Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML-RARalpha isoforms: a study of the PETHEMA group (original) (raw)
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Eastern Mediterranean health journal = La revue de santé de la Méditerranée orientale = al-Majallah al-ṣiḥḥīyah li-sharq al-mutawassiṭ, 2010
This cases series describes the profile of adult patients with acute promyelocytic leukaemia (APt) at a referral hospital in Qatar. Of 34 acute myeloid leukaemia (AML) cases diagnosed, 11(32%) were classified as APt. Disseminated intravascular coagulation was common at presentation (91%). Severe thrombocytopenia was seen in 73%, leukocytosis in 55% and severe anaemia in 45%. Only 2 patients were of the classic hypergranular type. In the remaining 9 patients, 3 morphological subtypes were recognized: microgranular variant (6 patients), hyperbasophilic (2 patients) and regular nuclear outline M3r (1 patient). Translocation t(15;17) was detected in 63% of cases. APL constitutes a high proportion of AML cases in Qatar, with considerable morphological heterogeneity and a oredominance of APL variants with unfavourable oresenting features.
Iranian Journal of Pathology
Scan to discover online Background & Objective: Acute Promyelocytic Leukemia (APL) is a medical emergency with potentially fatal complications. APL primarily results from a chromosomal translocation (t(15;17)(q22;q21)), leading to the formation of the PML-RARA fusion gene with three possible isoforms. This study aims to investigate the characteristics of Iranian APL patients, the distribution of PML-RARA isoforms, and survival analysis. Methods: We included 145 consecutive eligible patients in this study. Data were collected through archived documents and phone inquiries, following consent. Subsequently, we analyzed the data using SPSS software version 26.0. Results: We examined 75 men and 70 women, with a mean age of 34 years (range: 2-78 years). Besides t(15;17) (q22;q21), 45.6% had other chromosomal abnormalities. The prevalence of bcr1 and bcr3 isoforms was 73% and 27%, respectively. bcr3 correlated with higher white blood cell (WBC) counts, additional chromosomal abnormalities, and faster Complete Hematologic Response (CHR). Early death occurred in approximately 36% of all patients. The mean overall survival time was 73.5 months, with 120-month survival rates of 53.8% for all patients and 83.9% for those who achieved CHR. Univariate analysis identified old age, relapse, lower platelet (PLT) counts, higher WBC counts, and leukocytosis as survival risk factors. However, in multivariate analysis, only old age and higher WBC counts were identified as adverse prognostic factors. Conclusion: In Iranian APL patients, bcr1 predominates, while bcr3 correlates with higher WBC counts, high-risk categorization, additional chromosomal abnormalities, and faster CHR. Survival is negatively impacted by old age, relapse, lower PLT counts, higher WBC counts, and leukocytosis.
Acute Promyelocytic Leukaemia: An Experience with 6 Cases
Journal of Medicine, 2011
Background and objective: Acute promyelocytic leukaemia (APL) is considered a distinct entity among the acute myeloid leukemias (AML). Our purpose is to see its clinical presentations and outcome with ATRA (all- trans retinoic acid). Materials and methods: It is a retrospective descriptive study of 6 patients diagnosed by cytologic criteria between November 2008 and February 2011 in two tertiary care hospitals in Dhaka, Bangladesh. All specific information were recorded in standardized data sheet and analyzed manually. Results: Mean (SD) age of patients was 28.6 (±7.84) years with equal gender distribution. Fever >101°F, weakness, easy fatigability, and bleeding manifestations were common presentations (100%). The other manifestations were bony tenderness, purpuric spots or DIC, but no lymphadenopathy and organomegaly were seen. Mean (±SD) Hb was 9.03 (±1.06) gm/dl, in 66% ESR was >50mm in 1st hr with 50% leukocytosis and 50% leucopenia but 100% thrombocytopenia.In the periphe...
Journal of Dhaka Medical College, 2010
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 acc...
Acute promyelocytic leukemia.Therapy results and prognostic factors
Cancer, 1988
34 patients with acute promyelocytic leukemia (APL) were treated with daunorubicin (DNR) alone and simultaneous supportive therapy with low-dose heparin, platelet transfusions, and fresh frozen plasma. Two consecutive maintenance therapy regimens were employed in patients who achieved complete remission (CR): (I) a classical maintenance with methotrexate and 6-mercaptopurine, with DNR plus methyl-GAG reinductions; (2) from 1982 an intensive sequential combination therapy regimen was administered. CR was achieved in 23 patients (68%). Only one patient had leukemic resistance. Other failures were a consequence of postchemotherapy complications. A multivariate logistic regression analysis has been performed to evaluate the prognostic importance on response to remission induction of 25 patient and disease characteristics at diagnosis. The significant variables in decreasing order of significance were: serum albumin level, fever at diagnosis, serum creatinine level, and age. The median duration of remission and survival by Kaplan-Meier analysis were projected to be 24 and 25 months, respectively. Relapses occurred in 11 of 23 CR patients. Nine patients remained in the first remission from 5+ to 37+ months. Short-term (CR) and long-term results (duration of remission and survival) in APL treated for induction with DNR alone were similar to those obtained in other subtypes of acute myeloblastic leukemia by intensive combination chemotherapy.
South African Journal of Oncology
Background: Targeted therapies combined with anthracycline chemotherapy have improved the survival of patients with acute promyelocytic leukaemia (APL). High short-term mortality has been demonstrated in low- and upper-middle-income countries, with limited local data.Aim: This study aimed to describe the demographic variables, clinical characteristics and laboratory features associated with the short-term mortality of patients with APL.Setting: The Division of Clinical Haematology, Universitas Academic Hospital (UAH), Bloemfontein, South Africa.Methods: Demographic and clinical data were obtained from the patients’ files and the MEDITECH electronic filing system. Laboratory data were retrieved from TrakCare, the National Health Laboratory Service (NHLS) electronic database. Data were analysed to report the demographic variables, clinical characteristics and laboratory features, and the short-term mortality of all newly diagnosed patients treated for APL during the 5-year period, 201...
Mediterranean Journal of Hematology and Infectious Diseases, 2013
Context: Acute promyelocytic leukemia (APL), an AML subtype, is characterized morphologically by abnormal promyelocytes. Molecular studies show three possible bcr isoforms of PML-RARα fusion gene. This study undertakes analysis of PML-RARα bcr isoforms and their correlation with haematological parameters and response to treatment in Indian patients. Aims: To study different PML-RARα bcr isoforms in Indian patients and to find any correlation with various haematological parameters and response to treatment Settings and Design: Patients diagnosed as APL on morphology or flowcytometry and confirmed by RQ PCR were included in the study. Treated APL patients or patients with relapse and on followup were excluded from the study. Methods and Material: Twenty patients over thirty one months period were included. The clinical, haematological & morphological features were analysed, the latter using routine & special cytochemical stains on blood and bone marrow. Flow cytometric evaluation using 4-color Beckman Coulter FC 500 and molecular studies using RT PCR Fusion Quant® kits for bcr-1, bcr-2 and bcr-3 of PML-RARα bcr isoforms on the instrument Rotor Gene™ 3000 were performed. Statistical analysis used: Student t test was applied to correlate different bcr isoforms with various haematological parameters and response to treatment. Results: In our study, M:F ratio was 1.5:1 with median age 42 years, Hb -8.0 g/dl, TLC-7900/μl, and platelet -35000/ μl and varied clinical presentation. Four patients were microgranular variants, and the rest were hypergranular. MPO and CAE positivity were100% and for NSE it was
Haematologica, 2006
Acute promyelocytic leukemia (APL) is characterized by leukemic cells blocked at the promyelocytic stage of granulocytic differentiation. To date, it is still not clear whether CD34 expression identifies a subset of APL patients with peculiar characteristics. We, therefore, conducted a detailed analysis of CD34 expression at diagnosis in 136 adults with de novo APL. We investigated 136 newly diagnosed APL patients from four Italian Institutions. All 136 cases were tested for CD34 and CD2 expression: 124 (91%) cases were classified as hypergranular (M3) and 12 (9%) as the hyporgranular M3 variant (M3v). The parameters considered were white blood cell (WBC) and platelet counts, hemoglobin levels, percentage of peripheral blood leukemic promyelocytes (PBLP), CD15, CD56 and HLA-DR expression, and the PML/RARalpha isoform, to assess their relationship with CD34 and CD2 expression. CD34 expression was associated with the M3v subtype and higher proportion of HLA-DR+ and CD2+ cases. Moreove...