Chronic Myeloid Leukemia With Rare Variant b2a3 (e13a3) <i>BCR-ABL1</i> Fusion

Ha, Jihye; Cheong, June Won; Shin, Saeam; Lee, Seung Tae; Choi, Jong Rak

doi:10.3343/alm.2016.36.3.287

Cited by 7 publications

(7 citation statements)

References 10 publications

(14 reference statements)

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“…The clinical significance of the uncommon BCR-ABL1 transcripts is difficult to assess due to their rarity, however, the largest study of e1a2 transcript types indicated this transcript is associated with inferior outcomes to TKI therapy [Verma et al 2009]. In contrast, two case reports have indicated that patients with the e13a3 transcript tend to display good sensitivity to TKI therapy [McCarron et al 2015;Ha et al 2016]. The association between transcript type and outcome has long been debated.…”

Section: Bcr-abl1 Transcript Type As a Predictor Of Response To Tyrosmentioning

confidence: 99%

Current developments in molecular monitoring in chronic myeloid leukemia

Marum

Branford

2016

Therapeutic Advances in Hematology

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Molecular monitoring plays an essential role in the clinical management of chronic myeloid leukemia (CML) patients, and now guides clinical decision making. Quantitative reverse-transcriptase-polymerase-chain-reaction (qRT-PCR) assessment of BCR-ABL1 transcript levels has become the standard of care protocol in CML. However, further developments are required to assess leukemic burden more efficiently, monitor minimal residual disease (MRD), detect mutations that drive resistance to tyrosine kinase inhibitor (TKI) therapy and identify predictors of response to TKI therapy. Cartridge-based BCR-ABL1 quantitation, digital PCR and next generation sequencing are examples of technologies which are currently being explored, evaluated and translated into the clinic. Here we review the emerging molecular methods/technologies currently being developed to advance molecular monitoring in CML.

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Section: Bcr-abl1 Transcript Type As a Predictor Of Response To Tyrosmentioning

confidence: 99%

Current developments in molecular monitoring in chronic myeloid leukemia

Marum

Branford

2016

Therapeutic Advances in Hematology

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“…[ 6 ] Other studies have also reported a favorable response of patients with b2a3 fusion transcript on imatinib treatment. [ 4 5 6 7 8 9 10 ] Furthermore, Jinawath et al reported that CML with BCR/ABL a3 fusion transcripts might have a different response to tyrosine kinase inhibitors compared to CML with BCR/ABL a2 fusion transcripts because of the alterations of tertiary structure. [ 14 ]…”

Section: Resultsmentioning

confidence: 99%

“…[ 3 ] To date, only nine CML cases with this rare fusion transcript have been reported worldwide. [ 4 5 6 7 8 9 10 ] These infrequent fusion transcripts may escape detection or may be misdiagnosed when the commonly used techniques which detect typical fusion transcripts are used. [ 11 ]…”

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confidence: 99%

Chronic myeloid leukemia with a rare fusion transcript, b2a3 (e13a3) BCR–ABL1: A report of four cases from India

Sazawal

Chhikara

Singh

et al. 2019

South Asian J Cancer

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“…In chronic myeloid leukemia patients, this transcript results in an indolent and TKI-responsive form of disease [14–16]; however, its prognostic significance in adult Ph+ ALL patients remains unknown due to the limited number of annotated cases. While the possibility exists of lymphoid blast crisis in chronic myeloid leukemia (CML), this transformation in e13a3 BCR-ABL1 CML is rare [17]. In the absence of basophilia, thrombocytosis, and splenomegaly, this case likely represents de novo Ph+ ALL.…”

Section: Discussionmentioning

confidence: 99%

Molecular Monitoring in Adult Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia with the Variant e13a3 BCR-ABL1 Fusion

Crampe

Kearney

O’Brien

et al. 2019

Case Reports in Hematology

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Monitoring BCR-ABL1 transcript levels in patients with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) is a widely adopted method to assess response to therapy. However, a small minority of Ph+ ALL patients express variant BCR-ABL1 transcript types, usually due to splicing of alternative BCR or ABL1 exons. Whether patients expressing these rare, variant BCR-ABL1 transcripts have a distinct phenotype or response to therapy is not known due to the limited number of reported cases. Here, we report the presenting features of Ph+ ALL in a young adult with a variant e13a3 BCR-ABL1 fusion. Molecular monitoring reflected the disease response from diagnosis through allogeneic stem cell transplantation which resulted in undetectable e13a3 BCR-ABL1 transcripts. This case highlights the value of molecular monitoring in Ph+ ALL patients with variant BCR-ABL1 transcripts and the requirement for standardization of such assays.

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Chronic Myeloid Leukemia With Rare Variant b2a3 (e13a3) BCR-ABL1 Fusion

Cited by 7 publications

References 10 publications

Current developments in molecular monitoring in chronic myeloid leukemia

Current developments in molecular monitoring in chronic myeloid leukemia

Chronic myeloid leukemia with a rare fusion transcript, b2a3 (e13a3) BCR–ABL1: A report of four cases from India

Molecular Monitoring in Adult Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia with the Variant e13a3 BCR-ABL1 Fusion

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