How I treat Philadelphia chromosome–positive acute lymphoblastic leukemia

• Published in: Blood Vol. 116; no. 18; pp. 3409 - 3417
• Main Author: Fielding, Adele K.
• Format: Journal Article
• Language: English
• Published: Washington, DC Elsevier Inc 04.11.2010; Americain Society of Hematology
• Subjects: Antineoplastic Agents - administration & dosage; Antineoplastic Agents - therapeutic use; Antineoplastic Combined Chemotherapy Protocols; Benzamides; Biological and medical sciences; Central Nervous System Neoplasms - prevention & control; Clinical Trials as Topic; Dasatinib; Drug Resistance, Neoplasm; Fusion Proteins, bcr-abl - genetics; Hematologic and hematopoietic diseases; Hematopoietic Stem Cell Transplantation; Humans; Imatinib Mesylate; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Medical sciences; Mutation; Philadelphia Chromosome; Piperazines - administration & dosage; Piperazines - therapeutic use; Precursor Cell Lymphoblastic Leukemia-Lymphoma - diagnosis; Precursor Cell Lymphoblastic Leukemia-Lymphoma - drug therapy; Precursor Cell Lymphoblastic Leukemia-Lymphoma - genetics; Precursor Cell Lymphoblastic Leukemia-Lymphoma - therapy; Prognosis; Protein Kinase Inhibitors - administration & dosage; Protein Kinase Inhibitors - therapeutic use; Pyrimidines - administration & dosage; Pyrimidines - therapeutic use; Remission Induction - methods; Thiazoles - administration & dosage; Thiazoles - therapeutic use; Philadelphia positive acute lymphoblastic leukemia; Malignant hemopathy; Treatment; Hematology; Lymphoproliferative syndrome; Cancer
• ISSN: 0006-4971; 1528-0020; 1528-0020
• DOI: 10.1182/blood-2010-01-242750

The Philadelphia chromosome is present in approximately 20% to 30% of adults with acute lymphoblastic leukemia (ALL). The poor prognosis of this relatively uncommon acute leukemia has led to the rapid adoption of treatment strategies such as unrelated donor hematopoietic stem cell transplant and tyrosine kinase inhibitors into clinical practice, despite a relative paucity of randomized clinical trials. Recently, there has been a surge of interest in the underlying biology of ALL. In combination with an accumulation of more mature clinical study data in Philadelphia-positive ALL, it is increasingly possible to make more rational and informed treatment choices for patients of all ages. In this article, I review available data and indicate how I personally interpret current evidence to make pragmatic treatment choices with my patients, outside of clinical trials. My strongest recommendation is that all physicians who are treating this rare disease actively seek appropriate clinical trials for their patients wherever possible.