Promyelocytic Leukemia

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Martin S Tallman - One of the best experts on this subject based on the ideXlab platform.

  • Strategies for the Treatment of Acute Promyelocytic Leukemia
    Journal of The National Comprehensive Cancer Network, 2020
    Co-Authors: Olga Frankfurt, Martin S Tallman
    Abstract:

    The outcome of acute Promyelocytic Leukemia (APL) has improved dramatically during the past 40 years. Insights into the genetic and biologic mechanisms of APL lead to the development of specific and effective therapeutic strategies. This article discusses the therapeutic interventions that transformed APL from one of the most lethal Leukemias to one that is highly curable.

  • Acute Promyelocytic Leukemia in Children and Adolescents
    Acta Haematologica, 2014
    Co-Authors: Eytan M. Stein, Martin S Tallman
    Abstract:

    Acute Promyelocytic Leukemia (APL) is a rare subtype of AML characterized by a reciprocal balanced translocation between chromosomes 15 and 17 that fuses the PML gene with the RARα gene and leads to t

  • Acute Promyelocytic Leukemia
    Best Practice & Research Clinical Haematology, 2014
    Co-Authors: Martin S Tallman
    Abstract:

    Acute Promyelocytic Leukemia (APL) is designated M3 in the French-American-British (FAB) classification. Because of its unique clinical features and unique response to certain differentiation-inducing agents, and because of our advanced understanding of the molecular biology and treatment of this Leukemia, APL deserves to be presented and discussed in detail, apart from the other acute myeloid Leukemias.

  • Treatment of Acute Promyelocytic Leukemia Without Cytotoxic Chemotherapy
    Oncology, 2011
    Co-Authors: Jae H. Park, Martin S Tallman
    Abstract:

    There has been dramatic progress in the management of acute Promyelocytic Leukemia during the past three decades. Important insights into the pathogenesis of the disease have come to light and effective treatment has been developed.

  • how i treat acute Promyelocytic Leukemia
    Blood, 2009
    Co-Authors: Martin S Tallman, Jessica K Altman
    Abstract:

    Acute Promyelocytic Leukemia is the first malignant disease highly curable with targeted therapy directed at a unique molecular abnormality. The characteristic bleeding diathesis is the most notorious manifestation of the disease, which historically has accounted for a high mortality rate during induction. Acute Promyelocytic Leukemia is one of the few hematologic diseases that must be recognized under the microscope by the practicing hematologist because early institution of all-trans retinoic acid (ATRA) at the first suspicion of the disease before confirmation of the diagnosis and aggressive blood product support are critical to reduce early mortality. ATRA plus anthracycline-based chemotherapy for induction and consolidation followed by maintenance ATRA with low-dose chemotherapy is currently the standard of care. However, the combination of ATRA and arsenic trioxide, with minimal chemotherapy to control leukocytosis, is very effective therapy for newly diagnosed patients. This combination may replace conventional approaches for most, if not all, patients in the very near future. Acute Promyelocytic Leukemia should be considered in any patient with newly diagnosed acute myeloid Leukemia because the treatment is urgent and different from all other subtypes.

Raymond P Warrell - One of the best experts on this subject based on the ideXlab platform.

  • acute Promyelocytic Leukemia
    The New England Journal of Medicine, 1993
    Co-Authors: Raymond P Warrell, Zhenyi Wang, Laurent Degos
    Abstract:

    Acute Promyelocytic Leukemia (APL) is a distinct subtype of acute myeloid Leukemia (AML), identified by the French-American-British (FAB) classification as AML-M3. It accounts for approx 10–15% of all AML cases in most reports (1) but can be as high as 32% in some areas of China (2) and 46% among AML patients of American-Mexican descent (3). Clinically, APL is associated with a high incidence of coagulopathy, either disseminated intravascular coagulation (DIC) or hyperfibrinolysis, which are often aggravated during chemotherapy and result in death at an early stage of treatment. Cytogenetically, APL is characterized in 95% of the cases by a balanced reciprocal translocation between chromosomes 15 and 17, t(15;17)(q22;q21), which leads to the formation of two fusion genes, Promyelocytic Leukemia—retinoic acid receptor α(PML-RARα) or RARα-PML, the former being considered to play a crucial role in leukemogenesis (4).

  • differentiation therapy of acute Promyelocytic Leukemia with tretinoin all trans retinoic acid
    The New England Journal of Medicine, 1991
    Co-Authors: Raymond P Warrell, Stanley R. Frankel, Wilson H Miller, David A Scheinberg, L M Itri, Walter N Hittelman, Rohini Vyas, Michael Andreeff, Agostino Tafuri, Ann A Jakubowski
    Abstract:

    Abstract Background and Methods. Patients with acute Promyelocytic Leukemia have a characteristic (15;17) translocation, with a breakpoint on chromosome 17 in the region of the retinoic acid receptor—alpha (RAR-α). Since this receptor has been shown to be involved with growth and differentiation of myeloid cells in vitro, and since recent clinical studies have reported that tretinoin (all-trans-retinoic acid) induces complete remission in patients with acute Promyelocytic Leukemia, we studied the effects of tretinoin on cellular maturation and molecular abnormalities in patients undergoing the induction of remission with this agent. Results. Eleven patients with acute Promyelocytic Leukemia were treated with tretinoin administered orally at a dose of 45 mg per square meter of body-surface area per day. Nine of the 11 patients entered complete remission. In two patients, complete remission was preceded by striking leukocytosis that then resolved despite continued drug treatment. Serial studies of cellular ...

Laurent Degos - One of the best experts on this subject based on the ideXlab platform.

  • acute Promyelocytic Leukemia
    The New England Journal of Medicine, 1993
    Co-Authors: Raymond P Warrell, Zhenyi Wang, Laurent Degos
    Abstract:

    Acute Promyelocytic Leukemia (APL) is a distinct subtype of acute myeloid Leukemia (AML), identified by the French-American-British (FAB) classification as AML-M3. It accounts for approx 10–15% of all AML cases in most reports (1) but can be as high as 32% in some areas of China (2) and 46% among AML patients of American-Mexican descent (3). Clinically, APL is associated with a high incidence of coagulopathy, either disseminated intravascular coagulation (DIC) or hyperfibrinolysis, which are often aggravated during chemotherapy and result in death at an early stage of treatment. Cytogenetically, APL is characterized in 95% of the cases by a balanced reciprocal translocation between chromosomes 15 and 17, t(15;17)(q22;q21), which leads to the formation of two fusion genes, Promyelocytic Leukemia—retinoic acid receptor α(PML-RARα) or RARα-PML, the former being considered to play a crucial role in leukemogenesis (4).

  • Treatment of acute Promyelocytic Leukemia with all-rans retinoic acid
    Leukemia Research, 1991
    Co-Authors: Pierre Fenaux, Laurent Degos
    Abstract:

    Acute Promyelocytic Leukemia (APL) is a specific type of acute myeloid Leukemia (AML) characterized by the morphology of blast cells (M3 in the French American British classification of AML) [1 2], the t [15 17] translocation [3] which fuses the Promyelocytic Leukemia (PML) gene on chromosome 15 to the retinoic acid receptor (RARα) α gene on chromosome 17 [4 5], and by a coagulopathy combining disseminated intravascular coagulation (DIC) and fibrinolysis [6 8]. Until recently, intensive chemotherapy, usually combining an anthracycline and cytosine arabinoside (AraC) was the only effective treatment of APL [8-22].

Ann A Jakubowski - One of the best experts on this subject based on the ideXlab platform.

  • differentiation therapy of acute Promyelocytic Leukemia with tretinoin all trans retinoic acid
    The New England Journal of Medicine, 1991
    Co-Authors: Raymond P Warrell, Stanley R. Frankel, Wilson H Miller, David A Scheinberg, L M Itri, Walter N Hittelman, Rohini Vyas, Michael Andreeff, Agostino Tafuri, Ann A Jakubowski
    Abstract:

    Abstract Background and Methods. Patients with acute Promyelocytic Leukemia have a characteristic (15;17) translocation, with a breakpoint on chromosome 17 in the region of the retinoic acid receptor—alpha (RAR-α). Since this receptor has been shown to be involved with growth and differentiation of myeloid cells in vitro, and since recent clinical studies have reported that tretinoin (all-trans-retinoic acid) induces complete remission in patients with acute Promyelocytic Leukemia, we studied the effects of tretinoin on cellular maturation and molecular abnormalities in patients undergoing the induction of remission with this agent. Results. Eleven patients with acute Promyelocytic Leukemia were treated with tretinoin administered orally at a dose of 45 mg per square meter of body-surface area per day. Nine of the 11 patients entered complete remission. In two patients, complete remission was preceded by striking leukocytosis that then resolved despite continued drug treatment. Serial studies of cellular ...

Giorgina Specchia - One of the best experts on this subject based on the ideXlab platform.

  • Extramedullar Acute Promyelocytic Leukemia
    Haematology and Blood Transfusion Hämatologie und Bluttransfusion, 2020
    Co-Authors: Giorgina Specchia, E. M. Pogliani, Domenico Pastore, D. Mininni, V Rossi, Anna Mestice, Immacolata Attolico, Vincenzo Liso
    Abstract:

    Acute Promyelocytic Leukemia (APL) is a distinct type of acute myelogenous Leukemia (AML) with peculiar morphologic, cytogenetic and molecular characteristics. Although the most common site of relapse observed in APL patients is the bone marrow (BM), extramedullary disease (EMD) is occasionally observed in these patients. Some debate has arisen as to whether treatment of APL with ATRA predisposes patients to extramedullary relapse.

  • retinoic acid and arsenic trioxide for acute Promyelocytic Leukemia
    The New England Journal of Medicine, 2013
    Co-Authors: Francesco Lococo, Giuseppe Avvisati, Marco Vignetti, Christian Thiede, Simona Iacobelli, Felicetto Ferrara, Paola Fazi, Laura Cicconi, E Di Bona, Giorgina Specchia
    Abstract:

    Background All-trans retinoic acid (ATRA) with chemotherapy is the standard of care for acute Promyelocytic Leukemia (APL), resulting in cure rates exceeding 80%. Pilot studies of treatment with arsenic trioxide with or without ATRA have shown high efficacy and reduced hematologic toxicity. Methods

  • Genomic and molecular switching in relapsed acute Promyelocytic Leukemia
    Leukemia, 2008
    Co-Authors: Francesco Albano, Vincenzo Liso, Alessandra Pannunzio, Luisa Anelli, Antonella Zagaria, Mariano Rocchi, Giorgina Specchia
    Abstract:

    Acute Promyelocytic Leukemia (APL) is characterized by a recurrent translocation between chromosomes 15 and 17, resulting in the fusion of the Promyelocytic Leukemia gene (PML) to the retinoic acid receptor gene (RAR).1 Among APL cases with a PML-RAR rearrangement, chromosome 15 breakpoints fall within three breakpoint cluster regions (bcrs): bcr1 and bcr3 correspond to PML introns 6 and 3, respectively, whereas bcr2 is located within PML exon 6, or very occasionally within PML exon 5. On the contrary, a single RAR breakpoint region occurs within intron 2.2