Clinical Response to Venetoclax and Decitabine in Acute Promyelocytic Leukemia With a Novel RARA-THRAP3 Fusion: A Case Report

Song, Baoquan; Wang, Xin; Kong, Xiangpeng; Wang, Man; Li, Yao; Shen, Hongjie; Zhang, Jian; Qiu, Huiying

doi:10.3389/fonc.2022.828852

Cited by 16 publications

(10 citation statements)

References 13 publications

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“…Interestingly, in the literature, we found several descriptions of APL cases with rare fusion genes that were treated with venetoclax. The positive fusion genes in these cases included STAT5B::RARA, HNRNPC::RARA, and THRAP3::RARA [13][14][15]. These special types of APL cases, with no CNS involvement, have also achieved good results.…”

Section: Discussionmentioning

confidence: 91%

Treatment of central nervous system relapse in PLZF::RARA-positive acute promyelocytic leukemia by venetoclax combined with arubicin and cytarabine: a case report

Zhou

Zhu

Wang

et al. 2023

Preprint

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Background: Patients with refractory acute promyelocytic leukemia and central nervous system relapse often have a poor prognosis. Among them, patients with the PLZF::RARA rearrangement have a poor response to all-trans retinoic acid and conventional chemotherapy. Venetoclax, a selective inhibitor of B-cell lymphoma-2 (BCL-2), can cross the blood–brain barrier and has been widely used in the treatment of acute myeloid leukemia in recent years. Case presentation: We report a case of central nervous system relapse in a patient with acute promyelocytic leukemia with PLZF::RARA rearrangement who achieved complete remission after treatment with anthracycline cytotoxic chemotherapy in combination with venetoclax. The concentration of venetoclax in the cerebrospinal fluid (CSF) was found to be approximately 1/1000 of that in the plasma based on liquid chromatography–tandem mass spectrometry. After the first treatment course, the PLZF::RARA test result for the patient’s marrow fluid sample turned negative. After the third treatment course, abnormal promyelocytic leukemia cells in the CSF were not detected using flow cytometry, and the PLZF::RARA test in the CSF remained negative. Conclusion: This case report highlights a new approach to the treatment of central nervous system relapse in patients with PLZF::RARA-positive acute promyelocytic leukemia.

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Section: Discussionmentioning

confidence: 91%

Treatment of central nervous system relapse in PLZF::RARA-positive acute promyelocytic leukemia by venetoclax combined with arubicin and cytarabine: a case report

Zhou

Zhu

Wang

et al. 2023

Preprint

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“…Interestingly, we found several cases treated with Venetoclax-based therapy: Liu et al 12 reported a novel HNRNPC-RARA fusion in variant APL who was sensitive to Venetoclax-based therapy. Song et al 13 identified THRAP3 as novel RARA fusion gene. This patient was salvaged by low-dose Venetoclax and decitabine.…”

Section: Discussionmentioning

confidence: 99%

Treatment of STAT5b-RARA positive acute promyelocytic leukemia by Venetoclax combining with homoharringtonine, cytarabine: A case report and literature review

et al. 2022

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Introduction: Acute promyelocytic leukemia (APL) is mostly due to the chromosome translocation t (15; 17) (q22; q12), leading to the formation of PML-RARA fusion protein. Some patients carried rare translocation involving RARA gene, who were called variant APL caused by RAR family (RARA, RARB, and RARG) and partner genes. STAT5b-RARA was a rare type of molecular genetic abnormality with unfavorable prognosis which have been reported in only 18 cases in variant APL. Knowledge of STAT5b-RARA (+) APL treatment is still limited. Case report: We presented a 38-year-old female variant APL case, who was STAT5b-RARA positive detected by reverse transcription polymerase chain reaction. The patient failed to respond after four-drug combined induction chemotherapy: idarubicin, cytarabine, all trans retinoic acid, and arsenic trioxide (As 2 O 3 ). Then, the patient was re-induced with azacytidine, but still failed to achieve complete remission (CR). Next, she was treated with Venetoclax combining with homoharringtonine and cytarabine as the salvage therapy and achieved CR. Later, the patient received hematopoietic stem cell transplantation after 4 cycles of consolidation therapy. Conclusion: Venetoclax combining with homoharringtonine and cytarabine has been used as the salvage therapy in the STAT5b-RARA positive APL successfully.

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“…Furthermore, sporadic case reports documented the successful treatment of ATRA/ATO‐resistant variant APLs with venetoclax monotherapy or in combination with chemotherapy (Table 1). 6–10 This is the first study to report on the efficacy and possible mechanisms of venetoclax plus ATRA regimen as a differentiation induction therapy in patients with variant APL carrying TNRC18::RARA . Venetoclax in combination with ATRA possesses advantages in terms of time to CR compared to venetoclax monotherapy and safety profiles compared to venetoclax‐based chemotherapy, especially suitable for older patients who are ineligible for intensive chemotherapy.…”

Section: Figurementioning

confidence: 98%

Venetoclax overcomes resistance to all‐trans retinoic acid in a variant acute promyelocytic leukemia with TNRC18::RARA fusion

Xu,

Li,

Wang

et al. 2023

Molecular Carcinogenesis

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Acute promyelocytic leukemia (APL) is generally driven by PML::RARA, but approximately 2% of variant APL patients do not contain this fusion gene and pose challenges in diagnosis and treatment. Here, we reported an aggressive APL patient with variant TNRC18::RARA fusion gene, who was resistant to standard differentiation induction therapy consisting of all‐trans retinoic acid (ATRA) and arsenic trioxide but achieved complete remission with venetoclax plus ATRA. Mechanistically, venetoclax possesses synergistic effects in ATRA‐induced TNRC18::RARA‐positive cell differentiation.

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Clinical Response to Venetoclax and Decitabine in Acute Promyelocytic Leukemia With a Novel RARA-THRAP3 Fusion: A Case Report

Cited by 16 publications

References 13 publications

Treatment of central nervous system relapse in PLZF::RARA-positive acute promyelocytic leukemia by venetoclax combined with arubicin and cytarabine: a case report

Treatment of central nervous system relapse in PLZF::RARA-positive acute promyelocytic leukemia by venetoclax combined with arubicin and cytarabine: a case report

Treatment of STAT5b-RARA positive acute promyelocytic leukemia by Venetoclax combining with homoharringtonine, cytarabine: A case report and literature review

Venetoclax overcomes resistance to all‐trans retinoic acid in a variant acute promyelocytic leukemia with TNRC18::RARA fusion

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