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 language | English |
|---|---|
| Pages (from-to) | 4-12 |
| Number of pages | 9 |
| Journal | Seminars in Hematology |
| Volume | 38 |
| Issue number | 1 |
| Publication status | Published - 2001 |
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ASJC Scopus subject areas
- Hematology
Cite this
Acute promyelocytic leukemia : Clinical and morphologic features and prognostic factors. / Awisati, Giuseppe; Lo Coco, Francesco; Mandelli, Franco.
In: Seminars in Hematology, Vol. 38, No. 1, 2001, p. 4-12.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Acute promyelocytic leukemia
T2 - Clinical and morphologic features and prognostic factors
AU - Awisati, Giuseppe
AU - Lo Coco, Francesco
AU - Mandelli, Franco
PY - 2001
Y1 - 2001
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=0035145671&partnerID=8YFLogxK
M3 - Article
C2 - 11172535
AN - SCOPUS:0035145671
VL - 38
SP - 4
EP - 12
JO - Seminars in Hematology
JF - Seminars in Hematology
SN - 0037-1963
IS - 1
ER -