for promyelocytic leukemia/retinoic acid receptor-α during follow-up also had an impact on prognosis.

Original languageEnglish
Pages (from-to)4-12
Number of pages9
JournalSeminars in Hematology
Volume38
Issue number1
Publication statusPublished - 2001

ASJC Scopus subject areas

  • Hematology

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