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Genetic profile of primary myelofibrosis patients in Azerbaijan 阿塞拜疆原发性骨髓纤维化患者的遗传谱
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103925
Elmir Guluyev , Madad Abbasov , Gulnar Garayeva , Azer Kerimov
{"title":"Genetic profile of primary myelofibrosis patients in Azerbaijan","authors":"Elmir Guluyev , Madad Abbasov , Gulnar Garayeva , Azer Kerimov","doi":"10.1016/j.htct.2025.103925","DOIUrl":"10.1016/j.htct.2025.103925","url":null,"abstract":"<div><h3>Objective</h3><div>Primary myelofibrosis is a clonal myeloproliferative neoplasm characterized by atypical myeloid proliferation and significant symptom burden. Activation of the Jak-STAT signaling pathway plays a central role in the pathogenesis of this disease. Approximately 90% of patients have one of three genetic mutations: Jak2V17F, CALR and MPL. The Jak2V617F mutation is the most common mutation and has been found in 60%‒65% of patients. Last year in SOHO 2024 annual meeting we first demonstrated genetic mutations of primary myelofibrosis patients in Azerbaijan. However, in our study only a small number of patients underwent genetic testing. Here we have updated the data of our cohort. The main goal of our study was to know the genetic profile of primary myelofibrosis patients in Azerbaijan.</div></div><div><h3>Methodology</h3><div>We retrospectively analyzed 123 patients with primary myelofibrosis who underwent genetic testing. We created 2 groups according to JAK2 levels. Group comparability was assessed by comparing baseline demographics and follow-up time between groups. Normality and heteroscedasticity of continuous data were assessed using the Shapiro-Wilk and Levene tests, respectively. Continuous outcomes were compared using unpaired Student <em>t</em>-test, Welch <em>t</em>-test or Mann-Whitney <em>U</em> test, depending on the data distribution. Discrete outcomes were compared using Chi-Squared or Fisher's exact test, respectively. The alpha risk was set at 5% and two-tailed tests were used.</div></div><div><h3>Results</h3><div>A total of 123 patients underwent genetic testing. Jak2V617F was positive in 91 (74%), CALR was positive in 3 (2.4%), MPL was positive in 1 (0.8%) patient. 32 (26%) patients were Jak2 negative. The median allele burden was 68.21% (IQR = 46.16). Median age was 58.5-years, 58 (47.2%) patients were male. We separated patients to groups according to Jak2 mutations and compared their clinical laboratory characteristics (Table 1). There was no difference between two groups according to IPSS: Low – 27 (31.03%), INT1 – 42 (48.28%), INT2 – 17 (19.54%), High ‒ 1 (1.15%) in Jak2 positive (n = 87) vs. Negative (n = 31) Low ‒ 11 (35.48%), INT1 ‒ 12 (38.71%), INT2 ‒ 7 (22.58%), High – 1 (3.23%). Jak2V617F positivity was significantly associated with higher Hgb (p = -0.022), WBC (0.029), higher ANC (p = -0.008), higher eosinophil count (p = -0.03) and lower bone marrow blast count (p = -0.022). Jak2V617F positivity was also associated with lower LDH, lower TSS and higher PLT count, but this was not statistically significant. The splenomegaly rate didn't differ between groups (Jak2 positive ‒ 94.44% and Jak2 negative ‒ 84.62%; p = 0.203). Median follow-up was 34.61-months. Although statistically insignificant, Jak2V617F negative patients seems to have better OS than Jak2V617F positive patients (p = -0.644). Median OS didn't reach in Jak2 negative group vs. 155-months in Jak2 group (Fig. 1).</div></div><div><h3>Co","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103925"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
METHOTREXATE-ASSOCIATED STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS: TWO CASE REPORTS 甲氨蝶呤相关的史蒂文斯-约翰逊综合征和中毒性表皮坏死松解:2例报告
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103894
Yakup Unsal , Muhammed Murati , Guler Delibalta , Serdar Bedii Omay , Vildan Yazici , Nedim Polat
{"title":"METHOTREXATE-ASSOCIATED STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS: TWO CASE REPORTS","authors":"Yakup Unsal , Muhammed Murati , Guler Delibalta , Serdar Bedii Omay , Vildan Yazici , Nedim Polat","doi":"10.1016/j.htct.2025.103894","DOIUrl":"10.1016/j.htct.2025.103894","url":null,"abstract":"<div><h3>Objective</h3><div>Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare but severe mucocutaneous diseases. These conditions are mostly drug-induced and have high mortality rates. They are primarily characterized by skin and mucosal involvement. In addition to supportive treatments, plasmapheresis and immunosuppressive drugs are used in treatment. We present our experience with 2 cases of SJS/TEN that developed after high-dose Methotrexate use in the treatment of two different hematological malignancies.</div></div><div><h3>Case report 1</h3><div>A 58-year-old male was diagnosed with Primary Central Nervous System (CNS) Lymphoma in August 2024. The patient started on MATRIX (Methotrexate 3000 mg/m²/day, Cytarabine 1000 mg/m²/day, Rituximab 375 mg/m²/day) therapy. On September 2, 2024, the first cycle of treatment was administered. On the 8<sup>th</sup> day, erythematous rashes appeared on the palms and soles. The patient was consulted with dermatology and received local treatment for a suspected drug reaction. The lesions resolved. During the second cycle of MATRIX therapy on September 30, 2024, the patient received four doses of intrathecal Methotrexate 12 mg and Cytarabine 100 mg by October 3, 2024. On the fourth day of treatment, the creatinine level rose to 3.05 mg/dL (baseline 0.9 mg/dL). Suspecting toxic nephropathy, hydration therapy was initiated under nephrology consultation. On the 10<sup>th</sup> day of treatment, the patient developed a fever above 38°C, accompanied by erythematous lesions on the skin, particularly in the oral mucosa. Following the recommendation of the Infectious Diseases Department, treatment with Cefoperazone/Sulbactam and Micafungin was initiated. During the same period, the patient developed diarrhea (6‒8 times per day) and was given symptomatic treatment. On the 13<sup>th</sup> day of treatment, as skin rashes increased, Prednisolone (1 mg/kg) was initiated. However, the skin lesions continued to progress. On the 15<sup>th</sup> day of treatment, with a creatinine level of 1.8 mg/dL, the patient developed absolute neutropenia. Filgrastim (G-CSF) therapy was started. The patient was consulted with the Dermatology Department due to the skin lesions. Pale erythema on the skin and erythematous patches with targetoid vesicles on the extremities were observed. The body surface area involvement was estimated to be approximately 10%‒30%. A skin biopsy was performed, and the findings were reported as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN). Based on the current findings, the patient was diagnosed with SJS/TEN Overlap Syndrome, and pulse Prednisolone 500 mg was initiated for 3 days. Due to elevated acute phase reactants and absolute neutropenia, the Infectious Diseases Department recommended discontinuing Cefoperazone & Sulbactam treatment. The patient was then started on Meropenem and Daptomycin. On the 23<sup>rd</sup> day of treatment, as the skin lesions continu","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103894"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
REAL WORLD OUTCOMES OF HYPOMETHYLATING AGENTS AND VENETOCLAX COMBINATION THERAPY IN ACUTE MYELOID LEUKEMIA AND MYELODYSPLASTIC SYNDROME- SINGLE CENTER EXPERIENCE FROM A RESOURCE LIMITED COUNTRY 低甲基化药物和venetoclax联合治疗急性髓系白血病和骨髓增生异常综合征的现实世界结果——来自资源有限国家的单中心经验
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103918
Maryam Khan
{"title":"REAL WORLD OUTCOMES OF HYPOMETHYLATING AGENTS AND VENETOCLAX COMBINATION THERAPY IN ACUTE MYELOID LEUKEMIA AND MYELODYSPLASTIC SYNDROME- SINGLE CENTER EXPERIENCE FROM A RESOURCE LIMITED COUNTRY","authors":"Maryam Khan","doi":"10.1016/j.htct.2025.103918","DOIUrl":"10.1016/j.htct.2025.103918","url":null,"abstract":"<div><h3>Objective</h3><div>In this study, we aim to evaluate the safety, efficacy and response rates of venetoclax and HMA combination therapy in patients with AML and MDS. This study provides outcomes from a tertiary care center in Pakistan, giving insights into the outcomes and impact of this therapeutic regimen in a resource-limited country.</div></div><div><h3>Methodology</h3><div>We conducted a retrospective analysis on 96 patients of which 54 patients had AML and 42 had MDS. All the patients received venetoclax combined with HMA at a single center from January 2020 to December 2024. The primary outcomes measured for AML were Overall Survival (OS), Progression Free Survival (PFS) and response rates (Complete Remission [CR], Partial Remission [PR], Stable Disease [SD] and No Response [NR]) while for MDS response was assessed as per IWG criteria.</div></div><div><h3>Results</h3><div>AML cohort (n = 54) had a male-to-female ratio of 2:1, median age of 52 (IQR:37‒62.2). Risk stratification showed good risk in 3 (5.6%), intermediate in 37 (68.5%) and poor risk in 14 (25.9%) patients. Treatment was given in first line in 41 (75.9%). Indications for first line treatment were age or frailty in 27 (50%), infections in 3 (3.9%) and cardiotoxicity in 2 (3.8%). Total 44 (81.5%) patients received azacytidine and 10 (18.5%) decitabine. Overall Response Rate (ORR) after cycle 1, cycle 2 and EOT was 26 (48.1%), 34 (63%) and 36 (66.7%), CR rate was 15 (27.8%), 27 (50%) and 30 (55.6%) respectively. High dose cytarabine consolidation and venetoclax maintenance were given to 5 (9.3%) and 10 (18.5%) patients respectively. Seven (12.9%) patients underwent HSCT of which 5 (71.4%) received allogenic, 1 haploidentical and 1 received autologous transplant (28.5%). ORR at last follow-up was 27 (50%) of which 24 (88.8%) had CR and 3 (11.1) had CRi. There was no response in 14 (25.9%) and disease relapse in 10 (18.5%) patients. The OS of AML cohort was 77.4% with median 1250 survival days (95% CI 139‒2360) and DFS was 52.8% with median survival 438 days (95% CI 65‒761). The MDS cohort (n = 42) had a male-to-female ratio 9.5:1 with 51-years median age (IQR: 36.5‒57.5). Genetic mutations were present in 3 (7.3%) of which TP53 mutation, del7q were present in 1 each whereas 1 patient had ASXL1, TET2 EZH2, RUNX1 and STAG2 mutations. The median R-IPSS and IPSS scores were 5 (IQR: 4.2‒6) and 1.5 (IQR: 0.75‒2) respectively. Thirty-one (73.8%) patients received azacytidine and 11 (26.2%) decitabine. ORR at cycle 1, cycle 2 and EOT was 12 (28.6%), 21 (51.2%), 18 (42.9%) with CR rates of 2 (4.8%), 11 (26.2%) and 11 (26.2%) respectively. Febrile neutropenia was observed in 23 (54.8%) and cycles were interrupted due to cytopenia’s in 23 (54.8%) patients. Seven (17.1%) patients received allogenic HSCT and 2 (4.9%) received haploidentical HSCT. Five (12.2%) patients received venetoclax maintenance. Eight (21.1%) patients had disease relapse. The OS of MDS cohort was 59.5% with median","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103918"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534561","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A FUSION OF NUP214 TO ABL1 ON AMPLIFIED EPISOMES IN T-CELL ACUTE LYMPHOBLASTIC LEUKEMIA: CASE REPORT AND LITERATURE REVIEW nup214与abl1在t细胞急性淋巴细胞白血病扩增发作中的融合:病例报告和文献复习
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103879
Jiantuo Liu , Hongrui Li , Li Jiang , Xiangjun Chen , Yanli He
{"title":"A FUSION OF NUP214 TO ABL1 ON AMPLIFIED EPISOMES IN T-CELL ACUTE LYMPHOBLASTIC LEUKEMIA: CASE REPORT AND LITERATURE REVIEW","authors":"Jiantuo Liu ,&nbsp;Hongrui Li ,&nbsp;Li Jiang ,&nbsp;Xiangjun Chen ,&nbsp;Yanli He","doi":"10.1016/j.htct.2025.103879","DOIUrl":"10.1016/j.htct.2025.103879","url":null,"abstract":"<div><h3>Objective</h3><div>We describe a NUP214::ABL1 fusion identified in a case of T-cell Acute Lymphoblastic Leukemia (T-ALL).</div></div><div><h3>Methodology</h3><div>The clinical data of a NUP214::ABL1 fusion gene-positive T-ALL patient were retrospectively analyzed.</div></div><div><h3>Results</h3><div>A 13-year-old girl was admitted to our hospital complaining of lower limb edema and leukocytosis. She displayed recurrent edema of both thighs accompanied by cough. A peripheral blood examination showed the following counts: White Blood Cell Count (WBC) 352.5 × 10<sup>9</sup>/L, Neutrophil count 267.91 × 10<sup>9</sup>/L, Lymphocyte count 83.19 × 10<sup>9</sup>/L, Red Blood Cell Count (RBC) 2.2 × 10<sup>12</sup>/L, hemoglobin 67g/L, platelet count 79 × 10<sup>9</sup>/L, and C-Reactive Protein (CRP) 12.52 mg/L. Leukemic blasts accounted for 90% of the bone-marrow cells. The patient demonstrated a T-cell phenotype, and showed expression of CD2, CD3(dim), CD4, CD5, CD7(bri), CD10, CD34, CD38, CD99 and cCD3. A G-band-staining chromosomal analysis revealed normal karyotype. A Fluorescence In Situ Hybridization (FISH) analysis revealed ABL1 amplification (Fig. 1). A ph-like ALL33 fusion gene screening analysis discovered NUP214::ABL1 fusion. In conclusion, the child definitive diagnosed T-ALL with NUP214::ABL1 fusion. Complete remission was achieved after T-ALL induction therapy with vincristine, dexamethasone, PEG-L-asparaginase, daunorubicin, cyclophosphamide, cytarabine, mercaptopurine and dasatinib. To follow-up date, the patient's condition was stable in consolidation therapy phase.</div></div><div><h3>Conclusions</h3><div>NUP214::ABL1 fusion is present in 6% of T-ALL cases in both children and adults, it is cryptic by conventional cytogenetics but detected by FISH using a ABL1 probe. FISH analysis reveals multiple extrachromosomal ABL1 sites in metephase cells and amplified ABL1 signals in interphase cells. The amplified signals or episomes are the result of the excision of the 9q34 region between the ABL1 and NUP214 breakpoints followed by circularization of the fragment. NUP214::ABL1 fusion T-ALL represents a distinct form of high-risk leukaemia with early replase and poor prognosis. Because the ABL1 fusions are sensitive to Tyrosine Kinase Inhibitors (TKIs), the strategy of conventional chemotherapy with TKIs can improve outcome in NUP214::ABL1 fusion T-ALL.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103879"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534548","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A NEW LOOK AT THE TREATMENT OF PATIENTS WITH ACUTE LEUKEMIA 治疗急性白血病的新方法
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103880
MirEldar Babayev
{"title":"A NEW LOOK AT THE TREATMENT OF PATIENTS WITH ACUTE LEUKEMIA","authors":"MirEldar Babayev","doi":"10.1016/j.htct.2025.103880","DOIUrl":"10.1016/j.htct.2025.103880","url":null,"abstract":"<div><h3>Objective</h3><div>For the first time, we had to organize an induction period for the treatment of acute leukemia in an outpatient setting. The reason was the problems that arose during the hospitalization of patients during COVID-19 infection. to study the effectiveness of the obtained results and to find out the possibility and importance of widespread use of this tactic in the future.</div></div><div><h3>Methodology</h3><div>The study group included 25 patients diagnosed with acute leukemia. Among them, 21 patients had lymphoblastic leukemia (19 patients with B-cells, 4 patients with T-cells), 2 patients with myeloblastic leukemia (1 patient with M2, 1 patient with promyelocytic leukemia M3). The age range of the patients was from 2 years to 2 months to 15 years (median = 8.5-years). The male/female ratio was 10/15. Treatment of acute lymphoblastic leukemia was carried out according to the Moscow-Berlin-2015 program, the B-ImRG protocol was used in 15 patients, the A-SRG protocol in 2 patients, Bt(12:21) in 2 patients, the T-Low protocol in 1 patient and protocol T-ImRG in 3 patients. In one of the patients with Myeloblastic leukemia (M2), the “7+3” protocol was used, in the other (M3) the APL-.2000 protocol was used.</div></div><div><h3>Results</h3><div>Obtained showed that the induction period of treatment for patients with acute leukemia can be carried out completely on an outpatient basis. The organization of treatment in the “day hospital + night outpatient” format made it possible to carry out both the main treatment (chemotherapy) and concomitant therapy in a timely and without problems. Replacing intravenous “flush therapy” with oral fluids did not cause serious problems, including “lysis syndrome”. The initial leukocyte count (10.6‒116 × 10<sup>9/l</sup>), as well as the level of blastemia (4%‒99%) and blastosis (45.4%‒96.8%) did not cause serious concern in any patient, despite the standard of concomitant therapy. Biochemical parameters, including nitrogen metabolism parameters, fluctuated within normal limits in all patients. In all patients, the induction course was carried out to the end and ended in complete remission.</div></div><div><h3>Conclusion</h3><div>The results showed the possibility and prospects of further expansion of outpatient treatment of patients with acute leukemia.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103880"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534549","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
LONG-TERM AND LATE EFFECTS OF CHEMOTHERAPY IN CHILDHOOD ACLEUKEMIA AND THEIR MANAGEMENT SINGLE INSTITUTION OBSERVATIONUTE LYMPHOBLASTIC 单机构观察儿童急性白血病化疗的远期和晚期疗效及其处理
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103917
Konul Baghirova
{"title":"LONG-TERM AND LATE EFFECTS OF CHEMOTHERAPY IN CHILDHOOD ACLEUKEMIA AND THEIR MANAGEMENT SINGLE INSTITUTION OBSERVATIONUTE LYMPHOBLASTIC","authors":"Konul Baghirova","doi":"10.1016/j.htct.2025.103917","DOIUrl":"10.1016/j.htct.2025.103917","url":null,"abstract":"<div><h3>Introduction</h3><div>Acute Lymphoblastic Leukemia (ALL) remains the most common malignant disease of childhood, accounting for approximately 26% of pediatric oncology diagnoses. Modern treatment approaches, particularly multi-agent chemotherapy regimens such as the BFM protocol, have significantly improved 5-year survival rates, reaching up to 96%. However, the toxicity of chemotherapy regimens, particularly late effects, negatively impacts long-term quality of life and clinical outcomes. These adverse effects include cardiotoxicity, secondary malignancies, endocrine dysfunction, and neurological damage. Given the limitations of conventional treatment protocols, exploring less toxic and more effective therapeutic strategies is of critical importance.</div></div><div><h3>Objective</h3><div> <!-->• To identify the late effects of chemotherapy in pediatric patients treated for ALL.</div><div> <!-->• To propose effective strategies for the early detection and management of these effects.</div><div> <!-->• To compare the BFM protocol applied in Azerbaijan with international experiences.</div></div><div><h3>Materials and methods</h3><div>This study includes 120 pediatric patients diagnosed with ALL and treated at the National Hematology Center of Azerbaijan between 2020 and 2023. A retrospective analysis of patient records was conducted. The evaluation of late chemotherapy effects was performed using internationally standardized methodologies:</div><div> <strong>•</strong> Cardiotoxicity: Echocardiography and pro-BNP biomarker measurements.</div><div> <strong>•</strong> Neurotoxicity: Clinical neurological assessment and electrophysiological testing.</div><div> <strong>•</strong> Endocrine dysfunction: Thyroid function tests, insulin resistance evaluation, and growth hormone monitoring.</div><div> <strong>•</strong> Secondary malignancies: Long-term follow-up and biomolecular analyses.</div></div><div><h3>Results</h3><div>Among the analyzed patients, 38% exhibited various late-onset adverse effects. The incidence rates of key toxicity types were as follows:</div></div><div><h3>Conclusion</h3><div>This study demonstrates the significant late effects of chemotherapy in pediatric patients treated with the BFM protocol in Azerbaijan. The findings emphasize the importance of implementing routine monitoring mechanisms, particularly for cardiotoxicity and endocrine dysfunction. International literature suggests that incorporating novel therapeutic agents, such as CAR-T cell therapy and targeted therapies like blinatumomab, into treatment regimens may improve clinical outcomes.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103917"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534560","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
INVESTIGATION OF THE RELATIONSHIP BETWEEN COMPASSION AND BURNOUT AMONG HEMATOLOGIST AND ONCOLOGIST 血液科和肿瘤科医生同情心与职业倦怠关系的调查研究
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103908
Nisa Aliş , Aze Buyruk , Birol Güvenç , Berksoy Şahin , Naciye Nur Tozluklu
{"title":"INVESTIGATION OF THE RELATIONSHIP BETWEEN COMPASSION AND BURNOUT AMONG HEMATOLOGIST AND ONCOLOGIST","authors":"Nisa Aliş ,&nbsp;Aze Buyruk ,&nbsp;Birol Güvenç ,&nbsp;Berksoy Şahin ,&nbsp;Naciye Nur Tozluklu","doi":"10.1016/j.htct.2025.103908","DOIUrl":"10.1016/j.htct.2025.103908","url":null,"abstract":"<div><h3>Objective</h3><div>Burnout disproportionately affects hematologists and oncologists due to high-stress clinical environments, long working hours, and emotional demands of caring for critically ill patients. While compassion is integral to patient care, the relationship between compassion and burnout has not yet been sufficiently explored. This study investigates the relationship between compassion and burnout in hematologists and oncologists, contextualizing findings within using multivariate linear regression and Pearson's correlation analyses.</div></div><div><h3>Methodology</h3><div>A cross-sectional survey of 161 hematologists and oncologists was conducted using validated instruments: the Maslach Burnout Inventory (MBI) to assess burnout (burnout, depersonalization, personal achievement) and the Compassion Scale to measure compassion subdomains (kindness, indifference, common humanity, mindfulness, separation, disengagement). Participants were stratified by practice setting (academic vs. community), gender, and clinical focus.</div></div><div><h3>Results</h3><div>While the scores from the Burnout subscale and Depersonalization did not statistically predict the scores of the Compassion Scale (p &gt; 0.05) the scores from the Personal Achievement statistically predicted the scores of the Compassion Scale (β = -0.352; p &lt; 0.05). Pearson’s correlation analysis revealed statistically significant relationships between the Burnout scores, and Kindness, Common Humanity, Mindfulness, and Disengagement of the Compassion Scale (p &lt; 0.05) but not with the Indifference or Separation (p &gt; 0.05). A statistically significant relationships was only found between the Depersonalization scores and the Indifference (p &lt; 0.05) but not the other components of the Compassion Scale (p &gt; 0.05). While strong and positive correlations were found between the Personal Achievement scores and the Kindness and Common Humanity of the Compassion Scale, no significant relationships were observed with Disengagement, Mindfulness, Indifference, or Separation (p &gt; 0.05).</div></div><div><h3>Conclusion</h3><div>The compassion was not completely corelated with Burnout, but some subscales of Burnout were corelated with some subscales of the Compassion such as personal achievement increases, the levels of kindness, common humanity, and mindfulness also increases. Individuals with higher burnout levels exhibit increased indifference and as indifference increases, the relationship with kindness alsso strengthens.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103908"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534221","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
VENETOCLAX-BASED VERSUS 7+3 INDUCTION THERAPY IN FIT YOUNGER ADULTS WITH NEWLY DIAGNOSED NON-CBF AML 以venetoclax为基础的7+3诱导治疗对新诊断的非cbf aml的适合年轻成人的影响
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103876
Dmitrii Zhogolev, Nikita Pastukhov, Bella Aybova, Anna Smirnova, Ivan Moiseev, Sergey Bondarenko, Alexander Kulagin
{"title":"VENETOCLAX-BASED VERSUS 7+3 INDUCTION THERAPY IN FIT YOUNGER ADULTS WITH NEWLY DIAGNOSED NON-CBF AML","authors":"Dmitrii Zhogolev,&nbsp;Nikita Pastukhov,&nbsp;Bella Aybova,&nbsp;Anna Smirnova,&nbsp;Ivan Moiseev,&nbsp;Sergey Bondarenko,&nbsp;Alexander Kulagin","doi":"10.1016/j.htct.2025.103876","DOIUrl":"10.1016/j.htct.2025.103876","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Objective&lt;/h3&gt;&lt;div&gt;Venetoclax-based regimens have emerged as a standard therapeutic option for newly diagnosed Acute Myeloid Leukemia (AML) in patients deemed unfit for intensive chemotherapy. However, the efficacy of venetoclax in fit patients remains an area of ongoing investigation. Notably, in specific AML subsets, such as Core Binding Factor (CBF) AML, venetoclax-based therapy has demonstrated inferior outcomes compared to intensive chemotherapy. Despite these findings, direct comparative data between venetoclax-based therapies and intensive induction chemotherapy in fit patients with non-CBF AML remains limited. This study aims to evaluate and compare the clinical outcomes of fit younger adult patients with newly diagnosed non-CBF AML who underwent induction therapy with either venetoclax-based regimens or standard 7+3 chemotherapy.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methodology&lt;/h3&gt;&lt;div&gt;This retrospective cohort study included patients assessed at RM Gorbacheva Research Institute for eligibility for Allogeneic Hematopoietic Stem Cell Transplantation (allo-HSCT) from June 2020 to August 2024. Eligible patients were adults with non-CBF AML who received either 7+3 induction chemotherapy or venetoclax in combination with a Hypomethylating Agent (HMA) or Low-Dose Cytarabine (LDAC). Exclusion criteria included age &gt; 60-years and a Hematopoietic Cell Transplantation-Specific Comorbidity Index (HCT-CI) score &gt; 2. To minimize confounding, pairwise propensity score matching was performed based on age, secondary AML status, and ELN 2022 risk classification. Remission in this study referred to Complete Remission (CR), CR with partial or incomplete hematologic recovery, and a morphological leukemia-free state according to the ELN response criteria. Patients who failed to achieve remission after two induction cycles were categorized as refractory. Overall Survival (OS) was defined as the time from start of treatment to death from any cause. Event-Free Survival (EFS) included refractoriness, relapse, or death, with censoring at the last follow-up. Relapse was defined as the reappearance of ≥5% blasts in bone marrow or peripheral blood, or extramedullary disease. Non-Relapse Mortality (NRM) was defined as death in remission. Survival analysis was conducted using the Kaplan-Meier method and log-rank test. Cumulative incidences of relapse and NRM were assessed using competing risk models with Gray’s test. Statistical analyses were performed using R (version 4.4.2). The study adhered to the Declaration of Helsinki and Good Clinical Practice guidelines and was approved by the Pavlov University Ethical Committee.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;A total of 112 patients met the inclusion criteria, with 64.3% (n = 72) receiving 7+3 induction and 35.7% (n = 40) treated with venetoclax plus HMA/LDAC. After propensity score matching, each treatment arm included 26 patients. Baseline characteristics of the matched cohort are summarized in Table 1. Remission rate","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103876"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
TREATMENT MANAGEMENT IN MULTIPLE MYELOMA WITH RENAL DISORDER 多发性骨髓瘤合并肾脏疾病的治疗管理
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103905
Meryem Sener
{"title":"TREATMENT MANAGEMENT IN MULTIPLE MYELOMA WITH RENAL DISORDER","authors":"Meryem Sener","doi":"10.1016/j.htct.2025.103905","DOIUrl":"10.1016/j.htct.2025.103905","url":null,"abstract":"<div><h3>Introduction</h3><div>Multiple Myeloma (MM) is a plasma cell neoplasm characterized by the accumulation of monoclonal plasma cells in the bone marrow, leading to osteolytic lesions, anemia, infections, hypercalcemia, and kidney impairment. This review focuses on managing kidney disease in MM, particularly light chain cast nephropathy.</div></div><div><h3>Case</h3><div>A 66-year-old male with progressive fatigue, dizziness, weight loss, and recurrent pneumonia was referred for anemia evaluation on 28.11.2024. Laboratory results showed:</div><div> <!-->• Hemoglobin: 5.4 g/dL, Hematocrit: 18.3%, Platelets: 56 × 10³/uL</div><div> <!-->• Creatinine: 1.39 mg/dL, Calcium: 9.7 mg/dL, Total Protein: 9 g/L, Albumin: 2.9 g/L</div><div> <!-->• IgA: 4295 mg/dL, IgG: 141 mg/dL, IgM: &lt; 5 mg/dL</div><div>Peripheral smear showed rouleaux formation, and protein electrophoresis revealed a gamma peak. Immunofixation detected IgA-lambda bands, with bone marrow biopsy confirming 70% plasma cell infiltration. The patient was started on VCD chemotherapy (bortezomib, cyclophosphamide, dexamethasone). Neutropenia worsened, requiring G-CSF support. Renal function improved, and zoledronic acid was given for widespread lytic lesions. Due to Febrile Neutropenia (FEN), treatment was switched to VRD (bortezomib, lenalidomide, dexamethasone). After four cycles, symptoms improved, and cytopenia’s resolved. Although serum immunofixation remained positive, the patient achieved a Very Good Partial Response (VGPR). A follow-up bone marrow biopsy is planned after four more cycles, with Autologous Stem Cell Transplant (ASCT) scheduled if the response continues.</div></div><div><h3>Conclusion</h3><div>Managing MM with renal impairment requires balancing efficacy and toxicity. A four-drug regimen (Dara-CyBorD or Isa-CyBorD) is preferred in fit patients with severe AKI, while a three-drug regimen (Dara-Vd) is recommended for frail patients. If daratumumab or isatuximab is unavailable, CyBorD is an alternative. Bortezomib, daratumumab, and isatuximab can be safely used in kidney dysfunction without dose adjustments. Lenalidomide is avoided in AKI unless refractory. In this case, VCD was chosen initially, and after renal improvement, VRD was used despite FEN episodes. The patient achieved VGPR and is now planned for ASCT.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103905"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534307","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
THE SUCCESS LIES ON CLINICAL SUSPECT: THE SYNCHRONOUS CANCERS PRESENTING AS PULMONARY AND VERTEBRAL MASS LESIONS 成功在于临床怀疑:同步癌表现为肺和椎体肿块病变
IF 1.8
Hematology, Transfusion and Cell Therapy Pub Date : 2025-07-01 DOI: 10.1016/j.htct.2025.103906
Ulviyya Hasanzade, Metban Mastanzade, Sevgi Kalayoğlu Beşışık
{"title":"THE SUCCESS LIES ON CLINICAL SUSPECT: THE SYNCHRONOUS CANCERS PRESENTING AS PULMONARY AND VERTEBRAL MASS LESIONS","authors":"Ulviyya Hasanzade,&nbsp;Metban Mastanzade,&nbsp;Sevgi Kalayoğlu Beşışık","doi":"10.1016/j.htct.2025.103906","DOIUrl":"10.1016/j.htct.2025.103906","url":null,"abstract":"<div><h3>Objective</h3><div>It is a well-known epidemiological research issue that cancer patients are at high risk for developing multiple primary cancers. The risk increase is more likely among cancer survivors and elderly people. We present a case of synchronous cancers with pulmonary cancer and extramedullary plasmacytoma. A 68-year-old male patient was evaluated for back pain, walking difficulty, and urinary incontinence. MRI showed a vertebral mass lesion on T10‒11, with a pre-diagnosis of metastatic bone disorder. A PET-CT scan was performed to find out the primary cancer. This time, two mass lesions were striking: one on the right infrahilar region of the lungs and the other as a large lesion on the vertebras, as seen on MRI, which seemed to be two separate malignant lesions. Two biopsies were decided. The patient’s clinical picture deteriorated, and an urgent surgery for decompression and a diagnostic lung biopsy by bronchoscopy were performed. Histology of the vertebral lesion revealed kappa monotypic cell infiltration consistent with plasmacytoma, and histology of the lung revealed non-small cell lung carcinoma. He had a monoclonal gammopaty as IgG kappa with a level of 1.24 g/dL. Further investigation covered bone marrow, which confirmed the diagnosis of solitary plasmocytoma and primary lung carcinoma. Treatment was designed as radiotherapy for plasmocytoma and referral to the oncology unit with a recommendation for three monthly follow-ups for pursuing active myeloma development.</div></div><div><h3>Results</h3><div>Multiple cancers comprise two or more primary cancers occurring in an individual originating in a primary site or tissue and are neither an extension nor a recurrence or metastasis. According to the timing of the cancers' diagnosis, the development of different cancers may be differentiated as synchronous or metachronous. The risk for the development of multiple primary cancers may be multifactorial as inherited predisposition to cancer; the lifestyle, cancerogen exposure related with environmental factors; previous cancer and increased survival and surveillance of cancer patients. We highlighted the need for comprehensive epidemiological data collection in cancer patients by publishing this case.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103906"},"PeriodicalIF":1.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144534308","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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