TBLR1 Fuses To Retinoid Acid Receptor Alpha In a Variant t(3;17)(q26;q21) Translocation Of Acute Promyelocytic Leukemia
Yirui ChenShouyun LiChunlin ZhouJiawei ZhaoHaiyan XingZheng TianKejing TangYingchang MiQing RaoMin WangJianxiang Wang
2
Citation
0
Reference
10
Related Paper
Citation Trend
hemoglobin level were not significant predictors of ACS events.The rate of pain was not increased in those with asthma diagnosis (0.72 events per patient year for those with asthma and 0.60 events per patient year for those without asthma, p value=0.53).Furthermore, baseline white blood cell count, percent fetal hemoglobin and baseline hemoglobin were not significant predictors of pain episodes (Table 2).When compared to the remaining group of children without asthma (n=280), children taking daily inhaled corticosteroids (n=17) had an increased rate of ACS (0.43 events per patient-year vs. 0.16 events per patient-year; p-value=0.002)and no significant increase in rate of pain (0.99 events per patientyear vs. 0.59 events per patient-year; p=0.122) .Evidence from previous studies suggested that asthma is associated with increased rates of ACS events 2,3,4 and pain episodes. 2 The results of this study confirmed previous data indicating an association between asthma and ACS events, but did not reveal an association between asthma and pain events.The reasons for the lack of association between asthma and pain in the Créteil population are not known, but may be related to differences in how care is delivered in that center and/or unmeasured environmental factors.Two studies have assessed the impact of the environment on SCD-related pain episodes. 5,6 Both studies demonstrated that local environmental factors may contribute to the rate of hospitalization for pain. 5,6 The background rate of asthma among the children with SCA in France, 8.4%, was not significantly different from a comparable cohort of children of African descent living in France, 9.1%. 7 Taken together, these studies provide no evidence that a physician diagnosis of asthma occurs more frequently among children with sickle cell disease when compared to children without sickle cell anemia.In summary, we provide further support that asthma is a potentially treatable risk factor associated with ACS.Future prospective studies to classify lung disease associated with SCA and to determine the effectiveness of asthma management in preventing SCA-related morbidity are warranted.
Idarubicin
Tretinoin
Cite
Citations (20)
Objective To investigate the significance of detection of PML-RARα fusion gene in patients with acute promyelocytic leukemia(APL) before and after treatment and to study the relationship between PML-RAR fusion gene levels in patients and acute promyelocytic leukemia(APL) lesion process. Methods PML-RARα fusion gene levels of APL in bone marrow before and after treatment were detected by RT-PCR. Results There were 41 positive cases of detection of PML-RARα fusion gene among 51 patients with APL. The positive rate was 80.4%. 51 patients with acute promyelocytic leukemia(APL) were treated for 18 months(followed up), 3 in death and 3 positive. The positive rate was 5.9%. Conclusion Detection of PML-RARα fusion gene is important for APL diagnosis. Molecular relapse can be detected early by PML- RARα fusion gene regular detection. And acute promyelocytic leukemia(APL) can be treated in time to avoid hematological relapse.
Promyelocytic leukemia protein
Cite
Citations (0)
All-trans-retinoic acid is an effective induction treatment for acute promyelocytic leukemia. Although retinoic acid is well tolerated by the majority of patients with this disease, a potentially fatal complication of this kind of treatment has been reported: "the retinoic acid syndrome". We describe this syndrome, which occurred in one of our patients and was successfully treated by the administration of dexamethasone.
Tretinoin
Cite
Citations (3)
Objective To investigate the relapse risk assessment for patients with acute promyelocytic leukemia(APL)through testing PML-RARα fusion gene by real-time quantitative polymerase chain reaction(RQ-PCR).Methods Relative copies of PML-RARα fusion gene were measured in 25 patients with APL in phases of first diagnosis,complete remission(CR)and relapse.The minimal residual disease(MRD)situations were also monitored in 6 of the 25 patients.Results Different PML-RARα fusion gene expression levels were observed in patient groups of different phases of the disease.(P0.05).The expression level was high in patients at first diagnosis,and low during CR and high again when relapsed.Conclusion PML-RARα fusion gene test by RQ-PCR could be used for monitoring MRD in patients with APL.A higher expression level in the CR phase may be suggestive of a relapse.
Minimal Residual Disease
Cite
Citations (0)
Objective To illustrate the clinical relevance of distinct PML-RARα fusion gene isoforms in acute promyelocytic leukemia (APL). Methods The nested reverse transcriptase polymerase chain reaction (RT-PCR) was used to detect the long (L) or short (S) PML-RARα fusion gene isoforms in 92 newly diagnosed APL so as to evaluate the clinical feature, therapeutic reaction and prognosis of the two fusion gene isoforms. Results PML-RARα fusion gene was positive in all 92 APL patients, of which 52(56.5 %) was L type and 40 (43.5 %) was S type. There were no significant differences between L type and S type in the aspect of sex, age, white blood cell count,the percentage of bone marrow blasts plus promyeloeytes and chromosome before treatment. And there were no significant differences between the two isoforms in complete remission (CR) rate, the time of getting CR as well as the occurrence of retinoic acid syndrome (RAS), disseminated intravascular coagulation (DIC), intraeranial hemorrhage. Also, there were no significant differences in overall survival rate (OS) and relapse-free survival rate (RFS) between the two isoforms. Conclusion PML-RARα fusion gene isoforms in APL were not correlated with clinical therapeutic effect or prognosis.
Key words:
Leukemia, promyelocytic, acute; PML-RARα fusion gene; Receptors, retinoic acid
Promyelocytic leukemia protein
Fusion transcript
Cite
Citations (0)
To establish and evaluate a real time quantitative PCR (RQ-PCR) method for detection and quantification the PML/RAR alpha fusion gene transcripts in patients with acute promyelocytic leukemia (APL).Three pairs of primers and TaqMan probe were designed for detecting the most frequent PML/RAR alpha transcripts (L-form, S-form and V-form) and normal ABL was used as an internal control. A real time PCR condition was established to detect PML/RAR alpha and ABL positive templates with a series of dilutions. To evaluate this assay, bone marrow samples from 6 APL patients were detected.In repeated tests, maximal sensitivities of 10 copies/microL were obtained, while reproducible maximal sensitivity achieved 100 copies/microL. In 10 normal controls, no amplified fluorescent signals were detected. The median absolute and normalized amount of PML/RAR alpha fusion gene transcripts were 4.27 x 10(3)-3.36 x 10(5) copies/50 ng (median 4.33 x 10(4)copies/50 ng) and 29.38%-600.53% (median 48.12%) respectively. One case showed significant decrease of PML/RAR alpha fusion gene transcripts after induction therapy compared to that at the time of diagnosis, while the fusion transcripts significantly increased after relapsed.RQ-PCR is a sensitive, reliable quantitative assay and can be used in the diagnosis of APL and measurement of MRD.
TaqMan
Promyelocytic leukemia protein
Cite
Citations (0)
Promyelocytic leukemia protein
Tretinoin
Cite
Citations (2)
Objective To investigate the application of fluorescence in situ hybridization(FISH) for the detection of PML-RARa fusion gene in the diagnosis and treatment of acute promyelocytic leukemia(APL).Methods PML-RARa fusion gene detection was performed in 21 patients suspected of APL by FISH.Results Of the 21 suspected APL patients,fifteen cases were confirmed of APL.Positive PML-RARa fusion genes were found in 14 of the 15 APL patients,while one case with negative PML-RARa fusion gene was confirmed to be variant APL and was treated with all-trans retinoic acid(ATRA)-induction therapy but the efficacy was poor.The remaining 6 suspected APL patients with negative PML-RARa fusion gene were confirmed to be acute myeloid leukemia-M2.Conclusion Detection of PML-RARa fusion gene by FISH is sensitive and reliable,and is of great value for the diagnosis and treatment of APL.
Promyelocytic leukemia protein
Cite
Citations (0)
The addition of retinoic acid to human promyelocytic leukemia cells in culture results in their differentiation to mature myeloid forms with acquisition of the differentiated phenotype, i.e., the ability to reduce nitroblue tetrazolium. A heavily pretreated patient with acute promyelocytic leukemia and residual malignant cells in his marrow after multiple courses of chemotherapy was given 13-cis-retinoic acid upon demonstration of both morphologic and functional differentiation of his leukemic cells by transretinoic acid in vitro. The patient achieved a complete remission and was maintained on 13-cis-retinoic acid for 1 year, when the patient relapsed with a population of cells that were resistant to retinoic acid-induced differentiation.
Tretinoin
Cite
Citations (84)