Pub Date : 2025-01-01Epub Date: 2025-01-20DOI: 10.1016/j.lrr.2025.100499
E. Amabile, F. Fazio, M. Martelli, M.T. Petrucci
{"title":"Corrigendum to “First-line therapy with daratumumab, lenalidomide and dexamethasone for patient with POEMS syndrome: A case report” [Leukemia Research Reports (2024) Volume 22, 100491]","authors":"E. Amabile, F. Fazio, M. Martelli, M.T. Petrucci","doi":"10.1016/j.lrr.2025.100499","DOIUrl":"10.1016/j.lrr.2025.100499","url":null,"abstract":"","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100499"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144298687","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}
Pub Date : 2025-01-01Epub Date: 2025-03-14DOI: 10.1016/j.lrr.2025.100506
Alaa Eldein Yahia , Ibrahim Motabi , Abdullah A. Alsakkaf , Kamal Alzahrani , Laila M. Alsuhaibani , Bilal Albtoosh , Abdullah Khaled AlBathi , Abdullah M. Alrajhi
Acute lymphoblastic leukemia (ALL) represents only 20 % of adult acute leukemias, while Lymphoblastic lymphoma is even rarer, accounting for 2 % of adult non-Hodgkin lymphomas. T-acute lymphoblastic leukemia (T-ALL) and T-lymphoblastic lymphoma (T-LBL) are neoplasms characterized by the presence of immature T-cell precursors or lymphoblasts. Relapsed T-ALL or LBL is associated with a very poor prognosis, necessitating the exploration of novel therapeutic approaches. This case report describes the use of Venetoclax in combination with Cladribine, Idarubicin, and Cytarabine (CLIA) as salvage therapy for relapsed T-ALL/T-LBL. The treatment regimen resulted in remission and negative minimal residual disease. However, it was accompanied by delayed count recovery, febrile neutropenia, and Central Line-Associated Bloodstream Infection. The management of central nervous system involvement was challenging due to low platelet counts requiring transfusion support. The findings highlight the need for further investigation into the efficacy and optimal therapeutic regimen for relapsed T-ALL/T-LBL. Additionally, the case emphasizes the importance of early salvage therapy and potentially consolidative hematopoietic stem cell transplantation for improved survival outcomes in relapsed T-ALL/T-LBL patients.
{"title":"Venetoclax combination with Cladribine, idarubicin, Cytarabine for relapsed T-Cell acute lymphoblastic leukemia/lymphoblastic lymphoma treatment: A case report and literature review","authors":"Alaa Eldein Yahia , Ibrahim Motabi , Abdullah A. Alsakkaf , Kamal Alzahrani , Laila M. Alsuhaibani , Bilal Albtoosh , Abdullah Khaled AlBathi , Abdullah M. Alrajhi","doi":"10.1016/j.lrr.2025.100506","DOIUrl":"10.1016/j.lrr.2025.100506","url":null,"abstract":"<div><div>Acute lymphoblastic leukemia (ALL) represents only 20 % of adult acute leukemias, while Lymphoblastic lymphoma is even rarer, accounting for 2 % of adult non-Hodgkin lymphomas. T-acute lymphoblastic leukemia (T-ALL) and T-lymphoblastic lymphoma (T-LBL) are neoplasms characterized by the presence of immature T-cell precursors or lymphoblasts. Relapsed T-ALL or LBL is associated with a very poor prognosis, necessitating the exploration of novel therapeutic approaches. This case report describes the use of Venetoclax in combination with Cladribine, Idarubicin, and Cytarabine (CLIA) as salvage therapy for relapsed T-ALL/T-LBL. The treatment regimen resulted in remission and negative minimal residual disease. However, it was accompanied by delayed count recovery, febrile neutropenia, and Central Line-Associated Bloodstream Infection. The management of central nervous system involvement was challenging due to low platelet counts requiring transfusion support. The findings highlight the need for further investigation into the efficacy and optimal therapeutic regimen for relapsed T-ALL/T-LBL. Additionally, the case emphasizes the importance of early salvage therapy and potentially consolidative hematopoietic stem cell transplantation for improved survival outcomes in relapsed T-ALL/T-LBL patients.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100506"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143685840","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01Epub Date: 2024-12-18DOI: 10.1016/j.lrr.2024.100496
Whanhui Chi , Juhyeon Song , Tyler J. Varisco
Multiple myeloma (MM) is a rare hematologic malignancy with a 5-year survival rate of 52 %. For transplant-eligible MM patients, high-dose chemotherapy followed by autologous stem cell transplant (ASCT) is recommended. Given the complexities of the ASCT procedure, understanding patient-specific factors and their impact on treatment decisions is essential.
Our study examines patient characteristics and patterns of health resource utilization associated with ASCT receipt in patients with MM.
This retrospective study used the Merative™ MarketScan® database from 2017 to 2019 to analyze working-aged adults (18–65 years) with MM. We categorized 643 ASCT recipients by demographic characteristics (age, sex, region, employment status, year of ASCT procedure) and clinical factors (Charlson Comorbidity Index score). We assessed health resource utilization, focusing on ASCT-related costs, including total payments and hospitalization duration. Descriptive statistics were calculated for all variables, with means, medians, standard deviations for continuous variables, and frequencies for categorical variables. Pearson correlation assessed the relationship between total payment and hospitalization duration.
Over 80 % of patients were over 50, highlighting the need for age-specific clinical strategies. Most patients had CCI scores of 2–4, indicating a moderate comorbidity burden. The mean hospitalization duration was 21.71 days, with average ASCT costs totaling $166,235.99. The correlation coefficient of 0.21 indicated that total payments also increase as the number of hospitalization days increases.
These findings highlight the need for tailored care approaches and resource allocation in ASCT, informing future research and clinical decision-making.
{"title":"Retrospective claims data analysis of ASCT characteristics and costs for working-age, multiple myeloma patients in the US, 2017–2019","authors":"Whanhui Chi , Juhyeon Song , Tyler J. Varisco","doi":"10.1016/j.lrr.2024.100496","DOIUrl":"10.1016/j.lrr.2024.100496","url":null,"abstract":"<div><div>Multiple myeloma (MM) is a rare hematologic malignancy with a 5-year survival rate of 52 %. For transplant-eligible MM patients, high-dose chemotherapy followed by autologous stem cell transplant (ASCT) is recommended. Given the complexities of the ASCT procedure, understanding patient-specific factors and their impact on treatment decisions is essential.</div><div>Our study examines patient characteristics and patterns of health resource utilization associated with ASCT receipt in patients with MM.</div><div>This retrospective study used the Merative™ MarketScan® database from 2017 to 2019 to analyze working-aged adults (18–65 years) with MM. We categorized 643 ASCT recipients by demographic characteristics (age, sex, region, employment status, year of ASCT procedure) and clinical factors (Charlson Comorbidity Index score). We assessed health resource utilization, focusing on ASCT-related costs, including total payments and hospitalization duration. Descriptive statistics were calculated for all variables, with means, medians, standard deviations for continuous variables, and frequencies for categorical variables. Pearson correlation assessed the relationship between total payment and hospitalization duration.</div><div>Over 80 % of patients were over 50, highlighting the need for age-specific clinical strategies. Most patients had CCI scores of 2–4, indicating a moderate comorbidity burden. The mean hospitalization duration was 21.71 days, with average ASCT costs totaling $166,235.99. The correlation coefficient of 0.21 indicated that total payments also increase as the number of hospitalization days increases.</div><div>These findings highlight the need for tailored care approaches and resource allocation in ASCT, informing future research and clinical decision-making.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100496"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11730578/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984835","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-01Epub Date: 2025-08-07DOI: 10.1016/j.lrr.2025.100535
Tiffany Nong , Xiaoqiong Wang , Wenhui Li , Ellen Madarang , Justin Watts , Justin Taylor
Hairy cell leukemia (HCL) is a rare, indolent B-cell lymphoma that is highly responsive to the purine analog cladribine. A defining feature of classical HCL is the presence of the BRAF V600E mutation, which is almost universally present. The transformation of HCL to an aggressive large B-cell lymphoma is extremely rare. Here, we report a case of intercurrent presentation of worsening classical HCL with a rapidly evolving BRAF V600E-negative aggressive large B-cell lymphoma and associated hemophagocytic lymphohistiocytosis (HLH). The contiguous presentation of these three entities posed significant diagnostic and therapeutic challenges. Despite treatment with cladribine, the patient’s condition deteriorated, leading to a palliative focus. This case underscores the challenges of managing multiple intercurrent hematologic malignancies manifesting with overlapping symptoms and sites of organ involvement but non-overlapping treatments. The presence of HLH as a complication in patients with malignancy further complicates the clinical picture and requires early recognition and prompt intervention. Further research is needed to better understand the pathophysiological links between HCL, large cell transformation, and HLH. Additionally, registry or other cross-sectional studies with larger numbers of HCL patients could clarify the exact frequency of these rare but fatal complications.
{"title":"Intercurrent presentation of hairy cell leukemia and diffuse large B-cell lymphoma with hemophagocytic lymphohistiocytosis: a case report","authors":"Tiffany Nong , Xiaoqiong Wang , Wenhui Li , Ellen Madarang , Justin Watts , Justin Taylor","doi":"10.1016/j.lrr.2025.100535","DOIUrl":"10.1016/j.lrr.2025.100535","url":null,"abstract":"<div><div>Hairy cell leukemia (HCL) is a rare, indolent B-cell lymphoma that is highly responsive to the purine analog cladribine. A defining feature of classical HCL is the presence of the BRAF V600E mutation, which is almost universally present. The transformation of HCL to an aggressive large B-cell lymphoma is extremely rare. Here, we report a case of intercurrent presentation of worsening classical HCL with a rapidly evolving BRAF V600E-negative aggressive large B-cell lymphoma and associated hemophagocytic lymphohistiocytosis (HLH). The contiguous presentation of these three entities posed significant diagnostic and therapeutic challenges. Despite treatment with cladribine, the patient’s condition deteriorated, leading to a palliative focus. This case underscores the challenges of managing multiple intercurrent hematologic malignancies manifesting with overlapping symptoms and sites of organ involvement but non-overlapping treatments. The presence of HLH as a complication in patients with malignancy further complicates the clinical picture and requires early recognition and prompt intervention. Further research is needed to better understand the pathophysiological links between HCL, large cell transformation, and HLH. Additionally, registry or other cross-sectional studies with larger numbers of HCL patients could clarify the exact frequency of these rare but fatal complications.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"24 ","pages":"Article 100535"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144829174","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}
Pub Date : 2025-01-01Epub Date: 2025-04-12DOI: 10.1016/j.lrr.2025.100509
Mateusz Ziarkiewicz , Justyna Szczygieł , Marta Legatowicz-Koprowska , Joanna Drozd-Sokołowska , Piotr Boguradzki , Krzysztof Jamroziak , Grzegorz Basak
Background
AL amyloidosis is a systemic protein misfolding disorder characterized by organ deposition of monoclonal immunoglobulin fragments, with insidious onset and progressive course. The plasma cell clone in the bone marrow is relatively small and typically does not impair hematopoiesis, in contrast to multiple myeloma. Herein we present a novel observation of increased thrombocyte, leukocyte and erythrocyte counts in a subset of AL amyloidosis patients.
Material and Methods
We performed a retrospective analysis of medical records of all consecutive patients diagnosed with AL amyloidosis at the Medical University of Warsaw in years 2001–2022, which included clinical, pathological and laboratory data, as well as treatment protocols and outcomes.
Results
Twenty-three patients out of 124 (18.4 %) included had elevated blood counts: 17 (13.6 %) had leukocytosis with neutrophilia, 7 (5.6 %) had thrombocytosis, whereas 2 (1.6 %) had erythrocytosis. In comparison to the remaining AL population this subgroup was characterized by younger age (median 57 vs 62 years, p = 0.018), higher frequency of hepatomegaly (42.9 % vs.14.7 %, p = 0.004), higher median alkaline phosphatase concentration (129 U/L vs 93 U/L, p = 0.006) and more frequent hepatic amyloidosis (34.8 % vs 10.3 %, p = 0.003). None of the patients had definite features of a myeloproliferative neoplasm, although genetic testing was available in 5 out of 9 cases with thrombocytosis or erythrocytosis. There were no significant differences in terms of survival between patients with elevated cell counts and non-polycythemic patients (median overall survival 2.9 vs 6.6 years, p = 0.51, median event-free survival 0.7 vs 1.8 years, p = 0.29, respectively).
Conclusions
Elevated peripheral blood counts in a subset of patients with AL amyloidosis constitute a rare but significant phenomenon and appear to be associated with frequent hepatic involvement. We hypothesize that cytokine deregulation and hyposplenism may belong to its pathomechanisms.
{"title":"AL amyloidosis with elevated peripheral blood cell counts – A frequent association with liver involvement. A single-center retrospective study","authors":"Mateusz Ziarkiewicz , Justyna Szczygieł , Marta Legatowicz-Koprowska , Joanna Drozd-Sokołowska , Piotr Boguradzki , Krzysztof Jamroziak , Grzegorz Basak","doi":"10.1016/j.lrr.2025.100509","DOIUrl":"10.1016/j.lrr.2025.100509","url":null,"abstract":"<div><h3>Background</h3><div>AL amyloidosis is a systemic protein misfolding disorder characterized by organ deposition of monoclonal immunoglobulin fragments, with insidious onset and progressive course. The plasma cell clone in the bone marrow is relatively small and typically does not impair hematopoiesis, in contrast to multiple myeloma. Herein we present a novel observation of increased thrombocyte, leukocyte and erythrocyte counts in a subset of AL amyloidosis patients.</div></div><div><h3>Material and Methods</h3><div>We performed a retrospective analysis of medical records of all consecutive patients diagnosed with AL amyloidosis at the Medical University of Warsaw in years 2001–2022, which included clinical, pathological and laboratory data, as well as treatment protocols and outcomes.</div></div><div><h3>Results</h3><div>Twenty-three patients out of 124 (18.4 %) included had elevated blood counts: 17 (13.6 %) had leukocytosis with neutrophilia, 7 (5.6 %) had thrombocytosis, whereas 2 (1.6 %) had erythrocytosis. In comparison to the remaining AL population this subgroup was characterized by younger age (median 57 vs 62 years, <em>p</em> = 0.018), higher frequency of hepatomegaly (42.9 % vs.14.7 %, <em>p</em> = 0.004), higher median alkaline phosphatase concentration (129 U/L vs 93 U/L, <em>p</em> = 0.006) and more frequent hepatic amyloidosis (34.8 % vs 10.3 %, <em>p</em> = 0.003). None of the patients had definite features of a myeloproliferative neoplasm, although genetic testing was available in 5 out of 9 cases with thrombocytosis or erythrocytosis. There were no significant differences in terms of survival between patients with elevated cell counts and non-polycythemic patients (median overall survival 2.9 vs 6.6 years, <em>p</em> = 0.51, median event-free survival 0.7 vs 1.8 years, <em>p</em> = 0.29, respectively).</div></div><div><h3>Conclusions</h3><div>Elevated peripheral blood counts in a subset of patients with AL amyloidosis constitute a rare but significant phenomenon and appear to be associated with frequent hepatic involvement. We hypothesize that cytokine deregulation and hyposplenism may belong to its pathomechanisms.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100509"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143843498","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}
Mast cell leukemia (MCL) is a rare and aggressive form of systemic mastocytosis with a poor prognosis. Understanding the different therapeutic responses to corticosteroids in MCL is crucial for improving patient outcomes.
Case presentation
We present a case of a 74-year-old Chinese female with primary acute MCL who exhibited different responses to dexamethasone and methylprednisolone. She was admitted with persistent fever, dyspnea, severe fatigue, and bone pain, alongside splenomegaly and cytopenia. Diagnosis was confirmed through marrow aspirate analysis, chemical staining, flow cytometry, and biopsy, revealing atypical mast cells positive for CD117, CD9, CD81, CD33, CD13, CD4, and partially for CD56, but negative for CD2 and CD25. Next-generation sequencing identified heterozygous mutations in NRAS, DNMT3A, and TP53, with no KIT mutations. Initial treatment included corticosteroids and dasatinib. The patient showed a partial response to dexamethasone but significant improvement with methylprednisolone. Upon reintroduction of dexamethasone, symptoms recurred, which improved again after resuming methylprednisolone. The patient survived for three months post-diagnosis.
Conclusion
This case highlights the potential efficacy of methylprednisolone over dexamethasone in MCL treatment. This case underscores the importance of personalized treatment approaches in MCL, considering the distinct genetic profile and differential therapeutic responses to corticosteroids. Further research is needed to elucidate the mechanisms underlying these responses and to optimize treatment strategies for MCL.
{"title":"Case report: Experience of a rare case of primary acute mast cell leukemia","authors":"Zhijuan Pan, Ying Zhang, Yanru Guo, Jiajia Sun, Xinlei Guo, Zhiping Guo","doi":"10.1016/j.lrr.2025.100510","DOIUrl":"10.1016/j.lrr.2025.100510","url":null,"abstract":"<div><h3>Backgrounds</h3><div>Mast cell leukemia (MCL) is a rare and aggressive form of systemic mastocytosis with a poor prognosis. Understanding the different therapeutic responses to corticosteroids in MCL is crucial for improving patient outcomes.</div></div><div><h3>Case presentation</h3><div>We present a case of a 74-year-old Chinese female with primary acute MCL who exhibited different responses to dexamethasone and methylprednisolone. She was admitted with persistent fever, dyspnea, severe fatigue, and bone pain, alongside splenomegaly and cytopenia. Diagnosis was confirmed through marrow aspirate analysis, chemical staining, flow cytometry, and biopsy, revealing atypical mast cells positive for CD117, CD9, CD81, CD33, CD13, CD4, and partially for CD56, but negative for CD2 and CD25. Next-generation sequencing identified heterozygous mutations in <em>NRAS, DNMT3A</em>, and <em>TP5</em>3, with no <em>KIT</em> mutations. Initial treatment included corticosteroids and dasatinib. The patient showed a partial response to dexamethasone but significant improvement with methylprednisolone. Upon reintroduction of dexamethasone, symptoms recurred, which improved again after resuming methylprednisolone. The patient survived for three months post-diagnosis.</div></div><div><h3>Conclusion</h3><div>This case highlights the potential efficacy of methylprednisolone over dexamethasone in MCL treatment. This case underscores the importance of personalized treatment approaches in MCL, considering the distinct genetic profile and differential therapeutic responses to corticosteroids. Further research is needed to elucidate the mechanisms underlying these responses and to optimize treatment strategies for MCL.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100510"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143837824","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}
Pub Date : 2025-01-01Epub Date: 2025-11-25DOI: 10.1016/j.lrr.2025.100552
Liya Liu , Ying Yu , Xueyan Chen
Extramedullary breast relapse of leukemia is uncommon and primarily occurs in cases of acute myeloid leukemia(AML), and secondary acute lymphoblastic leukemia (ALL) affecting the breast may also occur occasionally. The mechanism of extramedullary involvement remains unclear; patients' risk of hematological recurrence is high. TCF3::PBX1 (also known as E2A:: PBX1) is an oncogenic transcription factor arising from the T (1; 19) (q23; P13) translocation, observed in 5 % to 6 % of childhood acute lymphoblastic leukemia (ALL) cases and approximately 3 % of adult ALL cases. Here, we report a case of B-ALL with TCF3::PBX1 fusion gene, where extramedullary breast infiltration was the earliest clinical indication of recurrence. Adult ALL with the TCF3::PBX1 fusion gene is relatively uncommon compared to other ALL. Despite the satisfactory effect of chemotherapy, the patient ultimately decided to discontinue treatment following breast infiltration by ALL.
{"title":"Extramedullary breast infiltration as the initial manifestation of adult acute B-lymphoblastic leukemia with TCF3::PBX1 fusion gene recurrence and adenofibroma: A case report","authors":"Liya Liu , Ying Yu , Xueyan Chen","doi":"10.1016/j.lrr.2025.100552","DOIUrl":"10.1016/j.lrr.2025.100552","url":null,"abstract":"<div><div>Extramedullary breast relapse of leukemia is uncommon and primarily occurs in cases of acute myeloid leukemia(AML), and secondary acute lymphoblastic leukemia (ALL) affecting the breast may also occur occasionally. The mechanism of extramedullary involvement remains unclear; patients' risk of hematological recurrence is high. <em>TCF3::PBX1</em> (also known as <em>E2A:: PBX1</em>) is an oncogenic transcription factor arising from the T (1; 19) (q23; P13) translocation, observed in 5 % to 6 % of childhood acute lymphoblastic leukemia (ALL) cases and approximately 3 % of adult ALL cases. Here, we report a case of B-ALL with <em>TCF3::PBX1</em> fusion gene, where extramedullary breast infiltration was the earliest clinical indication of recurrence. Adult ALL with the <em>TCF3::PBX1</em> fusion gene is relatively uncommon compared to other ALL. Despite the satisfactory effect of chemotherapy, the patient ultimately decided to discontinue treatment following breast infiltration by ALL.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"24 ","pages":"Article 100552"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145614382","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}
Pub Date : 2025-01-01Epub Date: 2025-07-23DOI: 10.1016/j.lrr.2025.100531
Hamid D. Habeeb Rjeib , Usama Al-Jumaily , Inas Muayad Mohammed Ali , Safa Faraj , Mohammed Fawzi Al-Qanbar , Dheyaa Aldeen Al-Khateeb , Shaima Jabbar
Background
Mature B-cell non-Hodgkin lymphoma (B-NHL) is a prevalent pediatric malignancy with significant treatment advancements. This study retrospectively analyzes clinical characteristics, treatment outcomes, and survival rates of children and adolescents diagnosed with B-NHL at Al-Hasan Al-Mojtaba Hospital between January 2014 and December 2024. A comparative analysis was performed between the LMB96 and R-CHOP regimens.
Methods
Patients with confirmed diagnoses of Large B-cell lymphoma or Burkitt’s lymphoma, based on WHO classification criteria, were included. Staging was conducted using the St. Jude system, and risk classification followed the FAB/LMB criteria. Treatment involved a modified LMB96 regimen, later replaced by R-CHOP in the last two years of the study. Event-free survival (EFS) was analyzed using Kaplan-Meier survival curves, with stratifications by staging, risk group, and gender.
Results
A total of 66 patients were included (median age: 5.8 years; 69.7 % male). Burkitt’s lymphoma was the predominant histology (86.3 %). The abdomen was the most common primary site (84.8 %). The majority of patients (72.7 %) presented with advanced-stage disease (Stages III and IV). Risk group classification identified 62.1 % of patients in Group B and 28.8 % in Group C. Kaplan-Meier survival analysis revealed Group A had the most favorable prognosis (EFS ∼100 %), followed by Group B (∼75 %), and Group C (∼50 %). Disease stage significantly influenced survival (p = 0.021), with Stage IV patients demonstrating the poorest outcomes. While female patients exhibited higher EFS than males, the difference was not statistically significant (p = 0.27). By the end of follow-up, 28.8 % of patients had experienced a fatal outcome.
Conclusion
Advanced-stage B-NHL remains prevalent, with significant survival differences based on staging and risk classification. The transition from LMB96 to R-CHOP warrants further evaluation to optimize pediatric treatment strategies. Larger studies are needed to validate observed gender-based survival trends.
{"title":"Treatment outcomes and survival analysis of pediatric mature B-Cell non-Hodgkin lymphoma: A retrospective study comparing LMB96 and R-CHOP regimens","authors":"Hamid D. Habeeb Rjeib , Usama Al-Jumaily , Inas Muayad Mohammed Ali , Safa Faraj , Mohammed Fawzi Al-Qanbar , Dheyaa Aldeen Al-Khateeb , Shaima Jabbar","doi":"10.1016/j.lrr.2025.100531","DOIUrl":"10.1016/j.lrr.2025.100531","url":null,"abstract":"<div><h3>Background</h3><div>Mature B-cell non-Hodgkin lymphoma (B-NHL) is a prevalent pediatric malignancy with significant treatment advancements. This study retrospectively analyzes clinical characteristics, treatment outcomes, and survival rates of children and adolescents diagnosed with B-NHL at Al-Hasan Al-Mojtaba Hospital between January 2014 and December 2024. A comparative analysis was performed between the LMB96 and R-CHOP regimens.</div></div><div><h3>Methods</h3><div>Patients with confirmed diagnoses of Large B-cell lymphoma or Burkitt’s lymphoma, based on WHO classification criteria, were included. Staging was conducted using the St. Jude system, and risk classification followed the FAB/LMB criteria. Treatment involved a modified LMB96 regimen, later replaced by R-CHOP in the last two years of the study. Event-free survival (EFS) was analyzed using Kaplan-Meier survival curves, with stratifications by staging, risk group, and gender.</div></div><div><h3>Results</h3><div>A total of 66 patients were included (median age: 5.8 years; 69.7 % male). Burkitt’s lymphoma was the predominant histology (86.3 %). The abdomen was the most common primary site (84.8 %). The majority of patients (72.7 %) presented with advanced-stage disease (Stages III and IV). Risk group classification identified 62.1 % of patients in Group B and 28.8 % in Group C. Kaplan-Meier survival analysis revealed Group A had the most favorable prognosis (EFS ∼100 %), followed by Group B (∼75 %), and Group C (∼50 %). Disease stage significantly influenced survival (<em>p</em> = 0.021), with Stage IV patients demonstrating the poorest outcomes. While female patients exhibited higher EFS than males, the difference was not statistically significant (<em>p</em> = 0.27). By the end of follow-up, 28.8 % of patients had experienced a fatal outcome.</div></div><div><h3>Conclusion</h3><div>Advanced-stage B-NHL remains prevalent, with significant survival differences based on staging and risk classification. The transition from LMB96 to R-CHOP warrants further evaluation to optimize pediatric treatment strategies. Larger studies are needed to validate observed gender-based survival trends.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"24 ","pages":"Article 100531"},"PeriodicalIF":0.9,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144739061","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}
Chronic myeloid leukemia (CML), is a myeloproliferative disease characterized by unregulated growth of blood forming cells in bone marrow and blood. The t(9;22)(q34;q11.2) translocation, which results in the formation of a hyperactive tyrosine kinase (BCR-ABL), is a hallmark of this disorder. Tyrosine kinase inhibitors such as imatinib has shown a great promise in reduction of CML cells. However, development of resistance to tyrosine kinase inhibitors has raised a great clinical concern about their future applications. Recently, non-coding RNAs, have shown to play significant regulatory roles in development of chemoresistance in CML cells. Discovering the underlying mechanisms of these non-coding RNAs might provide new opportunities for treating chemo-resistant forms of CML. These non-coding RNAs could be considered valuable therapeutic targets if they are found to play a role in the development of chemoresistance in CML cells. We mentioned the identified non-coding RNAs in development of chemoresistance in CML cells.
{"title":"Non-coding RNAs: Emerging contributors to chemoresistance in chronic myeloid leukemia","authors":"Laya Ghadyani nejhad , Mahsa Sohani , Nasrin Alizad Ghandforoush , Mohsen Nikbakht , Saeed Mohammadi , Mohammad Vaezi , Shahrbano Rostami , Bahram Chahardouli","doi":"10.1016/j.lrr.2025.100513","DOIUrl":"10.1016/j.lrr.2025.100513","url":null,"abstract":"<div><div>Chronic myeloid leukemia (CML), is a myeloproliferative disease characterized by unregulated growth of blood forming cells in bone marrow and blood. The t(9;22)(q34;q11.2) translocation, which results in the formation of a hyperactive tyrosine kinase (<em>BCR-ABL</em>), is a hallmark of this disorder. Tyrosine kinase inhibitors such as imatinib has shown a great promise in reduction of CML cells. However, development of resistance to tyrosine kinase inhibitors has raised a great clinical concern about their future applications. Recently, non-coding RNAs, have shown to play significant regulatory roles in development of chemoresistance in CML cells. Discovering the underlying mechanisms of these non-coding RNAs might provide new opportunities for treating chemo-resistant forms of CML. These non-coding RNAs could be considered valuable therapeutic targets if they are found to play a role in the development of chemoresistance in CML cells. We mentioned the identified non-coding RNAs in development of chemoresistance in CML cells.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100513"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143947518","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}