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Adele K. Fielding - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia
Current Hematologic Malignancy Reports, 2013Co-Authors: Adele K. Fielding, G. A. ZakoutAbstract:Philadelphia Chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) is characterized by expression of oncogenic fusion product BCR-ABL1, resulting from reciprocal translocation between Chromosomes 9 and 22 [t(9;22)(q34;q11.2)]. Previously perceived to confer poor outcome with at least 10 % lower chance of remission than standard-risk ALL. With the advent of targeted BCR-ABL specific tyrosine-kinase inhibitors (TKIs), higher remission rates were achieved, thus allowing more patients to proceed with the definitive treatment modality—allogeneic hematopoietic stem cell transplantation (alloHSCT). Prime challenges to treatment of Ph+ ALL include appropriate integration of TKIs into remission induction chemotherapeutic regimes, appropriate understanding and implementation of BCR-ABL monitoring for guiding therapeutic intervention(s), and minimizing transplant-related toxicities.
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Current Treatment of Philadelphia Chromosome–Positive Acute Lymphoblastic Leukemia
Hematology. American Society of Hematology. Education Program, 2011Co-Authors: Adele K. FieldingAbstract:The author discusses both the standards of care and more controversial areas in the treatment of Philadelphia Chromosome-positive acute lymphoblastic leukemia.
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Current treatment of Philadelphia Chromosome-positive acute lymphoblastic leukemia.
Haematologica, 2010Co-Authors: Adele K. FieldingAbstract:Philadelphia Chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) is a relatively uncommon disease. However, it accounts for about one quarter of adult cases of ALL. Due to the paucity of patients, randomized controlled trials of therapy are unusual. This, together with the fact that
Guilin Tang - One of the best experts on this subject based on the ideXlab platform.
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Secondary Philadelphia Chromosome acquired during therapy of acute leukemia and myelodysplastic syndrome
Modern Pathology, 2018Co-Authors: Habibe Kurt, Lan Zheng, Hagop M. Kantarjian, Guilin Tang, Farhad Ravandi-kashani, Guillermo Garcia-manero, Zimu Gong, Hesham M. Amin, Sergej N. Konoplev, Mark J. RoutbortAbstract:The Philadelphia Chromosome resulting from t(9;22)(q34;q11.2) or its variants is a defining event in chronic myeloid leukemia. It is also observed in several types of de novo acute leukemia, commonly in B lymphoblastic leukemia, and rarely in acute myeloid leukemia, acute leukemia of ambiguous lineage, and T lymphoblastic leukemia. Acquisition of the Philadelphia Chromosome during therapy of acute leukemia and myelodysplastic syndrome is rare. We reported 19 patients, including 11 men and 8 women with a median age of 53 years at initial diagnosis. The diagnoses at initial presentation were acute myeloid leukemia ( n = 11), myelodysplastic syndrome ( n = 5), B lymphoblastic leukemia ( n = 2), and T lymphoblastic leukemia ( n = 1); no cases carried the Philadelphia Chromosome. The Philadelphia Chromosome was detected subsequently at relapse, or at refractory stage of acute leukemia or myelodysplastic syndrome. Of 14 patients evaluated for the BCR-ABL1 transcript subtype, 12 had the e1a2 transcript. In 11 of 14 patients, the diseases before and after emergence of the Philadelphia Chromosome were clonally related by karyotype or shared gene mutations. Of 15 patients with treatment information available, 7 received chemotherapy alone, 5 received chemotherapy plus tyrosine kinase inhibitors, 2 received tyrosine kinase inhibitors only, and 1 patient was not treated. Twelve patients had follow-up after acquisition of the Philadelphia Chromosome; all had persistent/refractory acute leukemia. Thirteen of 15 patients died a median of 3 months after the emergence of the Philadelphia Chromosome. In summary, secondary Philadelphia Chromosome acquired during therapy is rare, and is associated with the e1a2 transcript subtype, terminal disease stage, and poor outcome.
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3q26.2/EVI1 rearrangement is associated with poor prognosis in classical Philadelphia Chromosome-negative myeloproliferative neoplasms
Modern Pathology, 2017Co-Authors: Zhihong Hu, Guilin Tang, L. Jeffrey Medeiros, Wei Wang, Zi Chen, Parsa Hodjat, Su Yang, Lianghua Fang, Yan Li, Srdan VerstovsekAbstract:Classical Philadelphia Chromosome-negative myeloproliferative neoplasms are a group of closely related myeloid disorders with different histologic features and clinical presentations at an early stage, but all later develop into a similar fibrotic stage with variable risk of acute transformation. The significance of 3q26.2/ EVI1 rearrangement has been well recognized in acute myeloid leukemia, myelodysplastic syndrome, and chronic myeloid leukemia. However, the clinical importance of 3q26.2/ EVI1 rearrangement in classical Philadelphia Chromosome-negative myeloproliferative neoplasms is unknown. Here we reported 15 patients with classical Philadelphia Chromosome-negative myeloproliferative neoplasms showing 3q26.2 rearrangement, including inv(3)(q21q26.2) ( n =6), t(3;21)(q26.2;q22)( n =4), t(3;3)(q21;q26.2)( n =3), inv(3)(q13.3q26.2)( n =1), and t(3;12)(q26.2;p13)( n =1). In addition to 3q26.2 rearrangement, 9 of 15 cases had other concurrent karyotypical abnormalities, including -7/7q- and -5/5q-. There were 8 men and 7 women with a median age of 59 years (range, 35–79 years) at initial diagnosis of myeloproliferative neoplasms: 8 patients had primary myelofibrosis, 4 had polycythemia vera, and 3 had essential thrombocythemia. JAK2 V617F mutation was detected in 8/14 patients, including 4/4 with polycythemia vera. The median interval from the initial diagnosis of myeloproliferative neoplasms to the detection of 3q26.2 rearrangement was 44 months (range, 1–219 months). At time of emergence of 3q26.2 rearrangement, 11 patients were in blast phase and 2 patients had increased blasts (6–19%). Dyspoiesis, predominantly in megakaryocytes, were detected in all patients with adequate specimens at time of 3q26.2 rearrangement. Following 3q26.2 rearrangement, 12 patients received chemotherapy, but none of them achieved complete remission. Of 14 patients with follow-up information, all died with a median overall survival time of only 3 months (range 0–14 months) after the emergence of 3q26.2 rearrangement. In summary, 3q26.2 rearrangement in classical Philadelphia Chromosome-negative myeloproliferative neoplasms is associated with other concurrent cytogenetic abnormalities, a rapid disease progression and blast transformation, a poor response to chemotherapy and a dismal prognosis.
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3q26.2/EVI1 rearrangement is associated with poor prognosis in classical Philadelphia Chromosome-negative myeloproliferative neoplasms
Modern pathology : an official journal of the United States and Canadian Academy of Pathology Inc, 2017Co-Authors: L. Jeffrey Medeiros, Guilin Tang, Wei Wang, Zi Chen, Parsa Hodjat, Su Yang, Lianghua Fang, Srdan VerstovsekAbstract:3q26.2/ EVI1 rearrangement is associated with poor prognosis in classical Philadelphia Chromosome-negative myeloproliferative neoplasms
Srdan Verstovsek - One of the best experts on this subject based on the ideXlab platform.
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3q26.2/EVI1 rearrangement is associated with poor prognosis in classical Philadelphia Chromosome-negative myeloproliferative neoplasms
Modern Pathology, 2017Co-Authors: Zhihong Hu, Guilin Tang, L. Jeffrey Medeiros, Wei Wang, Zi Chen, Parsa Hodjat, Su Yang, Lianghua Fang, Yan Li, Srdan VerstovsekAbstract:Classical Philadelphia Chromosome-negative myeloproliferative neoplasms are a group of closely related myeloid disorders with different histologic features and clinical presentations at an early stage, but all later develop into a similar fibrotic stage with variable risk of acute transformation. The significance of 3q26.2/ EVI1 rearrangement has been well recognized in acute myeloid leukemia, myelodysplastic syndrome, and chronic myeloid leukemia. However, the clinical importance of 3q26.2/ EVI1 rearrangement in classical Philadelphia Chromosome-negative myeloproliferative neoplasms is unknown. Here we reported 15 patients with classical Philadelphia Chromosome-negative myeloproliferative neoplasms showing 3q26.2 rearrangement, including inv(3)(q21q26.2) ( n =6), t(3;21)(q26.2;q22)( n =4), t(3;3)(q21;q26.2)( n =3), inv(3)(q13.3q26.2)( n =1), and t(3;12)(q26.2;p13)( n =1). In addition to 3q26.2 rearrangement, 9 of 15 cases had other concurrent karyotypical abnormalities, including -7/7q- and -5/5q-. There were 8 men and 7 women with a median age of 59 years (range, 35–79 years) at initial diagnosis of myeloproliferative neoplasms: 8 patients had primary myelofibrosis, 4 had polycythemia vera, and 3 had essential thrombocythemia. JAK2 V617F mutation was detected in 8/14 patients, including 4/4 with polycythemia vera. The median interval from the initial diagnosis of myeloproliferative neoplasms to the detection of 3q26.2 rearrangement was 44 months (range, 1–219 months). At time of emergence of 3q26.2 rearrangement, 11 patients were in blast phase and 2 patients had increased blasts (6–19%). Dyspoiesis, predominantly in megakaryocytes, were detected in all patients with adequate specimens at time of 3q26.2 rearrangement. Following 3q26.2 rearrangement, 12 patients received chemotherapy, but none of them achieved complete remission. Of 14 patients with follow-up information, all died with a median overall survival time of only 3 months (range 0–14 months) after the emergence of 3q26.2 rearrangement. In summary, 3q26.2 rearrangement in classical Philadelphia Chromosome-negative myeloproliferative neoplasms is associated with other concurrent cytogenetic abnormalities, a rapid disease progression and blast transformation, a poor response to chemotherapy and a dismal prognosis.
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3q26.2/EVI1 rearrangement is associated with poor prognosis in classical Philadelphia Chromosome-negative myeloproliferative neoplasms
Modern pathology : an official journal of the United States and Canadian Academy of Pathology Inc, 2017Co-Authors: L. Jeffrey Medeiros, Guilin Tang, Wei Wang, Zi Chen, Parsa Hodjat, Su Yang, Lianghua Fang, Srdan VerstovsekAbstract:3q26.2/ EVI1 rearrangement is associated with poor prognosis in classical Philadelphia Chromosome-negative myeloproliferative neoplasms
Hagop M. Kantarjian - One of the best experts on this subject based on the ideXlab platform.
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Secondary Philadelphia Chromosome acquired during therapy of acute leukemia and myelodysplastic syndrome
Modern Pathology, 2018Co-Authors: Habibe Kurt, Lan Zheng, Hagop M. Kantarjian, Guilin Tang, Farhad Ravandi-kashani, Guillermo Garcia-manero, Zimu Gong, Hesham M. Amin, Sergej N. Konoplev, Mark J. RoutbortAbstract:The Philadelphia Chromosome resulting from t(9;22)(q34;q11.2) or its variants is a defining event in chronic myeloid leukemia. It is also observed in several types of de novo acute leukemia, commonly in B lymphoblastic leukemia, and rarely in acute myeloid leukemia, acute leukemia of ambiguous lineage, and T lymphoblastic leukemia. Acquisition of the Philadelphia Chromosome during therapy of acute leukemia and myelodysplastic syndrome is rare. We reported 19 patients, including 11 men and 8 women with a median age of 53 years at initial diagnosis. The diagnoses at initial presentation were acute myeloid leukemia ( n = 11), myelodysplastic syndrome ( n = 5), B lymphoblastic leukemia ( n = 2), and T lymphoblastic leukemia ( n = 1); no cases carried the Philadelphia Chromosome. The Philadelphia Chromosome was detected subsequently at relapse, or at refractory stage of acute leukemia or myelodysplastic syndrome. Of 14 patients evaluated for the BCR-ABL1 transcript subtype, 12 had the e1a2 transcript. In 11 of 14 patients, the diseases before and after emergence of the Philadelphia Chromosome were clonally related by karyotype or shared gene mutations. Of 15 patients with treatment information available, 7 received chemotherapy alone, 5 received chemotherapy plus tyrosine kinase inhibitors, 2 received tyrosine kinase inhibitors only, and 1 patient was not treated. Twelve patients had follow-up after acquisition of the Philadelphia Chromosome; all had persistent/refractory acute leukemia. Thirteen of 15 patients died a median of 3 months after the emergence of the Philadelphia Chromosome. In summary, secondary Philadelphia Chromosome acquired during therapy is rare, and is associated with the e1a2 transcript subtype, terminal disease stage, and poor outcome.
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soho state of the art update and next questions Philadelphia Chromosome positive acute lymphoblastic leukemia
Clinical Lymphoma Myeloma & Leukemia, 2018Co-Authors: Nicholas J Short, Hagop M. Kantarjian, Ching-hon Pui, Anthony H Goldstone, Elias JabbourAbstract:Abstract The widespread adoption of Bcr–Abl-directed tyrosine kinase inhibitors (TKIs) into first-line regimens for patients with Philadelphia Chromosome (Ph)-positive (Ph+) acute lymphoblastic leukemia (ALL) has revolutionized the outcomes of patients with this disease. Whereas Ph+ ALL was historically associated with cure rates of
G. A. Zakout - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia
Current Hematologic Malignancy Reports, 2013Co-Authors: Adele K. Fielding, G. A. ZakoutAbstract:Philadelphia Chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) is characterized by expression of oncogenic fusion product BCR-ABL1, resulting from reciprocal translocation between Chromosomes 9 and 22 [t(9;22)(q34;q11.2)]. Previously perceived to confer poor outcome with at least 10 % lower chance of remission than standard-risk ALL. With the advent of targeted BCR-ABL specific tyrosine-kinase inhibitors (TKIs), higher remission rates were achieved, thus allowing more patients to proceed with the definitive treatment modality—allogeneic hematopoietic stem cell transplantation (alloHSCT). Prime challenges to treatment of Ph+ ALL include appropriate integration of TKIs into remission induction chemotherapeutic regimes, appropriate understanding and implementation of BCR-ABL monitoring for guiding therapeutic intervention(s), and minimizing transplant-related toxicities.
