Hematological and molecular remission with combination chemotherapy in a patient with PLZF-RARα acute promyelocytic leukemia (APML)

George, Biju; Poonkuzhali, B.; Srivastava, Vivi M.; Chandy, Mammen; Srivastava, Alok

doi:10.1007/s00277-004-0979-z

Cited by 6 publications

(3 citation statements)

References 8 publications

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“…Case i undertook ATRA, DA(daunorubicin for 3 days and cytarabine for 7 days) and 3 courses of CAG, and achieved CR at last. In case f [22] the patient got CR with DA and medium-dose of cytarabine. In case r [23] the patient was 83 years old, with the therapy of ATRA and daunorubicin for 3 courses, complete histological response (CHR) was achieved and survived for more than 24 months.…”

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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Section: Literature Review and Discussionmentioning

confidence: 99%

Characterization of atypical acute promyelocytic leukaemia

Wang

Wang²,

Zhang³

2019

Medicine

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“…The efficacy of combination therapy with anthracyclines and cytarabine along with ATRA in inducing remission has been previously reported. 22 Rohr et al describe two cases in which the patients did not achieve remission following induction with ATRA and anthracycline combination but achieved partial remission after reinduction in one of the cases. 23 Even though our case (case 1) was not in remission post induction, no ZBTB16 - RARA transcripts were detected by RT-qPCR post consolidation with high dose cytarabine.…”

Section: Discussionmentioning

confidence: 99%

Molecular Heterogeneity in Acute Promyelocytic Leukemia - A Single Centre Experience From India

Rabade

Raval

Subramanian

et al. 2018

Mediterr J Hematol Infect Dis

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Atypical breakpoints and variant APL cases involving alternative chromosomal aberrations are seen in a small subset of acute promyelocytic leukemia (APL) patients. Over seven different partner genes for RARA have been described. Although rare, these variants prove to be a diagnostic challenge and require a combination of advanced cytogenetic and molecular techniques for accurate characterization. Heterogeneity occurs not only at the molecular level but also at clinico-pathological level influencing treatment response and outcome. In this case series, we describe the molecular heterogeneity of APL with a focus on seven variant APL cases from a single tertiary cancer center in India over a period of two and a half years.We discuss five cases with ZBTB16-RARA fusion and two novel PML-RARA variants, including a Bcr3 variant involving fusion of PML exon4 and RARA exon3 with an additional 40 nucleotides originating from RARA intron2, another involving exon 6 of PML and exon 3 of RARA with addition of 126 nucleotides, which mapped to the central portion of RARA intron 2. To the best of our knowledge, this is the first case series of this kind from India.

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“…However, a number of studies suggest that this subset of APL is not completely resistant to differentiation approaches and may be ATRA responsive since some differentiation response to ATRA was observed, and complete differentiation was obtained when cytokines or cytostatics were used in addition to RA [10]. Despite the peculiar clinical and laboratorial presentation, there was a suspicion of promyelocytic leukemia, and patients have been treated following standardized protocols of the IC-APL, but none of them achieved remission.…”

Section: Discussionmentioning

confidence: 99%

Acute promyelocytic leukemia associated with the PLZF-RARA fusion gene: two additional cases with clinical and laboratorial peculiar presentations

et al. 2011

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Acute promyelocytic leukemia (APL) is characterized by the presence of the t(15;17) and PML-RARa rearrangement, with good response to treatment with retinoids. However, few cases of variant APL involving alternative chromosomal aberrations have been reported, including t(11;17)(q23;q21) (Wells et al. in Nat Genet 17:109-113, 1; Arnould et al. in Hum Mol Genet 8:1741-1749, 2) t(5;17)(q35;q12-21), t(11;17)(q13;q21) (Grimwade et al in Blood 96:1297-1308, 3) and der(17) (Rego et al. in Blood (ASH Annual Meeting Abstracts)114:Abstract 6, 4), whereby RARa is fused to the PLZF, NPM, NuMA, and STAT5b genes, respectively, have been described. These cases are characterized by distinct morphology, clinical presentation, and in respect to PLZF, a lack of differentiation response to retinoids leading to the need of different approaches concerning diagnostic methods and therapeutics. This paper describes two cases of APL associated with the PLZF-RARA fusion gene enrolled in the IC-APL trial that is a non-randomized, multicenter study conducted in Brazil, Mexico, Chile and Uruguay with the aim to improve the treatment outcome of APL patients in developing countries. These cases, although rare, offer a challenge to its early recognition and proper conduction.

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Hematological and molecular remission with combination chemotherapy in a patient with PLZF-RARα acute promyelocytic leukemia (APML)

Cited by 6 publications

References 8 publications

Characterization of atypical acute promyelocytic leukaemia

Characterization of atypical acute promyelocytic leukaemia

Molecular Heterogeneity in Acute Promyelocytic Leukemia - A Single Centre Experience From India

Acute promyelocytic leukemia associated with the PLZF-RARA fusion gene: two additional cases with clinical and laboratorial peculiar presentations

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