scholarly journals EPIDEMIOLOGY, DIAGNOSIS AND TREATMENT OF ACUTE PROMYELOCYTIC LEUKEMIA IN CHILDREN: THE EXPERIENCE IN CHINA

2012 ◽  
Vol 4 (1) ◽  
pp. e2012012 ◽  
Author(s):  
Li Zhang ◽  
Xiaofan Zhu
Keyword(s):  
Developing Countries ◽  
Treatment Failure ◽  
Acute Promyelocytic Leukemia ◽  
Promyelocytic Leukemia ◽  
Early Mortality ◽  
Term Survival ◽  
The Status ◽  
Treatment Abandonment ◽  
Leukemia In Children

The limited available data suggest that the rate of early mortality is high and that long-term survival is poor in many developing countries. Death from bleeding and infection during chemotherapy, relapse and treatment abandonment are among the main cause of treatment failure in APL children. The status of children APL treatment in China is not described in general.

Two Cycles of Risk-Adapted Consolidation Leads to a Favorable Long-Term Prognosis in Patients with Acute Promyelocytic Leukemia After Standard AIDA Induction: Results From 141 Patients Treated in the SAL-AIDA-2000 Trial

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 765-765 ◽  
Author(s):  
Uwe Platzbecker ◽  
Ulrich Schuler ◽  
Martin Bornhäuser ◽  
Michael Kramer ◽  
Markus Schaich ◽  
...  
Keyword(s):  
High Risk ◽  
Acute Promyelocytic Leukemia ◽  
Promyelocytic Leukemia ◽  
Induction Treatment ◽  
Term Survival ◽  
Consolidation Treatment ◽  
High Risk Patients ◽  
Risk Patients ◽  
Disease Free

Abstract Abstract 765 Background: The combination of ATRA and idarubicin (AIDA) for induction therapy of acute promyelocytic leukemia (APL) yields complete remission (CR) rates of > 90%. If this is followed by intensive consolidation treatment, about 85% of patients are disease free and alive after 6 years. In fact, three cycles of consolidation treatment are considered the therapeutic standard; however, it is unclear how much treatment intensity is necessary for long-term survival. In order to address this question, we analyzed the clinical course of patients enrolled into the APL study of the German Study Alliance Leukemia (SAL), which contained only two courses of consolidation. Patients and Methods: All patients ≥ 18 years diagnosed with cytogenetically confirmed APL were eligible for inclusion in the risk-adapted SAL-AIDA-2000 trial. Enrolled patients received standard induction treatment with ATRA (45 mg/m2/d until CR) and idarubicin (12 mg/m2 for 4 doses every other day). After CR, non-high-risk patients (WBC ≤ 10,000/μL) received daunorubicin (45/60 mg/m2 days 1–3, dose depending on age) as first consolidation and mitoxantrone (10 mg/m2 days 2–4) as second consolidation. High-risk patients received additional cytarabine in both consolidation cycles (100/200 mg/m2 continuous infusion over 7 days in 1st consolidation and 1000/3000 mg/m2 twice daily on 4 days in 2nd consolidation, dose depending on age). After 2 cycles of consolidation, all patients were scheduled for 24 months of maintenance with 6-mercaptopurin (90 mg/m2 daily), methotrexate (15 mg/m2 weekly), and ATRA (45 mg/m2 for 15 days every 3 months). The following outcomes were analyzed: CR rate, induction deaths, disease-free survival (DFS), and overall survival (OS). Results: Between January 1999 and October 2010, 141 patients were enrolled in the trial. The median age at diagnosis was 51 years (range, 19–82), and 41 (29%) patients had a WBC >10,000/μL (high risk). The CR rate was 92% in the entire cohort; 95% in patients ≤ 60 and 86% in patients > 60 years (p=0.082). No significant differences in CR rates were seen between high-risk and non-high-risk patients (88% vs 94%, p=0.213). Three patients died during induction treatment (2%). After a median follow up of 55 months, the median DFS and OS were not reached. The estimated 6-year DFS was 80% (95%–CI 72%–88%) in all patients; 84% in patients ≤ 60 and 72% in patients > 60 years (p=0.140). The estimated 6-year OS was 77% in all patients; 84% (95%–CI 76%–92%) in the younger and 62% (95%–CI 47%–78%) in the elderly group (p=0.004). No significant survival differences between the high-risk and the non-high-risk patients were observed, neither for DFS (6-year DFS 78% (95%–CI 64–93%) vs 81% (95%–CI 72%–91%), p=0.625) nor for OS (6-year OS 71% (95%–CI 57%–86%) vs 79% (95%–CI 70–89), p=0.207). Conclusions: Our results confirm the efficacy of a risk-adapted approach both in high-risk and non-high-risk APL patients. The similarity for DFS and OS times between these two groups demonstrate the efficacy of cytarabine added to anthracyclines during consolidation in high-risk patients. Both CR rates and survival outcomes are comparable to the results obtained in the AIDA0493 and AIDA2000 trials by the GIMEMA group, which used three cycles of higher-dosed consolidation. In light of the data, modification in number and intensity of consolidation cycles may result in a less toxic but equally effective option for the treatment of APL and should be considered for further evaluation in a randomized clinical trial. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Coagulopathy in Acute Promyelocytic Leukemia: Can We Go Beyond Supportive Care?

2021 ◽  
Vol 8 ◽  
Author(s):  
Bryan C. Hambley ◽  
Ciprian Tomuleasa ◽  
Gabriel Ghiaur
Keyword(s):  
Supportive Care ◽  
Acute Promyelocytic Leukemia ◽  
Causes Of Death ◽  
Promyelocytic Leukemia ◽  
Therapeutic Interventions ◽  
Term Survival ◽  
Common Causes ◽  
Thrombotic Complications ◽  
Risk Of Hemorrhage

Acute promyelocytic leukemia (APL) is characterized by frequent complications due to a distinct coagulopathy. While advances in treatments have improved long-term survival, hemorrhagic and thrombotic complications remain the most common causes of death and morbidity. Improved understanding of the mechanisms of the coagulopathy associated with APL may lead to therapeutic interventions to mitigate the risk of hemorrhage and thrombosis.

scholarly journals Acute Promyelocytic Leukemia in Children: A Model of Precision Medicine and Chemotherapy-Free Therapy

2021 ◽  
Vol 22 (2) ◽  
pp. 642
Author(s):  
Carmelo Gurnari ◽  
Maria Teresa Voso ◽  
Katia Girardi ◽  
Angela Mastronuzzi ◽  
Luisa Strocchio
Keyword(s):  
Precision Medicine ◽  
Acute Promyelocytic Leukemia ◽  
Survival Rates ◽  
Early Death ◽  
Pseudotumor Cerebri ◽  
Promyelocytic Leukemia ◽  
All Trans Retinoic Acid ◽  
Free Treatment ◽  
Leukemia In Children

Acute promyelocytic leukemia (APL) represents a paradigm of precision medicine. Indeed, in the last decades, the introduction of all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) completely revolutionized the therapeutic approach to this previously highly fatal disorder. This entirely chemotherapy-free treatment, which provided excellent survival rates, has been initially validated in adults and, recently, translated in the pediatric setting. This review summarizes currently available data on the use of ATRA and ATO combination in pediatric APL, providing a particular focus on peculiar issues and challenges, such as the occurrence of pseudotumor cerebri and death during induction (early death), as well as the advantage offered by the ATO/ATRA combination in sparing long-term sequelae.

Improving acute promyelocytic leukemia (APL) outcome in developing countries through networking, results of the International Consortium on APL

Blood ◽  
2013 ◽  
Vol 121 (11) ◽  
pp. 1935-1943 ◽  
Author(s):  
Eduardo M. Rego ◽  
Haesook T. Kim ◽  
Guillermo J. Ruiz-Argüelles ◽  
Maria Soledad Undurraga ◽  
Maria del Rosario Uriarte ◽  
...  
Keyword(s):  
Developing Countries ◽  
Acute Promyelocytic Leukemia ◽  
Promyelocytic Leukemia ◽  
Early Mortality ◽  
International Consortium ◽  
Clinical Network ◽  
Key Points

Key Points For patients in developing countries with APL, a clinical network of institutions made it possible to reduce significantly the early mortality and improve the OS.

scholarly journals Long-term survival in a patient with acute promyelocytic leukemia with isolated meningeal relapse

2010 ◽  
Vol 45 (3) ◽  
pp. 208 ◽  
Author(s):  
Se Hyung Kim ◽  
Jina Yun ◽  
Hyun Jung Kim ◽  
Chan Kyu Kim ◽  
Sung Kyu Park ◽  
...  
Keyword(s):  
Acute Promyelocytic Leukemia ◽  
Promyelocytic Leukemia ◽  
Term Survival ◽  
Long Term Survival

scholarly journals Status of Early Mortality in Newly Diagnosed Cases of Acute Promyelocytic Leukaemia (APL) in BSMMU Hospital

1970 ◽  
Vol 18 (2) ◽  
pp. 150-154
Author(s):  
F Rahman ◽  
ABM Yunus ◽  
AL Kabir ◽  
M Begum ◽  
A Aziz ◽  
...  
Keyword(s):  
Acute Promyelocytic Leukemia ◽  
Acute Promyelocytic Leukaemia ◽  
Early Death ◽  
Promyelocytic Leukemia ◽  
Early Mortality ◽  
Promyelocytic Leukaemia ◽  
Laboratory Evaluation ◽  
Total Study Population ◽  
Term Survival ◽  
Study Population

Background: Acute promyelocytic leukemia (APL), is now curable in most cases with current treatment strategies. But limited available data suggest that the rate of early mortality is high and long term survival is poor in many developing countries. To improve the survival rate by reducing early mortality, study of the status of early mortality as the main cause of treatment failure for the management of APL as a heamatological emergency appears to have a great significance. Materials and method: Diagnosis of acute promyelocytic leukemia was done from bone marrow morphological study by experienced haematologist. After that cytogenetic study to see chromosomal translocation 15 and 17 and molecular study to see PML-RARá fusion protein was also done. Clinical and laboratory evaluation was done to all of them. Chemotherapy according to IC-APL protocol was given to 30 out of 40 patients. Incidence of early mortality (death within 14 days of diagnosis) was recorded and also categorized according to cause of death. Pattern of supportive cares given to the patients such as management of coagulopathy. ATRA syndrome or sepsis to treat the disease as a medical emergency was also recorded. Result: Selected clinical, laboratory and outcome data of 40 cases of APL who were admitted in Haematology department of BSMMU was surveyed. The median age overall was 32 yrs. The median WBC count at diagnosis was 7.75×109/L and median platelet count 25×109/L. Laboratory evidence of DIC was noted in 37.5% of patients. Death occurred within 14 days of diagnosis (early death) in 10 (25%) cases. Bleeding was the most common cause of early death, then infectious complication and ATRA syndrome. Among 30 patients who got chemotherapy complete remission (CR) was 66.6%. So only 50% of the total study population was able to achieve CR. So due to early mortality, a significant portion of the study population could not receive protocol based treatment. Conclusion: To improve outcome by reducing initial mortality early recognition and treatment of APL is required in specialized institutions, by prompt and aggressive supportive therapy as well as immediate starting of antileukemic agent such as ATRA, arsenic trioxide etc.  Key words: Acute Promyelocytic Leukaemia (APL); mortality. DOI: 10.3329/jdmc.v18i2.6277 J Dhaka Med Coll. 2009; 18(2) : 150-154

Retrospective Study on 46 Chinese Children with Acute Promyelocytic Leukemia: A Single Center Experience in China.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4567-4567
Author(s):  
Yongmin Tang ◽  
Xiaojun Xu ◽  
Chan Liao ◽  
Limin Zou ◽  
Hua Song ◽  
...  
Keyword(s):  
Overall Survival ◽  
Acute Promyelocytic Leukemia ◽  
Fusion Gene ◽  
Promyelocytic Leukemia ◽  
Induction Treatment ◽  
Event Free Survival ◽  
Term Survival ◽  
Free Survival ◽  
Wbc Count

Abstract Acute promyelocytic leukemia (APL) is a specific type of hematopoietic malignancy, accounting for ~ 10% of the de novo acute myeloid leukemia (AML). During the old days, severe complications as disseminated intravascular coagulation (DIC) and intracranial hemorrhage were the most common causes of treatment failure after conventional chemotherapy without all-trans retinoic acid (RA). Owing to the application of RA for the induction treatment, the overall survival (OS), the disease free survival (DFS) and the event free survival (EFS) rates have been dramatically improved in adult patients with APL. However, data on long-term outcome of APL in children, especially in Chinese children, have been very limited. Objective The aim of this study was to investigate the clinical biological features, diagnosis, prognosis and long-term survival in childhood APL. Methods 46 children (26 boys and 20 girls, aged 1.5 ~13.8 yrs with a median of 9.3 yrs) with APL from April 1998 to October 2005 were enrolled into this study. Immunophenotyping analysis was carried out in 43 patients using multi-parameter flow cytometry. 32 patients went through PML/RARα fusion gene detection using RT-PCR. Induction treatment consisted of ATRA and daunorubicin(DNR) or pirarubicin (THP) followed by 6 courses of multi-drug chemotherapy consolidation and a long-term maintenance therapy including ATRA, high dose Ara-C (HD-Ara-C), DNR+Ara-C (DA), homoharringtonine+Ara-C (HA) and etoposide+Ara-C (EA). Results Pale, hemorrhage and fever were the most common symptoms in APL patients at the time of presentation. 19 patients (41.3%) were found to have WBC count more than 10.0×109/L at diagnosis. Immunophenotyping analysis showed that CD13, CD33, CD117 and MPO were the most commonly expressed antigens while HLA-DR, CD14 and CD34 were mostly the negative markers on APL cells. 71.9%(23/32) of the patients analyzed were PML/RARα fusion gene positive. Of the 39 patients receiving treatment, 36 children (92.3%) achieved complete remission. 7 children replased during therapy, and 3 relapsed after finishing the entire courses. The 5-year cumulative incidence of relapse (CIR) was 28.6%. The estimated overall survival (OS) rates at one, three and five years were 86.1%, 76.1%and 70.2%, respectively while the event free survival (EFS) rates were 78.4%, 63.6%and 53.1%, respectively. The probability of remaining alive after 5 years for patients with WBC≤10.0×109/L group was 81.4%, significantly higher than those with WBC>10.0×109/L group (51.6%, P=0.026). 5 children with positive PML/RARα S (short) subtype died eventually although all of them achieved CR, which was significantly different from the group with the L (long) subtype (13/13, P=0.001). Conclusion Induction with ATRA + DNR or THP is an effective and safe therapy for newly diagnosed childhood APL with very high long-term survival rates after 2.5 years of alternative multi-drug chemotherapy and maintenance. High WBC count and S subtype of PML-RARα are the two poor prognostic factors for children with APL.

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