Successful treatment of a patient with acute promyelocytic leukemia with a<i>STAT5B</i>/<i>RARA</i>fusion gene using decitabine

Wang, Anyou; Cai, Xiaoyan; Qiang, Ping; Duan, Qiaohong

doi:10.1080/10428194.2017.1357176

Cited by 10 publications

(10 citation statements)

References 10 publications

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“…Disease remained incentive to ATRA. No APL differentiation was observed with ATRA or ATO, while an ATRA and decitabine combination showed some response [139] so that this disease remains a therapeutic challenge [138]. 4.1.12.…”

Section: Npm-rara T(5;17)(q35;q21)mentioning

confidence: 99%

Classic and Variants APLs, as Viewed from a Therapy Response

Geoffroy

2020

Cancers

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Most acute promyelocytic leukemia (APL) are caused by PML-RARA, a translocation-driven fusion oncoprotein discovered three decades ago. Over the years, several other types of rare X-RARA fusions have been described, while recently, oncogenic fusion proteins involving other retinoic acid receptors (RARB or RARG) have been associated to very rare cases of acute promyelocytic leukemia. PML-RARA driven pathogenesis and the molecular basis for therapy response have been the focus of many studies, which have now converged into an integrated physio-pathological model. The latter is well supported by clinical and molecular studies on patients, making APL one of the rare hematological disorder cured by targeted therapies. Here we review recent data on APL-like diseases not driven by the PML-RARA fusion and discuss these in view of current understanding of “classic” APL pathogenesis and therapy response.

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Section: Npm-rara T(5;17)(q35;q21)mentioning

confidence: 99%

Classic and Variants APLs, as Viewed from a Therapy Response

Geoffroy

2020

Cancers

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“…After 1 year of follow-up, the patient remains in CR. [16] In our case, ATRA and DA was given in the induction treatment, Case 4 got CR after taking the chemotherapy of CAG regimen.…”

Section: Literature Review and Discussionmentioning

confidence: 86%

“…On the 75th the STAT5b-RARα recurred and finally died of pneumorrhagia. In case 11, [16] after 2 unsuccessful courses with ATRA, ATO, mitoxantrone and IA, decitabine and AA (aclacimomycin and cytarabine)/IA combination therapy (decitabine 25 mg/d for 3 days in every course, decitabine +AA for 3 courses, and decitabine+ IA for 3 courses) was given. The patient achieved a CR after the first course of decitabine treatment and STAT5b-RARα fusion transcript changed to be negative at last.…”

Section: Literature Review and Discussionmentioning

confidence: 99%

Characterization of atypical acute promyelocytic leukaemia

Wang

Wang²,

Zhang³

2019

Medicine

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Rationale: The vast majority of acute promyelocytic leukemia (APL) is characterized with a specific chromosomal translocation t (15, 17) (q22, q21), which fuses PML-RARα leading to a good response to all-trans retinoic acid (ATRA) and arsenic trioxide (ATO). However, there are few cases of atypical APL, including PLZF-RARα, F1P1L1-RARα, STAT5b-RARα, et al. Neither PLZF-RARα nor STAT5b-RARα are sensitive to ATRA and ATO, and the prognosis is poor. Patient concerns: Here we have 3 cases (PLZF-RARα, n = 2; STAT5b-RARα, n = 1). Case A, A 53-year-old Chinese female had suffered ecchymosis in both legs for 3 days. Case B, A 44 years old male suffered pain from lower limbs and hip. Case C, 52-year-old male patient presented with fever for 3 weeks invalid to antibiotics and gingival bleeding for 1 week. Diagnoses: With RT-PCR and karyotype, Case A is diagnosed with STAT5b-RARα-positive APL. Case B, C are diagnosed with PLZF-RARα-positive APL. Interventions: In case A, ATO, and ATRA were used for induction treatment. In Case B, ATO, and chemotherapy with DA were given in the first induction treatment. In Case C, ATRA, and ATO were used immediately, subsequently, chemotherapy was added with DA, ATRA, and CAG combination treatment, and medium-dose cytarabine with daunorubicin were given regularly. Outcomes: In Case A, the patient refused the following treatment and discharged on day 25. In Case B, the patient got the disseminated intravascular coagulation (DIC).In Case C, the patient has survived for 7 months and remains CR. Lessons: Both STAT5b-RARα-positive APL and PLZF-RARα-positive APL appear to be resistant to both ATRA and ATO, so combined chemotherapy and allo-HSCT should be considered. Since the prognosis and long-term outcome are poor, more clinical trials, and researches should be taken.

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“…14 Further support of the role of hypomethylating agents is provided by a case of refractory APL with variant translocation STAT5B/RARA where decitabine was used in combination with alternating cycles of idarubicin/cytarabine and aclacinomycin/cytarabine inducing a complete and durable response. 15 Although this patient presented with a low WBC CT and low-risk APL clinical features, she harbored the FLT3-ITD mutation which some view as an added risk factor in acute myeloid leukemia. Her robust molecular response after decitabine single agent therapy is remarkable.…”

Section: Case Reportsmentioning

confidence: 81%

Serendipity: decitabine monotherapy induced complete molecular response in a 77-year-old patient with acute promyelocytic leukemia

et al. 2019

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Acute promyelocytic leukemia (APL) is an uncommon but very aggressive subtype of acute myeloid leukemia (AML), accounting for 10-15% of adult AML with 600-800 new cases per year in the United States. 1,2 Over 95% of cases involve translocation t(15;17)(q22;q21), which results in the promyelocytic leukemia retinoic acid receptor alpha (PML-RARa) rearrangement and fusion protein. 3 Most of the remaining APL cases harbor a fusion protein involving RARa and another partner. 4 The PML-RARa fusion gene initiates APL by blocking differentiation and increasing self-renewal and proliferation of leukemic progenitor cells.

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Successful treatment of a patient with acute promyelocytic leukemia with aSTAT5B/RARAfusion gene using decitabine

Cited by 10 publications

References 10 publications

Classic and Variants APLs, as Viewed from a Therapy Response

Classic and Variants APLs, as Viewed from a Therapy Response

Characterization of atypical acute promyelocytic leukaemia

Serendipity: decitabine monotherapy induced complete molecular response in a 77-year-old patient with acute promyelocytic leukemia

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