Follicular lymphoma (FL) is the second most common subtype of non-Hodgkin lymphoma (NHL) and comprises 9 % of total NHL in India. Rituximab maintenance (RM) after induction immunochemotherapy improves PFS. However, currently, no data exists from the Indian continent regarding the impact of Rituximab maintenance (RM) on overall survival (OS) & progression-free survival (PFS). Our study aims to assess if RM improves the outcomes in FL patients.
Methods
This retrospective study included 95 patients diagnosed with FL meeting GELF criteria and registered at our institute All India Institute of Medical Sciences Delhi between January 2012 -December 2023. Among these, forty -four (46 %) patients received 2 years of RM every 2 months after induction immunochemotherapy. The primary outcome were overall survival (OS) and progression-free survival. Secondary outcomes were factors affecting OS & PFS.
Results
Out of the 95 patients who met GELF criteria, 52 were male and 43 were female, with a median age of 53 years (range: 27–81). Advanced Ann Arbor stage III/IV comprises 82 (86 %) patients. Bone marrow involvement and bulky disease were observed in 36 (38 %) and 29 (31 %) patients respectively. Based on the Follicular Lymphoma International Prognostic Index (FLIPI-1), 27 (28 %) were low risk, 9 (10 %) intermediate risk, and 59 (62 %) were high risk. The complete remission (CR) was achieved in 71 % of patients. During follow-up, 27 (28 %) patients experienced relapsed and 8 patients transformed to diffuse large B-cell lymphoma (DLBCL). At a median follow-up of 63 months, the median OS was not reached. The median PFS was 122 months in the RM group and 94 months in the non-RM group, with 5-year PFS of 91.6 % vs 59.3 %, respectively (log-rank p = 0.017). Rituximab maintenance was independently associated with improved PFS (adjusted HR 0.28, 95 % CI: 0.12–0.65, p = 0.003). Failure to attain CR (aHR 2.49, 95 % CI: 1.20–5.19, p = 0.015) and bone marrow involvement (aHR 2.92, 95 % CI: 0.99–8.63, p = 0.05) were independently associated with inferior PFS.
Conclusions
Rituximab maintenance after induction immunochemotherapy significantly improved PFS. This is the first study from India demonstrating the impact of rituximab maintenance in FL.
滤泡性淋巴瘤(FL)是第二常见的非霍奇金淋巴瘤(NHL)亚型,占印度NHL总数的9%。诱导免疫化疗后利妥昔单抗维持(RM)改善PFS。然而,目前还没有来自印度大陆的关于利妥昔单抗维持(RM)对总生存期(OS)和无进展生存期(PFS)影响的数据。我们的研究旨在评估RM是否能改善FL患者的预后。方法回顾性研究纳入2012年1月至2023年12月在我们研究所全印度医学科学研究所登记的95例符合GELF标准的FL患者。其中,44例(46%)患者在诱导免疫化疗后每2个月接受2年的化疗。主要终点是总生存期(OS)和无进展生存期。次要结局是影响OS和PFS的因素。结果95例符合GELF标准的患者中,男性52例,女性43例,中位年龄53岁(范围:27-81岁)。晚期安娜堡III/IV期包括82例(86%)患者。骨髓受累36例(38%),大面积病变29例(31%)。根据滤泡性淋巴瘤国际预后指数(FLIPI-1), 27例(28%)为低危,9例(10%)为中危,59例(62%)为高危。71%的患者达到完全缓解(CR)。在随访期间,27例(28%)患者复发,8例转化为弥漫性大b细胞淋巴瘤(DLBCL)。在中位随访63个月时,中位OS未达到。RM组和非RM组的中位PFS分别为122个月和94个月,5年PFS分别为91.6%和59.3% (log-rank p = 0.017)。利妥昔单抗维持与PFS改善独立相关(调整后危险度0.28,95% CI: 0.12-0.65, p = 0.003)。未能达到CR (aHR 2.49, 95% CI: 1.20 ~ 5.19, p = 0.015)和骨髓受累(aHR 2.92, 95% CI: 0.99 ~ 8.63, p = 0.05)与PFS较差独立相关。结论诱导免疫化疗后维持斯利妥昔单抗可显著改善PFS。这是印度第一个证明利妥昔单抗维持对FL影响的研究。
{"title":"Impact of Rituximab Maintenance on outcomes in Follicular Lymphoma: An indian experience","authors":"Harish Pant , Ajay Gogia , Atul Sharma , Naveet Wig , Hari Krishna Raju Sagiraju , Saumyaranjan Mallick , Ritu Gupta , Ahitagni Biswas","doi":"10.1016/j.lrr.2025.100554","DOIUrl":"10.1016/j.lrr.2025.100554","url":null,"abstract":"<div><h3>Background</h3><div>Follicular lymphoma (FL) is the second most common subtype of non-Hodgkin lymphoma (NHL) and comprises 9 % of total NHL in India. Rituximab maintenance (RM) after induction immunochemotherapy improves PFS. However, currently, no data exists from the Indian continent regarding the impact of Rituximab maintenance (RM) on overall survival (OS) & progression-free survival (PFS). Our study aims to assess if RM improves the outcomes in FL patients.</div></div><div><h3>Methods</h3><div>This retrospective study included 95 patients diagnosed with FL meeting GELF criteria and registered at our institute All India Institute of Medical Sciences Delhi between January 2012 -December 2023. Among these, forty -four (46 %) patients received 2 years of RM every 2 months after induction immunochemotherapy. The primary outcome were overall survival (OS) and progression-free survival. Secondary outcomes were factors affecting OS & PFS.</div></div><div><h3>Results</h3><div>Out of the 95 patients who met GELF criteria, 52 were male and 43 were female, with a median age of 53 years (range: 27–81). Advanced Ann Arbor stage III/IV comprises 82 (86 %) patients. Bone marrow involvement and bulky disease were observed in 36 (38 %) and 29 (31 %) patients respectively. Based on the Follicular Lymphoma International Prognostic Index (FLIPI-1), 27 (28 %) were low risk, 9 (10 %) intermediate risk, and 59 (62 %) were high risk. The complete remission (CR) was achieved in 71 % of patients. During follow-up, 27 (28 %) patients experienced relapsed and 8 patients transformed to diffuse large B-cell lymphoma (DLBCL). At a median follow-up of 63 months, the median OS was not reached. The median PFS was 122 months in the RM group and 94 months in the non-RM group, with 5-year PFS of 91.6 % vs 59.3 %, respectively (log-rank <em>p</em> = 0.017). Rituximab maintenance was independently associated with improved PFS (adjusted HR 0.28, 95 % CI: 0.12–0.65, <em>p</em> = 0.003). Failure to attain CR (aHR 2.49, 95 % CI: 1.20–5.19, <em>p</em> = 0.015) and bone marrow involvement (aHR 2.92, 95 % CI: 0.99–8.63, <em>p</em> = 0.05) were independently associated with inferior PFS.</div></div><div><h3>Conclusions</h3><div>Rituximab maintenance after induction immunochemotherapy significantly improved PFS. This is the first study from India demonstrating the impact of rituximab maintenance in FL.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"25 ","pages":"Article 100554"},"PeriodicalIF":0.9,"publicationDate":"2025-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145692159","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 : 2025-11-25DOI: 10.1016/j.lrr.2025.100553
Yanyan Qiu, Huarong Zhou, Shaoyuan Wang
Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia characterized by the promyelocytic leukemia-retinoic acid receptor alpha (PML-RARA) fusion gene and exceptional responsiveness to differentiation therapy with all-trans retinoic acid (ATRA) and arsenic trioxide (Arsenic Trioxide). However, rare variant forms of APL involving alternative RARA fusion partners, such as FIP1L1-RARA, are typically resistant to ATRA/ATO-based regimens and are associated with poor clinical outcomes. We report a rare case of FIP1L1-RARA-positive APL in a patient who initially presented with clinical, morphological, and immunophenotypic features consistent with classical APL. Standard diagnostic evaluations, including morphology, flow cytometry, and fluorescence in situ hybridization (FISH), failed to detect the fusion abnormality. The diagnosis was ultimately confirmed by RNA sequencing (RNA-Seq). Empirical induction with ATRA and ATO was initiated but resulted in persistent and progressive promyelocytosis. Anthracycline-based chemotherapy was subsequently administered, followed by azacitidine combined with venetoclax in the subsequent course of treatment. Although partial hematologic improvement was observed, the overall response remained suboptimal. This case underscores the diagnostic challenges of atypical APL and highlights the pivotal role of RNA-Seq in identifying cryptic RARA rearrangements. A literature review suggests that FIP1L1-RARA-positive APL represents a biologically and clinically distinct entity with highly variable treatment responses and poor prognosis. Early molecular diagnosis and prompt implementation of conventional chemotherapy or targeted therapies such as venetoclax may be essential to improving outcomes in this rare APL subtype.
{"title":"A rare case of variant acute promyelocytic leukemia with FIP1L1-RARA fusion gene: case report and literature review","authors":"Yanyan Qiu, Huarong Zhou, Shaoyuan Wang","doi":"10.1016/j.lrr.2025.100553","DOIUrl":"10.1016/j.lrr.2025.100553","url":null,"abstract":"<div><div>Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia characterized by the promyelocytic leukemia-retinoic acid receptor alpha (<em>PML-RARA</em>) fusion gene and exceptional responsiveness to differentiation therapy with all-trans retinoic acid (ATRA) and arsenic trioxide (Arsenic Trioxide). However, rare variant forms of APL involving alternative <em>RARA</em> fusion partners, such as <em>FIP1L1-RARA</em>, are typically resistant to ATRA/ATO-based regimens and are associated with poor clinical outcomes. We report a rare case of <em>FIP1L1-RARA</em>-positive APL in a patient who initially presented with clinical, morphological, and immunophenotypic features consistent with classical APL. Standard diagnostic evaluations, including morphology, flow cytometry, and fluorescence in situ hybridization (FISH), failed to detect the fusion abnormality. The diagnosis was ultimately confirmed by RNA sequencing (RNA-Seq). Empirical induction with ATRA and ATO was initiated but resulted in persistent and progressive promyelocytosis. Anthracycline-based chemotherapy was subsequently administered, followed by azacitidine combined with venetoclax in the subsequent course of treatment. Although partial hematologic improvement was observed, the overall response remained suboptimal. This case underscores the diagnostic challenges of atypical APL and highlights the pivotal role of RNA-Seq in identifying cryptic <em>RARA</em> rearrangements. A literature review suggests that <em>FIP1L1-RARA</em>-positive APL represents a biologically and clinically distinct entity with highly variable treatment responses and poor prognosis. Early molecular diagnosis and prompt implementation of conventional chemotherapy or targeted therapies such as venetoclax may be essential to improving outcomes in this rare APL subtype.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"25 ","pages":"Article 100553"},"PeriodicalIF":0.9,"publicationDate":"2025-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145625109","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 characterized by aberrant BCR::ABL1 tyrosine kinase activity in hematopoietic stem cells. Although tyrosine kinase inhibitors (TKIs) have revolutionized CML treatment, resistance remains a major clinical challenge. This review provides a comprehensive overview of CML, including its epidemiology, pathophysiology, diagnosis, and treatment, as outlined in the latest WHO consensus classification. Current treatment paradigms and the prospects for treatment-free remission (TFR) are also explored. The primary focus is on elucidating the molecular mechanisms of TKI resistance, emphasizing both well-known pathways such as PI3K/AKT, MAPK, JAK/STAT, and alternative pathways including SRC/AKT. This review stands out by integrating recent discoveries regarding genetic mutations within the BCR::ABL1 gene, alongside other molecular alterations contributing to resistance. By synthesizing this knowledge, it aims to guide clinical practitioners, investigators, and translational researchers in developing innovative strategies to overcome resistance and improve patient outcomes in CML.
{"title":"Mechanisms and signaling pathways of tyrosine kinase inhibitor resistance in chronic myeloid leukemia: A comprehensive review.","authors":"Meriem Lahmouad, Zahrae Rachid, Rawane Bellemrrabet, Jihane Zerrouk, Khan Wen Goh, Abdelhakim Bouyahya, Youssef Aboussalah","doi":"10.1016/j.lrr.2025.100533","DOIUrl":"10.1016/j.lrr.2025.100533","url":null,"abstract":"<p><p>Chronic Myeloid Leukemia (CML) is characterized by aberrant BCR::ABL1 tyrosine kinase activity in hematopoietic stem cells. Although tyrosine kinase inhibitors (TKIs) have revolutionized CML treatment, resistance remains a major clinical challenge. This review provides a comprehensive overview of CML, including its epidemiology, pathophysiology, diagnosis, and treatment, as outlined in the latest WHO consensus classification. Current treatment paradigms and the prospects for treatment-free remission (TFR) are also explored. The primary focus is on elucidating the molecular mechanisms of TKI resistance, emphasizing both well-known pathways such as PI3K/AKT, MAPK, JAK/STAT, and alternative pathways including SRC/AKT. This review stands out by integrating recent discoveries regarding genetic mutations within the BCR::ABL1 gene, alongside other molecular alterations contributing to resistance. By synthesizing this knowledge, it aims to guide clinical practitioners, investigators, and translational researchers in developing innovative strategies to overcome resistance and improve patient outcomes in CML.</p>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"24 ","pages":"100533"},"PeriodicalIF":0.9,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12355124/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144875742","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.2025.100512
Phu Chi Dung , Huynh Duc Vinh Phu , Cao Van Dong , Chau Thuy Ha , Nguyen Thi Thanh Ha , Tran Ngoc Xuan Thy , Le Vu Ha Thanh , Huynh Nghia , Nguyen Tan Binh , Hoang Anh Vu , Phan Thi Xinh , Cao Sy Luan
Background
BCR::ABL1 kinase domain (KD) mutations represent a common cause of resistance to tyrosine kinase inhibitors in chronic myeloid leukemia (CML) patients. The frequency and pattern of KD mutations differ among populations worldwide. However, the characteristics of KD mutations in Vietnamese patients remain unclear.
Methods
A retrospective cohort study of CML patients at Blood Transfusion Hematology Hospital who were resistant to frontline imatinib between Oct 2010 and Oct 2018. Direct sequencing technique was performed to detect KD mutations.
Results
488 imatinib-resistant CML patients were included in our study. The median age of the patients was 39, with the majority (82.1 %) diagnosed with chronic phase at the time of resistance. KD mutations were identified in 173 (35.5 %) patients, with 8 cases involving novel variants. The KD mutations predominantly localized within the P-loop of BCR::ABL1 (36.7 %). G250E was the most common mutation, followed by Y253H, M351T, and M244V. In particular, Y253H, T315I, F359V, F317L, E355G, and Q252H were frequently observed in accelerated phase and blast crisis patients. In addition, M244V, T315I, E459K, E255K, F317L, Q252H and E355G were all observed in primary resistant patients.
Conclusion
The emergence of certain specific mutations may serve as the early indicators of leukemic progression, necessitating prompt intervention for better disease control.
{"title":"Characteristics of BCR::ABL1 kinase domain mutations in Vietnamese chronic myeloid leukemia patients","authors":"Phu Chi Dung , Huynh Duc Vinh Phu , Cao Van Dong , Chau Thuy Ha , Nguyen Thi Thanh Ha , Tran Ngoc Xuan Thy , Le Vu Ha Thanh , Huynh Nghia , Nguyen Tan Binh , Hoang Anh Vu , Phan Thi Xinh , Cao Sy Luan","doi":"10.1016/j.lrr.2025.100512","DOIUrl":"10.1016/j.lrr.2025.100512","url":null,"abstract":"<div><h3>Background</h3><div><em>BCR::ABL1</em> kinase domain (KD) mutations represent a common cause of resistance to tyrosine kinase inhibitors in chronic myeloid leukemia (CML) patients. The frequency and pattern of KD mutations differ among populations worldwide. However, the characteristics of KD mutations in Vietnamese patients remain unclear.</div></div><div><h3>Methods</h3><div>A retrospective cohort study of CML patients at Blood Transfusion Hematology Hospital who were resistant to frontline imatinib between Oct 2010 and Oct 2018. Direct sequencing technique was performed to detect KD mutations.</div></div><div><h3>Results</h3><div>488 imatinib-resistant CML patients were included in our study. The median age of the patients was 39, with the majority (82.1 %) diagnosed with chronic phase at the time of resistance. KD mutations were identified in 173 (35.5 %) patients, with 8 cases involving novel variants. The KD mutations predominantly localized within the P-loop of BCR::ABL1 (36.7 %). G250E was the most common mutation, followed by Y253H, M351T, and M244V. In particular, Y253H, T315I, F359V, F317L, E355G, and Q252H were frequently observed in accelerated phase and blast crisis patients. In addition, M244V, T315I, E459K, E255K, F317L, Q252H and E355G were all observed in primary resistant patients.</div></div><div><h3>Conclusion</h3><div>The emergence of certain specific mutations may serve as the early indicators of leukemic progression, necessitating prompt intervention for better disease control.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100512"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143887379","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 : 2025-01-01DOI: 10.1016/j.lrr.2025.100511
Cecilia Anna Fidanza , Ramona Cassin , Francesca Cavallaro , Giorgio Alberto Croci , Francesca Gaia Rossi Dardanoni , Wilma Barcellini , Francesco Passamonti
We report a rare case of vanishing bile duct syndrome (VBDS) associated with diffuse large B cell lymphoma (DLBCL). VBDS is an uncommon ductopenic disorder associated with various underlying conditions, for which timely treatment is crucial to prevent poor outcomes. Our patient received six cycles of R-CHOP chemotherapy every 21 days with halved doxorubicin and vincristine doses, and prophylactic intrathecal chemotherapy. A complete response of DLBCL and normalization of liver parameters were achieved upon completion of treatment. We conclude that, when VBDS is diagnosed, one can consider to urgently reach lymphoma remission even balancing the dose reduction of hepatotoxic drugs.
{"title":"A case report of vanishing bile duct syndrome and diffuse large b cell lymphoma: An uncommon association","authors":"Cecilia Anna Fidanza , Ramona Cassin , Francesca Cavallaro , Giorgio Alberto Croci , Francesca Gaia Rossi Dardanoni , Wilma Barcellini , Francesco Passamonti","doi":"10.1016/j.lrr.2025.100511","DOIUrl":"10.1016/j.lrr.2025.100511","url":null,"abstract":"<div><div>We report a rare case of vanishing bile duct syndrome (VBDS) associated with diffuse large B cell lymphoma (DLBCL). VBDS is an uncommon ductopenic disorder associated with various underlying conditions, for which timely treatment is crucial to prevent poor outcomes. Our patient received six cycles of R-CHOP chemotherapy every 21 days with halved doxorubicin and vincristine doses, and prophylactic intrathecal chemotherapy. A complete response of DLBCL and normalization of liver parameters were achieved upon completion of treatment. We conclude that, when VBDS is diagnosed, one can consider to urgently reach lymphoma remission even balancing the dose reduction of hepatotoxic drugs.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100511"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143878653","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 : 2025-01-01DOI: 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}
Hypereosinophilic syndrome (HES) was first described in 1968 by Hardy and Anderson. It is a group of rare, multisystemic and heterogeneous pathologies, characterized by significant morbidity and mortality. The occurrence of clonal hypereosinophilic syndrome associated with FIP 1L1-PDGFRA+ is estimated to range between 0.31 and 6.3 cases per million individuals. The organs most commonly impacted are the heart and spleen, with the lungs being the next most affected. Clonal hypereosinophilic syndromes with exclusive pulmonary involvement are exceptional. Due to the rarity of clonal HES, this report aims to not only describe the patient's clinical, biological, and radiological manifestations of clonal HES but also enrich the literature to ameliorate the management of this uncommon syndrome.
we report the case of a patient with past medical history of obstructive bronchopneumopathy who was presented with cough and dyspnea. Investigations revealed peripheral blood hypereosinophilia (between 4000 and 9000/mm3) which lead us to suspect clonal hypereosinophilic syndrome (HES). This diagnosis was confirmed by cytogenetics/fluorescence in situ hybridization (FISH) which demonstrated a positive FIP 1L1-PDGFRA rearrangement. The CTAP confirmed isolated lung involvement with interstitial infiltrate of the subpleural territories of both lung bases and the bronchoalveolar lavage showed eosinophil count elevated at 15%. The patient was treated by imatinib at a dose of 100 mg/day was initiated. The patient follow-up showed a reduction in eosinophils count to 7500/mm3 at two months of treatment. A molecular evaluation is scheduled in 3 months to assess the response to imatinib.
{"title":"Clonal eosinophilia with exclusive pulmonary involvement driven by PDGFRA rearrangement treated with imatinib: A case report","authors":"Zaineb Mlayah, Inés Ben-Rekaya, Inaam Bizid, Nader Slama, Sara Boukhris, Mohamed-Adnene Laatiri","doi":"10.1016/j.lrr.2025.100502","DOIUrl":"10.1016/j.lrr.2025.100502","url":null,"abstract":"<div><div>Hypereosinophilic syndrome (HES) was first described in 1968 by Hardy and Anderson. It is a group of rare, multisystemic and heterogeneous pathologies, characterized by significant morbidity and mortality. The occurrence of clonal hypereosinophilic syndrome associated with FIP 1L1-PDGFRA+ is estimated to range between 0.31 and 6.3 cases per million individuals. The organs most commonly impacted are the heart and spleen, with the lungs being the next most affected. Clonal hypereosinophilic syndromes with exclusive pulmonary involvement are exceptional. Due to the rarity of clonal HES, this report aims to not only describe the patient's clinical, biological, and radiological manifestations of clonal HES but also enrich the literature to ameliorate the management of this uncommon syndrome.</div><div>we report the case of a patient with past medical history of obstructive bronchopneumopathy who was presented with cough and dyspnea. Investigations revealed peripheral blood hypereosinophilia (between 4000 and 9000/mm3) which lead us to suspect clonal hypereosinophilic syndrome (HES). This diagnosis was confirmed by cytogenetics/fluorescence in situ hybridization (FISH) which demonstrated a positive FIP 1L1-PDGFRA rearrangement. The CTAP confirmed isolated lung involvement with interstitial infiltrate of the subpleural territories of both lung bases and the bronchoalveolar lavage showed eosinophil count elevated at 15%. The patient was treated by imatinib at a dose of 100 mg/day was initiated. The patient follow-up showed a reduction in eosinophils count to 7500/mm3 at two months of treatment. A molecular evaluation is scheduled in 3 months to assess the response to imatinib.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"23 ","pages":"Article 100502"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143550306","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.2024.100494
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.2025.100519
Magdalena Witkowska , Mikołaj Malicki , Weronika Marcinkowska , Kacper Kościelny , Adrianna Kowalik , Damian Mikulski , Grzegorz Mirocha
With the introduction of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) and radiation combined, the prognosis of Hodgkin lymphoma (HL) has significantly improved, with 5-year overall survival of around 90 %. While the lymphoma has become highly curable, the side effects of ABVD treatment are dire and warrant continuous review. Immune checkpoint inhibitors, including nivolumab, have demonstrated high therapeutic efficacy in relapsed and refractory HL patients. Nevertheless, despite much data, the therapy duration and long-term efficacy question remains unresolved. In this retrospective study, in a cohort of 10 patients, we observed a high complete response (CR) rate, while during long-term observation, 5 patients relapsed, and 3 had autoimmune treatment-related complications.
{"title":"Nivolumab for relapsed and refractory classical Hodgkin lymphoma: retrospective single center analysis","authors":"Magdalena Witkowska , Mikołaj Malicki , Weronika Marcinkowska , Kacper Kościelny , Adrianna Kowalik , Damian Mikulski , Grzegorz Mirocha","doi":"10.1016/j.lrr.2025.100519","DOIUrl":"10.1016/j.lrr.2025.100519","url":null,"abstract":"<div><div>With the introduction of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) and radiation combined, the prognosis of Hodgkin lymphoma (HL) has significantly improved, with 5-year overall survival of around 90 %. While the lymphoma has become highly curable, the side effects of ABVD treatment are dire and warrant continuous review. Immune checkpoint inhibitors, including nivolumab, have demonstrated high therapeutic efficacy in relapsed and refractory HL patients. Nevertheless, despite much data, the therapy duration and long-term efficacy question remains unresolved. In this retrospective study, in a cohort of 10 patients, we observed a high complete response (CR) rate, while during long-term observation, 5 patients relapsed, and 3 had autoimmune treatment-related complications.</div></div>","PeriodicalId":38435,"journal":{"name":"Leukemia Research Reports","volume":"24 ","pages":"Article 100519"},"PeriodicalIF":0.7,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144481226","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}