Incidence of Philadelphia-chromosome in acute myelogenous leukemia and biphenotypic acute leukemia patients: And its role in their outcome

Atfy, Maha; Azizi, Nashwa M Al; El-Naggar, A. M.

doi:10.1016/j.leukres.2011.04.011

Cited by 21 publications

(19 citation statements)

References 12 publications

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“…Remarkably, co-expression of lymphoid markers was reported in 34.2 % of these cases (14/41), but was not sufficient for a classification as MPAL or BAL according to WHO or EGIL respectively. This is in line with a recent finding by Atfy et al reporting an aberrant lymphoid coexpression in seven out of nine BCR-ABL+ AML cases [80].…”

Section: Clinical Features and Diagnosissupporting

confidence: 93%

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BCR-ABL-positive acute myeloid leukemia: a new entity? Analysis of clinical and molecular features

et al. 2016

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BCR-ABL-positive acute myeloid leukemia (AML) is a rare subtype of AML that is now included as a provisional entity in the 2016 revised WHO classification of myeloid malignancies. Since a clear distinction between de novo BCR-ABL+ AML and chronic myeloid leukemia (CML) blast crisis is challenging in many cases, the existence of de novo BCR-ABL+ AML has been a matter of debate for a long time. However, there is increasing evidence suggesting that BCR-ABL+ AML is in fact a distinct subgroup of AML. In this study, we analyzed all published cases since 1975 as well as cases from our institution in order to present common clinical and molecular features of this rare disease. Our analysis shows that BCR-ABL predominantly occurs in AML-NOS, CBF leukemia, and AML with myelodysplasia-related changes. The most common BCR-ABL transcripts (p190 and p210) are nearly equally distributed. Based on the analysis of published data, we provide a clinical algorithm for the initial differential diagnosis of BCR-ABL+ AML. The prognosis of BCR-ABL+ AML seems to depend on the cytogenetic and/or molecular background rather than on BCR-ABL itself. A therapy with tyrosine kinase inhibitors (TKIs) such as imatinib, dasatinib, or nilotinib is reasonable, but-due to a lack of systematic clinical data-their use cannot be routinely recommended in first-line therapy. Beyond first-line treatment of AML, the use of TKI remains an individual decision, both in combination with intensive chemotherapy and/or as a bridge to allogeneic stem cell transplantation. In each single case, potential benefits have to be weighed against potential risks.

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Section: Clinical Features and Diagnosissupporting

confidence: 93%

“…In a detailed analysis by the German ALL group, 30 % of their biphenotypic acute leukemia (BAL) patients were BCR-ABL positive [79]. In another case series, Afty et al [80] found a shorter survival in the BCR-ABL+ BAL as compared to BCR-ABL+ AML. Furthermore, patients with BAL were older than patients with AML.…”

Section: ) Bcr-abl+ Mixed Phenotype Acute Leukemiamentioning

confidence: 99%

BCR-ABL-positive acute myeloid leukemia: a new entity? Analysis of clinical and molecular features

et al. 2016

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“…5 Ph+ AML cases reported prior to the use of TKIs are not described in our study. 6,23 Ph+ AML and CML-MBC are often clinically similar, and a history of hemogram abnormalities is often unknown. In 2013, two studies showed the specificity of NPM1 mutation in AML and its absence in CML.…”

Section: Discussionmentioning

confidence: 99%

Rare but authentic Philadelphia-positive acute myeloblastic leukemia: Two case reports and a literature review of characteristics, treatment and outcome

Réboursière

Chantepie

Gac

et al. 2015

Hematology/Oncology and Stem Cell Therapy

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The Philadelphia chromosome (Ph+), corresponding to translocation t(9;22), is found in chronic myeloid leukemia (CML) and acute lymphoblastic leukemia. Several cases of Ph+ acute myeloid leukemia (AML) have been reported in the literature. A retrospective study of Ph+ AML between 2001 and 2012 was conducted through a review of the literature. Among 400 AML patients, two cases of Ph+ AML (0.5%) were identified and treated with conventional chemotherapy with or without tyrosine kinase inhibitors (TKIs), followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT). One patient had a complex karyotype including 7 monosomy (-7) and p190 BCR-ABL fusion transcript. Both patients remain in complete molecular remission. To date, 21 Ph+ AML cases treated with TKIs have been described in the literature with a median overall survival of 18months. One-third of the patients had additional karyotypic abnormalities, and 14% had -7. Molecular analysis showed 59% p210 and 41% p190 fusion protein. Relapse rate was observed in 38% of patients with p190 compared to 10% in patients with p210. Allo-HSCT was performed in eight patients; two relapsed (25%). Cytogenetic (-7) and molecular features help to distinguish Ph+ AML from CML. Survival improved with TKIs, particularly in association with conventional chemotherapy and allo-HSCT. Further studies of Ph+ AML patients are needed to better define this entity, its prognostic value, and therapeutic strategy.

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“…Ph -positive AML is rare, representing 0.35-2% of all new cases of AML (11, 28). Three quarters of such patients express the p210 bcr/abl transcript length (11).…”

Section: Hematologist’s Expert Opinionmentioning

confidence: 99%

A 75-Year-Old Woman With Thoracic Spinal Cord Compression and Chloroma (granulocytic sarcoma)

Baikaidi¹,

Chung²,

Tallman³

et al. 2012

Seminars in Oncology

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Incidence of Philadelphia-chromosome in acute myelogenous leukemia and biphenotypic acute leukemia patients: And its role in their outcome

Cited by 21 publications

References 12 publications

BCR-ABL-positive acute myeloid leukemia: a new entity? Analysis of clinical and molecular features

BCR-ABL-positive acute myeloid leukemia: a new entity? Analysis of clinical and molecular features

Rare but authentic Philadelphia-positive acute myeloblastic leukemia: Two case reports and a literature review of characteristics, treatment and outcome

A 75-Year-Old Woman With Thoracic Spinal Cord Compression and Chloroma (granulocytic sarcoma)

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