Pub Date : 2025-01-01DOI: 10.1016/j.lrr.2024.100494
Donghyun Kim , Grerk Sutamtewagul , Yeonhwa Yu
Prognostication of acute myeloid leukemia (AML) at initial diagnosis relies on identification of pre-determined underlying genetic abnormalities. Nevertheless, the disease course of AML remains highly unpredictable and robust reliable prognostic biomarkers for newly diagnosed AML are lacking. We retrospectively explored two publicly available AML RNA-Seq datasets and found that inferior overall survival was associated with high-FLT3 and low-NPM1 transcript levels (“FLT3high/NPM1low”) compared to low-FLT3 and high-NPM1 transcript levels (“FLT3low/NPM1high”) in adult de novo AML patients, with a hazard ratio for death of at least 2. Transcript level-dependent differential overall survival was independent from the underlying FLT3 or NPM1 genotypes. Our two-gene RNA expression-based de novo AML risk stratification may supplement and fine-tune traditional genetic aberration-based prognostication methods.
{"title":"FLT3 and NPM1 mRNA expression-based risk stratification of de novo acute Myeloid Leukemia","authors":"Donghyun Kim , Grerk Sutamtewagul , Yeonhwa Yu","doi":"10.1016/j.lrr.2024.100494","DOIUrl":"10.1016/j.lrr.2024.100494","url":null,"abstract":"<div><div>Prognostication of acute myeloid leukemia (AML) at initial diagnosis relies on identification of pre-determined underlying genetic abnormalities. Nevertheless, the disease course of AML remains highly unpredictable and robust reliable prognostic biomarkers for newly diagnosed AML are lacking. We retrospectively explored two publicly available AML RNA-Seq datasets and found that inferior overall survival was associated with high-<em>FLT3</em> and low-<em>NPM1</em> transcript levels (“<em>FLT3</em><sup>high</sup>/<em>NPM1</em><sup>low</sup>”) compared to low-<em>FLT3</em> and high-<em>NPM1</em> transcript levels (“<em>FLT3</em><sup>low</sup>/<em>NPM1</em><sup>high</sup>”) in adult <em>de novo</em> AML patients, with a hazard ratio for death of at least 2. Transcript level-dependent differential overall survival was independent from the underlying <em>FLT3</em> or <em>NPM1</em> genotypes. Our two-gene RNA expression-based <em>de novo</em> AML risk stratification may supplement and fine-tune traditional genetic aberration-based prognostication methods.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100494"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11743808/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143013510","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}
Brentuximab vedotin (BV) has demonstrated efficacy against CD30+ peripheral T-cell lymphoma (PTCL). We herein report a case of CD30+ peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) that emerged one month after completing BV, cyclophosphamide, doxorubicin, and prednisone (BV-CHP) therapy for anaplastic large cell lymphoma (ALCL) and responded to retreatment with BV monotherapy. This case suggests that CD30+ PTCL emerging shortly after BV-CHP therapy may respond to retreatment with BV monotherapy, even if the phenotype differs from the initial diagnosis.
{"title":"Retreatment with brentuximab vedotin for discordant peripheral T-cell lymphomas","authors":"Gen Hasegawa , Noriharu Nakagawa , Yoshimichi Ueda , Masahide Yamazaki","doi":"10.1016/j.lrr.2025.100500","DOIUrl":"10.1016/j.lrr.2025.100500","url":null,"abstract":"<div><div>Brentuximab vedotin (BV) has demonstrated efficacy against CD30<sup>+</sup> peripheral T-cell lymphoma (PTCL). We herein report a case of CD30<sup>+</sup> peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) that emerged one month after completing BV, cyclophosphamide, doxorubicin, and prednisone (BV-CHP) therapy for anaplastic large cell lymphoma (ALCL) and responded to retreatment with BV monotherapy. This case suggests that CD30<sup>+</sup> PTCL emerging shortly after BV-CHP therapy may respond to retreatment with BV monotherapy, even if the phenotype differs from the initial diagnosis.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100500"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11786849/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143081026","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 : 2025-01-01DOI: 10.1016/j.lrr.2025.100501
Saja I. AbuGhannam , Celina R. Andonie , Yousef Abu Asbeh , Aliaa’ Khalili
Multiple myeloma is a hematological malignancy that results from the proliferation of abnormal plasma cells, typically invading the bone marrow but occasionally involving other areas of the body. We present a rare case of a 58-year-old male patient who presented with right-sided thoracic wall mass, which was eventually diagnosed through imaging and biopsy as paraskeletal extramedullary plasmacytoma with concurrent multiple myeloma. The patient exhibited symptoms of chest pain and swelling, with radiological features of a large right-sided chest wall mass. The diagnosis shows the fact that radiological presentations are quite nonspecific, often mimicking other malignancies. Such cases, therefore, require further assistance from thoracic surgery and interventional radiology in addition to advanced imaging techniques such as FDG- PET. He was subsequently treated with a three-drug regimen (VTD-Zometa protocol) including velcade, thalidomide, and dexamethasone, considering radiation versus complete surgical exicion due to the size of the mass. This case also supports the idea that clinical diversity exists among multiple myeloma and that considering paraskeletal extramedullary plasmacytoma must be given due importance when dealing with differential diagnosis of chest wall tumors in old age, although extremely rare. Early diagnosis and exclusion of all other possible diagnoses are also critical for the best possible treatment planning and outcome.
{"title":"A rare presentation of multiple myeloma with concurrent paraskeletal extramedullary thoracic plasmacytoma: A case report","authors":"Saja I. AbuGhannam , Celina R. Andonie , Yousef Abu Asbeh , Aliaa’ Khalili","doi":"10.1016/j.lrr.2025.100501","DOIUrl":"10.1016/j.lrr.2025.100501","url":null,"abstract":"<div><div>Multiple myeloma is a hematological malignancy that results from the proliferation of abnormal plasma cells, typically invading the bone marrow but occasionally involving other areas of the body. We present a rare case of a 58-year-old male patient who presented with right-sided thoracic wall mass, which was eventually diagnosed through imaging and biopsy as paraskeletal extramedullary plasmacytoma with concurrent multiple myeloma. The patient exhibited symptoms of chest pain and swelling, with radiological features of a large right-sided chest wall mass. The diagnosis shows the fact that radiological presentations are quite nonspecific, often mimicking other malignancies. Such cases, therefore, require further assistance from thoracic surgery and interventional radiology in addition to advanced imaging techniques such as FDG- PET. He was subsequently treated with a three-drug regimen (VTD-Zometa protocol) including velcade, thalidomide, and dexamethasone, considering radiation versus complete surgical exicion due to the size of the mass. This case also supports the idea that clinical diversity exists among multiple myeloma and that considering paraskeletal extramedullary plasmacytoma must be given due importance when dealing with differential diagnosis of chest wall tumors in old age, although extremely rare. Early diagnosis and exclusion of all other possible diagnoses are also critical for the best possible treatment planning and outcome.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100501"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143453726","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 : 2025-01-01DOI: 10.1016/j.lrr.2024.100497
Kiyotaka Kawauchi , Toshie Ogasawara
Primary effusion lymphoma (PEL)-like lymphoma is a rare variant of PEL that exhibits diverse clinical behaviors, ranging from mild to aggressive disease courses. The clinicopathological features and effective treatments for this type of lymphoma have not been well defined. We found that proteasome inhibitors were effective in inhibiting the growth and survival of OGU1 cells, which were derived from a patient with aggressive PEL-like lymphoma, highlighting the critical role of proteasome activity in the proliferation of PEL-like lymphoma cells. This suggests that proteasome inhibitors, such as bortezomib, could be promising therapeutic options for patients who respond poorly to conventional chemotherapy.
{"title":"Proteasome inhibitors prevent tumor cell proliferation in HHV-8-unrelated PEL-like lymphoma","authors":"Kiyotaka Kawauchi , Toshie Ogasawara","doi":"10.1016/j.lrr.2024.100497","DOIUrl":"10.1016/j.lrr.2024.100497","url":null,"abstract":"<div><div>Primary effusion lymphoma (PEL)-like lymphoma is a rare variant of PEL that exhibits diverse clinical behaviors, ranging from mild to aggressive disease courses. The clinicopathological features and effective treatments for this type of lymphoma have not been well defined. We found that proteasome inhibitors were effective in inhibiting the growth and survival of OGU1 cells, which were derived from a patient with aggressive PEL-like lymphoma, highlighting the critical role of proteasome activity in the proliferation of PEL-like lymphoma cells. This suggests that proteasome inhibitors, such as bortezomib, could be promising therapeutic options for patients who respond poorly to conventional chemotherapy.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100497"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11750559/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024853","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 : 2025-01-01DOI: 10.1016/j.lrr.2025.100504
Saja Asakrah , Kristin K. Deeb , Nikolaos Papadantonakis , George Deeb
Myeloid malignancies following treatment for plasma cell neoplasms (PCN) are infrequent but is a serious complication, often exhibiting complex karyotype and TP53 mutations. Plasma cell myeloma lineage switch to a myeloid malignancy with evident clonal relatedness is seldomly reported. Here, we report a unique case of acute myeloid leukemia with monocytic differentiation that shares clonal features with an antecedent plasma cell myeloma with t(4;14)(FGFR3::IGH). This phenomenon differs from therapy-related myeloid neoplasm arising from an unrelated clone and underscores the need to elucidate the role of mutations in pathways such as MAPK (e.g., BRAF and KRAS) into lineage plasticity.
{"title":"Trans-differentiation of plasma cell neoplasm to acute myeloid leukemia with monocytic features: Case report of divergent phenotype with identical genotype","authors":"Saja Asakrah , Kristin K. Deeb , Nikolaos Papadantonakis , George Deeb","doi":"10.1016/j.lrr.2025.100504","DOIUrl":"10.1016/j.lrr.2025.100504","url":null,"abstract":"<div><div>Myeloid malignancies following treatment for plasma cell neoplasms (PCN) are infrequent but is a serious complication, often exhibiting complex karyotype and <em>TP53</em> mutations. Plasma cell myeloma lineage switch to a myeloid malignancy with evident clonal relatedness is seldomly reported. Here, we report a unique case of acute myeloid leukemia with monocytic differentiation that shares clonal features with an antecedent plasma cell myeloma with t(4;14)(<em>FGFR3::IGH</em>). This phenomenon differs from therapy-related myeloid neoplasm arising from an unrelated clone and underscores the need to elucidate the role of mutations in pathways such as MAPK (e.g., <em>BRAF</em> and <em>KRAS</em>) into lineage plasticity.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100504"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143479979","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 : 2025-01-01DOI: 10.1016/j.lrr.2025.100505
Zhaoyang Hong , Fan Wang
Background
Atypical Chronic Myeloid Leukemia (aCML) is a rare and aggressive myelodysplastic syndrome/myeloproliferative neoplasm. This study aimed to provide a comprehensive understanding of the epidemiology, clinical characteristics, and survival outcomes of aCML patients.
Methods
The study utilized data from the Surveillance, Epidemiology, and End Results (SEER) database from 2001 to 2020. The age-adjusted incidence rate (AIR) of aCML was calculated, and survival outcomes were analyzed using the Kaplan-Meier method and accelerated failure time (AFT) regression analysis.
Results
The AIR of aCML was found to be 0.024 per 100,000 person-years, with the highest rate observed in 2020. The incidence of aCML increased with age and was higher in males. The study cohort predominantly consisted of elderly White individuals, with an average age at diagnosis of 68.2 ± 15.3 years. The median overall survival (OS) and disease-specific survival (DSS) were 1.4 years and 1.7 years, respectively. Older age was independently associated with worse survival outcomes. Notably, treatment delay and chemotherapy did not significantly impact OS or DSS.
Conclusions
This study provides comprehensive insights into the epidemiology, clinical characteristics, and survival outcomes of aCML, highlighting its rarity, aggressive nature, and poor prognosis. Further research is needed to validate these findings and explore novel therapeutic strategies for improving outcomes in this challenging hematologic malignancy.
{"title":"Comprehensive analysis of Atypical chronic myeloid leukemia (aCML): Epidemiology, clinical features, and survival outcomes based on SEER database insights","authors":"Zhaoyang Hong , Fan Wang","doi":"10.1016/j.lrr.2025.100505","DOIUrl":"10.1016/j.lrr.2025.100505","url":null,"abstract":"<div><h3>Background</h3><div>Atypical Chronic Myeloid Leukemia (aCML) is a rare and aggressive myelodysplastic syndrome/myeloproliferative neoplasm. This study aimed to provide a comprehensive understanding of the epidemiology, clinical characteristics, and survival outcomes of aCML patients.</div></div><div><h3>Methods</h3><div>The study utilized data from the Surveillance, Epidemiology, and End Results (SEER) database from 2001 to 2020. The age-adjusted incidence rate (AIR) of aCML was calculated, and survival outcomes were analyzed using the Kaplan-Meier method and accelerated failure time (AFT) regression analysis.</div></div><div><h3>Results</h3><div>The AIR of aCML was found to be 0.024 per 100,000 person-years, with the highest rate observed in 2020. The incidence of aCML increased with age and was higher in males. The study cohort predominantly consisted of elderly White individuals, with an average age at diagnosis of 68.2 ± 15.3 years. The median overall survival (OS) and disease-specific survival (DSS) were 1.4 years and 1.7 years, respectively. Older age was independently associated with worse survival outcomes. Notably, treatment delay and chemotherapy did not significantly impact OS or DSS.</div></div><div><h3>Conclusions</h3><div>This study provides comprehensive insights into the epidemiology, clinical characteristics, and survival outcomes of aCML, highlighting its rarity, aggressive nature, and poor prognosis. Further research is needed to validate these findings and explore novel therapeutic strategies for improving outcomes in this challenging hematologic malignancy.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100505"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143437342","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 : 2025-01-01DOI: 10.1016/j.lrr.2024.100493
XS Bao, DH Gong, KG Zhou, W Huang
Abdominal multiple extramedullary plasmacytoma (EMP) is a rare disease. CD38-negative relapsed/refractory EMP after treatment with daratumumab has never been reported. In 2020, a patient with jaundice was diagnosed with plasmacytoma in another hospital, which progressed one year after receiving multiline therapy. In July 2021, he was admitted to our hospital and showed CD38-pogative plasmacytoma. The patient received 2 cycles of treatment including daratumumab, venetoclax and DCEP chemotherapy and achieved partial remission. However, he developed ascites and eventually died. Our case indicated that multiple EMP has much lower incidence and far worse prognosis than solitary EMP.
{"title":"A rare case of CD38-negative abdominal multiple extramedullary plasmacytoma and literature review","authors":"XS Bao, DH Gong, KG Zhou, W Huang","doi":"10.1016/j.lrr.2024.100493","DOIUrl":"10.1016/j.lrr.2024.100493","url":null,"abstract":"<div><div>Abdominal multiple extramedullary plasmacytoma (EMP) is a rare disease. CD38-negative relapsed/refractory EMP after treatment with daratumumab has never been reported. In 2020, a patient with jaundice was diagnosed with plasmacytoma in another hospital, which progressed one year after receiving multiline therapy. In July 2021, he was admitted to our hospital and showed CD38-pogative plasmacytoma. The patient received 2 cycles of treatment including daratumumab, venetoclax and DCEP chemotherapy and achieved partial remission. However, he developed ascites and eventually died. Our case indicated that multiple EMP has much lower incidence and far worse prognosis than solitary EMP.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100493"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11731487/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984853","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 : 2025-01-01DOI: 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 : 2025-01-01DOI: 10.1016/j.lrr.2024.100498
V Da Silva Constante , H Couvert , A Wolfromm , M Ilzkovitz
We describe the case of a female patient with May-Hegglin syndrome who developed peripheral T-cell lymphoma not otherwise specified. The patient presents with systemic lupus erythematous phenotype and myelofibrosis secondary to T-cell lymphoma. Peripheral T-cell lymphoma not otherwise specified, represents 25 % of all peripheral T-cell lymphoma. Its diagnosis remains challenging due to the polymorphous clinical presentation and pathological heterogeneity. Myelofibrosis associated with malignant lymphomas is rare and peripheral T-cell lymphoma is even rarer. To our knowledge, this is the first case to describe an association between May-Hegglin syndrome and a peripheral T-cell lymphoma.
{"title":"When the mask slips: A peripheral T-cell lymphoma disguised as lupus with myelofibrosis in a patient with May-Hegglin syndrome","authors":"V Da Silva Constante , H Couvert , A Wolfromm , M Ilzkovitz","doi":"10.1016/j.lrr.2024.100498","DOIUrl":"10.1016/j.lrr.2024.100498","url":null,"abstract":"<div><div>We describe the case of a female patient with May-Hegglin syndrome who developed peripheral T-cell lymphoma not otherwise specified. The patient presents with systemic lupus erythematous phenotype and myelofibrosis secondary to T-cell lymphoma. Peripheral T-cell lymphoma not otherwise specified, represents 25 % of all peripheral T-cell lymphoma. Its diagnosis remains challenging due to the polymorphous clinical presentation and pathological heterogeneity. Myelofibrosis associated with malignant lymphomas is rare and peripheral T-cell lymphoma is even rarer. To our knowledge, this is the first case to describe an association between May-Hegglin syndrome and a peripheral T-cell lymphoma.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100498"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11728067/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142979897","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 : 2025-01-01DOI: 10.1016/j.lrr.2024.100495
Abdur Jamil , Zaheer Qureshi , Zain Mary El-amir , Gillian Kupakuwana-Suk , Hamzah Akram , Mohsin Ahmad , Eric Huselton
Objective
This mini-review provides an overview of the current evidence for Revumenib, a first-in-class menin inhibitor, in treating AML with KMT2A rearrangements or NPM1 mutations. This therapy represents a promising advancement by selectively disrupting leukemogenic pathways.
Summary
The clinical promise of Revumenib in genetically defined AML highlights its potential role in shaping the future treatment landscape. This mini-review underscores the need for ongoing trials to define optimal dosing, safety protocols, and combination therapies, with the ultimate goal of establishing Revumenib as a standard of care for high-risk AML subsets.
{"title":"Targeting menin for precision therapy in high-risk acute myeloid leukemia","authors":"Abdur Jamil , Zaheer Qureshi , Zain Mary El-amir , Gillian Kupakuwana-Suk , Hamzah Akram , Mohsin Ahmad , Eric Huselton","doi":"10.1016/j.lrr.2024.100495","DOIUrl":"10.1016/j.lrr.2024.100495","url":null,"abstract":"<div><h3>Objective</h3><div>This mini-review provides an overview of the current evidence for Revumenib, a first-in-class menin inhibitor, in treating AML with KMT2A rearrangements or NPM1 mutations. This therapy represents a promising advancement by selectively disrupting leukemogenic pathways.</div></div><div><h3>Summary</h3><div>The clinical promise of Revumenib in genetically defined AML highlights its potential role in shaping the future treatment landscape. This mini-review underscores the need for ongoing trials to define optimal dosing, safety protocols, and combination therapies, with the ultimate goal of establishing Revumenib as a standard of care for high-risk AML subsets.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100495"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11732178/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142984840","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}
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