{"title":"Bridging therapy with valemetostat to allogeneic hematopoietic cell transplantation for adult T-cell leukemia/lymphoma.","authors":"Shota Furukawa, Ayumu Ito, Koji Jimbo, Masataka Sakashita, Sakiko Harada, Mamoru Nagata, Takayuki Fujii, Asako Usui, Mizuki Watanabe, Wataru Takeda, Tsuneaki Hirakawa, Kazuaki Yokoyama, Aki Sato, Makoto Yamagishi, Kaoru Uchimaru, Yasuhito Nannya, Takahiro Fukuda","doi":"10.3960/jslrt.25017","DOIUrl":"10.3960/jslrt.25017","url":null,"abstract":"<p><p>Adult T-cell leukemia/lymphoma (ATL) is a peripheral T-cell malignancy caused by human T-cell leukemia virus-1. Patients diagnosed with aggressive ATL have a poor prognosis. As response duration to conventional multiagent chemotherapy is short, upfront allogeneic hematopoietic cell transplantation (allo-HCT) is recommended. As disease status at the time of allo-HCT is the primary prognostic factor, bridging therapy to allo-HCT is sometimes necessary during donor coordination. However, conventional chemotherapies are often toxic, particularly for older patients and those with inadequate hematopoietic recovery. Anti-CCR4 monoclonal antibody mogamulizumab adversely affects post-transplant survival due to the high risk of severe graft-versus-host disease (GVHD). Available data on other novel agents for ATL are limited. The present case study details an ATL patient who exhibited chemotherapy intolerance, but was successfully bridged to unrelated peripheral blood stem cell transplantation (uPBSCT) with valemetostat, a selective dual inhibitor of enhancer of zeste homolog 1 and 2. Despite severe bone marrow failure, valemetostat was well tolerated by the patient and led to successful disease control prior to uPBSCT, which is considered to have contributed to a reduced risk of post-transplant relapse and an improved prognosis. No significant adverse effects of valemetostat were observed early after transplantation, in contrast to the increased incidence of severe GVHD and non-relapse mortality reported following allo-HCT after mogamulizumab therapy. These findings suggest that valemetostat is a promising option as a bridging therapy to allo-HCT for aggressive ATL.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"180-184"},"PeriodicalIF":1.4,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12598706/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144986400","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}
{"title":"Initial disease presentation with myelomatous pleural effusion is an ominous sign for multiple myeloma: report of a case emphasizing cytological features and ancillary studies.","authors":"Jie-Yang Jhuang, Yen-Chuan Hsieh, Yu-Ting Kuo, Nai-Wen Kang, Shih-Sung Chuang","doi":"10.3960/jslrt.25029","DOIUrl":"10.3960/jslrt.25029","url":null,"abstract":"<p><p>Multiple myeloma (MM) is a marrow-based, multi-focal neoplastic proliferation of clonal plasma cells. Myelomatous pleural effusion (MPE) is considered a distinctive form of extramedullary disease and usually develop in disease progression or recurrence, but rarely at initial disease presentation of MM. Herein we report a 47-year-old male patient with MM who presented initially with MPE. The typical cytological features (eccentric nuclei and amphophilic cytoplasm with paranuclear hof), flow cytometric immunophenotyping, and cell block preparation with immunohistochemistry confirmed MPE. After establishing the diagnosis of stage III myeloma through bone marrow study, laboratory and radiological corrections, the patient was treated with chemotherapy and palliative radiotherapy. Unfortunately his disease progressed and he passed away in six months. Our case illustrated the importance of detailed cytological examination with ancillary studies for the challenging diagnosis of an extremely rare initial disease presentation of MM with MPE, which is an ominous sign.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"185-188"},"PeriodicalIF":1.4,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12598721/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144755565","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}
{"title":"Advances in clonality analysis of human T-cell leukemia virus type 1-infected cells: from Southern blotting to high-throughput sequencing.","authors":"Hiroo Katsuya, Yorifumi Satou, Shinya Kimura","doi":"10.3960/jslrt.25035","DOIUrl":"10.3960/jslrt.25035","url":null,"abstract":"<p><p>Human T-cell leukemia virus type 1 (HTLV-1) causes persistent infection and can lead to adult T-cell leukemia-lymphoma (ATL), a hematological malignancy with a poor prognosis. In ATL patients, monoclonal expansion of HTLV-1-infected cells is observed. Each HTLV-1-infected clone harbors a unique proviral integration site. Based on this feature, various methods have been developed to detect and characterize HTLV-1-infected clones. Traditional Southern blotting detects monoclonal proliferation; however, it lacks sensitivity for detecting minor clones. Polymerase chain reaction (PCR)-based approaches, including inverse PCR and linker-mediated PCR, enhance sensitivity and can detect clonal proliferation even in asymptomatic carriers. The advent of high-throughput sequencing has enabled high-resolution quantitative analysis of the clonality of HTLV-1-infected cells based on proviral integration sites. Additionally, HTLV-1 DNA capture-seq allows simultaneous identification of the proviral structure and the degree of clonal abundance of the expanded infected clones. The modified non-restrictive linear amplification-mediated PCR and sequencing is a rapid and cost-effective tool for integration site analysis that is suitable for clinical application. These approaches could help analyze HTLV-1 pathogenesis, monitor clonal dynamics, and improve patient care strategies.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"167-173"},"PeriodicalIF":1.4,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12598731/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144986246","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}
{"title":"Polymerase chain reaction amplification and molecular anatomy of IGH::BCL2 junctions created by ins(14;18)(q32;q21q21).","authors":"Hitoshi Ohno, Fumiyo Maekawa, Hayashida Masahiko, Kayo Takeoka","doi":"10.3960/jslrt.25023","DOIUrl":"10.3960/jslrt.25023","url":null,"abstract":"","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"218-222"},"PeriodicalIF":1.4,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12598722/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144755566","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}
{"title":"Rapidly progressive fatal hepatic failure due to IgM-related AL amyloidosis in Waldenström's macroglobulinemia: an autopsy case report.","authors":"Asuka Shibamiya, Chikako Ohwada, Keisuke Kirito, Van Tai Nguyen, Akihiro Shoji, Akane Tanaka, Emi Togasaki, Reiko Watanabe, Menghan Yang, Yuichiro Hayashi, Takayuki Shiomi, Chiaki Nakaseko","doi":"10.3960/jslrt.25034","DOIUrl":"10.3960/jslrt.25034","url":null,"abstract":"<p><p>IgM-related AL amyloidosis is a rare and distinct clinical entity, often associated with underlying lymphoproliferative disorders such as Waldenström's macroglobulinemia (WM) or lymphoplasmacytic lymphoma (LPL). Unlike non-IgM AL amyloidosis, it exhibits unique organ involvement patterns and generally poorer prognosis. We report a 66-year-old woman diagnosed with WM complicated by systemic IgM-κ AL amyloidosis. She received combination chemotherapy with rituximab and bendamustine (BR), resulting in a reduction of serum IgM levels. Despite the hematologic improvement, her liver dysfunction rapidly progressed, and she died of hepatic failure just two months after diagnosis. Pathological autopsy revealed massive IgM-κ amyloid deposition in the liver and multiple organs, with no residual lymphoma in the bone marrow or lymph nodes. These findings suggest that extensive hepatic amyloid infiltration was already present at diagnosis, and that organ response could not be achieved despite hematologic improvement. This case highlights the aggressive nature of IgM-related AL amyloidosis and the critical importance of early detection, especially when liver dysfunction is observed. Current therapies targeting the underlying clone may not be sufficient in cases with advanced organ involvement, emphasizing the urgent need for novel strategies to facilitate amyloid clearance and protect organ function.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"195-201"},"PeriodicalIF":1.4,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12598709/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144986389","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}
{"title":"Postmarketing surveillance of brentuximab vedotin for previously untreated Hodgkin lymphoma in Japanese patients.","authors":"Kiyoshi Okazuka, Takayuki Shimizu, Ryosuke Tawara, Masako Matsuo, Kayoko Ichihara, Koji Izutsu","doi":"10.3960/jslrt.24078","DOIUrl":"10.3960/jslrt.24078","url":null,"abstract":"<p><p>Febrile neutropenia (FN) poses a life-threatening risk in patients with Hodgkin lymphoma (HL) undergoing treatment with brentuximab vedotin and doxorubicin, vinblastine, and dacarbazine (A+AVD), especially in elderly patients. Although primary prophylaxis with granulocyte-colony stimulating factor (G-CSF) mitigated FN risk in ECHELON-1, limited safety data exist in Japanese patients, especially regarding neutropenia and FN after A+AVD. We assessed the real-world safety of A+AVD as first-line treatment of Japanese patients with HL. This postmarketing surveillance study collected data between November 2018 and December 2022 (study registration: JRCT1080224121; NCT03729609). Data included treatment details, response, and safety (specific focus on grade ≥3 neutropenia/FN). A total of 112 patients with previously untreated HL receiving A+AVD were included in the analysis. Overall, neutropenia incidence was 58.0%, and FN incidence was 27.7%. Two FN-related deaths occurred. Within 14 days after the first dose of A+AVD, neutropenia occurred in 26.5% and 39.7% of patients with and without early G-CSF administration (EGA), respectively, and in 27.5% and 42.6% with and without prophylactic G-CSF administration (PGA), respectively. FN occurred in 26.5% and 19.2% of patients with and without EGA, respectively, and in 17.7% and 24.6% with and without PGA, respectively. Among the 96 patients with image evaluation, excluding those whose dose was not the approved dose, 89.6% achieved a partial response or better. The real-world safety profile of A+AVD aligned with prior findings, emphasizing the importance of PGA to prevent neutropenia and FN.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"84-92"},"PeriodicalIF":1.4,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12351249/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144201313","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}
{"title":"The outcome of patients with peripheral T cell lymphoma treated with doxorubicin-based (CHOP) and pirarubicin-based regimen (THP-COP) regimen: a single institution experience.","authors":"Noriyoshi Iriyama, Katsuhiro Miura, Hiromichi Takahashi, Masaru Nakagawa, Kazuhide Iizuka, Shimon Otake, Takashi Hamada, Takashi Koike, Kazuya Kurihara, Toshihide Endo, Hideki Nakamura","doi":"10.3960/jslrt.25010","DOIUrl":"10.3960/jslrt.25010","url":null,"abstract":"<p><p>This study investigates the outcomes of patients with peripheral T-cell lymphoma (PTCL) treated with a doxorubicin-based regimen (CHOP) and a pirarubicin-based regimen (THP-COP). Newly diagnosed patients with PTCL between 2001 and 2021 were classified by initial treatment, either CHOP or THP-COP regimen. The treatment response, event-free survival (EFS), and overall survival (OS) were assessed. Overall, 65 patients were analyzed, with 41 classified into the CHOP group and 24 into the THP-COP group. Dose-intensified regimen was applied in 22 patients (53.7%) in the CHOP group and 16 (66.7%) in the THP-COP group. When stratified by treatment, the complete response (CR) rates in the CHOP group and the THP-COP group were 66% and 46% (P = 0.273), the 3-year EFS rates were 52.1% and 29.2% (P = 0.0492), and the 3-year OS rates were 72.7% and 48.6% (P = 0.0718), respectively. When stratified by treatment intensity, the CR rates in the dose-intensified group and the conventional dose group were 65% and 50% (P = 0.230), the 3-year EFS rates were 45.9% and 39.6% (P = 0.995), and the 3-year OS rates were 61.1% and 66.4% (P = 0.267), respectively. This study revealed no significant advantage of the THP-COP regimen over the CHOP regimen regarding treatment outcomes for newly diagnosed PTCLs.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"93-100"},"PeriodicalIF":1.4,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12351251/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144201315","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}
{"title":"Successful treatment of smoldering multiple myeloma-associated type I cryoglobulinemia with bortezomib and autologous peripheral blood stem cell transplantation.","authors":"Daiki Hirano, Hiroatsu Iida, Akari Iwakoshi, Akiyo Sunohara, Michita Suzuki, Yasuhiro Suzuki, Nobuhiko Imahashi, Tatsuya Adachi, Hirokazu Nagai","doi":"10.3960/jslrt.25001","DOIUrl":"10.3960/jslrt.25001","url":null,"abstract":"<p><p>A 52-year-old male developed multiple skin ulcers/purpura in the bilateral lower limbs. A detailed examination led to a diagnosis of type I cryoglobulinemia-related skin lesions, with smoldering multiple myeloma (SMM) as a background disease. After 5 years, dermal symptoms became severe. Although there were no multiple myeloma (MM)-related symptoms other than the skin, induction therapy with bortezomib and dexamethasone (BD) was performed. Since dermal symptoms and SMM responded well to treatment, high-dose chemotherapy (melphalan) with autologous peripheral blood stem cell transplantation (ASCT) was subsequently performed. There were no serious treatment-associated adverse events. In addition, a marked response was achieved, and the disease state was stable for ≥24 months. Only a few similar case reports have been reported to date. According to a recent study, when type I cryoglobulinemia becomes symptomatic, the treatment of respective hematological diseases as a background factor and patient-matched individualized treatment should be performed. The present case suggests that it is a feasible, safe and effective treatment strategy to consider the eligibility for high-dose chemotherapy with ASCT and then perform it, even for SMM-associated type I cryoglobulinemia.</p>","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":" ","pages":"121-128"},"PeriodicalIF":1.4,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12351230/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144201314","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}
{"title":"Highlights: Immunotherapy for B-cell lymphoma.","authors":"Kana Miyazaki","doi":"10.3960/jslrt.25084","DOIUrl":"10.3960/jslrt.25084","url":null,"abstract":"","PeriodicalId":520662,"journal":{"name":"Journal of clinical and experimental hematopathology : JCEH","volume":"65 4","pages":"223-224"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12883021/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145829698","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}