{"title":"","authors":"","doi":"10.11406/rinketsu.66.134","DOIUrl":"https://doi.org/10.11406/rinketsu.66.134","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"134-135"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569183","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}
{"title":"[Long-term remission achieved with allogeneic hematopoietic stem cell transplantation at first complete remission in a pediatric patient with TCF7::SPI1 fusion-positive T-cell acute lymphoblastic leukemia].","authors":"Mayuko Noguchi, Kai Yamasaki, Sakiko Azuma, Natsumi Kikuchi, Chika Nitani, Keiko Okada, Nobutaka Kiyokawa, Kiyotaka Isobe, Junko Takita, Hiroyuki Fujisaki, Junichi Hara","doi":"10.11406/rinketsu.66.49","DOIUrl":"10.11406/rinketsu.66.49","url":null,"abstract":"<p><p>T-cell acute lymphocytic leukemia (T-ALL) with SPI1 fusion, a leukemia subtype first identified in Japan, has a very poor prognosis. A 7-year-old boy was admitted to our hospital with fever, cervical lymphadenopathy, eyelid edema, and purpura. White blood cell count was markedly increased (551,000/µl). Flow cytometric analysis revealed cyCD3, CD1a, CD8, and HLA-DR positive T-ALL, and fusion gene screening identified TCF7::SPI1 fusion. The patient was treated according to the JPLSG ALL-T11 protocol. He responded poorly to prednisolone, but favorably to L-asparaginase. After completion of early intensification therapy, molecular remission was confirmed. However, due to the patient's poor response to prednisolone, and the presence of the SPI1 fusion gene, hematopoietic stem cell transplantation from an HLA-matched unrelated donor was performed in first remission. So far, the patient has been in remission for 36 months from the time of onset. Hematopoietic stem cell transplantation in first remission may be effective treatment for patients with T-ALL and SPI1 fusion.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"49-53"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383991","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}
{"title":"[Treatment outcomes of axicabtagene ciloleucel for relapsed/refractory diffuse large B-cell lymphoma: a retrospective analysis at a single institution].","authors":"Wataru Kitamura, Nobuharu Fujii, Chihiro Kamoi, Toshiki Terao, Akira Yamamoto, Hiroki Kobayashi, Takumi Kondo, Keisuke Seike, Hideaki Fujiwara, Keiko Fujii, Noboru Asada, Daisuke Ennishi, Yoshinobu Maeda","doi":"10.11406/rinketsu.66.81","DOIUrl":"10.11406/rinketsu.66.81","url":null,"abstract":"<p><p>The advent of anti-CD19 chimeric antigen receptor-T cell therapy has dramatically changed the treatment strategy for relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL). Three products are recently available in Japan, but to the best of our knowledge, real-world data are only available for tisagenlecleucel. This study was a retrospective analysis of 27 patients who received axicabtagene ciloleucel (axi-cel) for R/R DLBCL at our institution. Cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome occurred in 24 (88.9%) and 8 patients (29.6%), respectively, and corticosteroids were used in 19 patients (70.4%). The median follow-up period was 8.1 months (range, 1.0-23.2), and the 6-month progression-free survival and overall survival rates were 80.2% (95% confidence interval [CI], 58.8-91.3) and 92.0% (95%CI, 71.6-97.9), respectively. Although our study was limited by its small sample size and short follow-up period, it demonstrated that axi-cel was highly effective and safe at our institution.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"81-91"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569247","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}
{"title":"[The Utility of Flow Cytometry Profiling Using CD7 and CADM1 in adult T-cell leukemia/lymphoma].","authors":"Koji Jimbo","doi":"10.11406/rinketsu.66.209","DOIUrl":"https://doi.org/10.11406/rinketsu.66.209","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 4","pages":"209-219"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144014191","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}
{"title":"","authors":"","doi":"10.11406/rinketsu.66.397","DOIUrl":"https://doi.org/10.11406/rinketsu.66.397","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"397-398"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227922","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}
{"title":"[Detection of neutralizing antibody against SARS-CoV-2 in a patient with X-linked agammaglobulinemia receiving immunoglobulin replacement therapy].","authors":"Hiroyasu Kaya, Hideki Tani, Masae Itamochi, Kazunori Oishi","doi":"10.11406/rinketsu.66.130","DOIUrl":"10.11406/rinketsu.66.130","url":null,"abstract":"<p><p>Patients with X-linked agammaglobulinemia (XLA) cannot produce antibodies. In this study, we investigated antibodies against SARS-CoV-2 in a man in his 40s with XLA who had no history of COVID-19 or vaccination against COVID-19. Even though his close contact mother contracted SARS-CoV-2, he had never been infected. We measured N-IgG and S-IgG antibodies, as well as neutralizing activity. Serum antibody titers were higher after administration of immunoglobulin, and the antibody titers of immunoglobulin preparations were high as well. Our data revealed that regular administration of immunoglobulins sufficiently increases virus-specific neutralizing antibody activity in patients with XLA.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"130-132"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569203","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}
{"title":"[Rebalancing therapy for congenital hemophilia].","authors":"Kagehiro Amano","doi":"10.11406/rinketsu.66.488","DOIUrl":"10.11406/rinketsu.66.488","url":null,"abstract":"<p><p>Conventional treatments for hemophilia have focused on replacing the missing coagulation factor, but a new treatment concept called rebalancing therapy has recently emerged. Rebalancing therapy corrects bleeding tendency by adjusting the balance between coagulation and anticoagulation, and specifically targets TFPI, AT and APC. The anti-TFPI agents concizumab and marstacimab are administered subcutaneously and have been approved for use in Japan. They are monoclonal antibodies that target the K2 domain of TFPI, and improve hemostatic function by inhibiting the binding of TFPI to FXa and TF/FVIIa complexes. The siRNA drug fitusiran is used as an anti-AT agent that reduces the synthesis of AT, and SerpinPC as an anti-APC agent that specifically inhibits APC. This article will outline the concept of rebalancing therapy and the results of clinical trials, as well as precautions and potential issues during treatment.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"488-494"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577218","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}
{"title":"","authors":"","doi":"10.11406/rinketsu.66.143","DOIUrl":"https://doi.org/10.11406/rinketsu.66.143","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 3","pages":"143"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143774958","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}
Fumihiko Ouchi, Yotaro Motomura, Yasumasa Nakata, Kota Yoshifuji, Nagisa Chubachi, Kumi Kashiwagi, Yuko Momose, Yuri Takimoto, Akiko Uchiyama, Toshikage Nagao, Noriko Uemura, Takehiko Mori
{"title":"[Improvement of extensive epidermolysis due to severe acute graft-versus-host disease through long-term multidisciplinary skin care].","authors":"Fumihiko Ouchi, Yotaro Motomura, Yasumasa Nakata, Kota Yoshifuji, Nagisa Chubachi, Kumi Kashiwagi, Yuko Momose, Yuri Takimoto, Akiko Uchiyama, Toshikage Nagao, Noriko Uemura, Takehiko Mori","doi":"10.11406/rinketsu.66.100","DOIUrl":"10.11406/rinketsu.66.100","url":null,"abstract":"<p><p>A 41-year-old woman with myelodysplastic syndrome underwent unrelated bone marrow transplantation following conditioning with fludarabine, busulfan, total body irradiation, and anti-thymocyte globulin. She received tacrolimus and short-term methotrexate for graft-versus-host disease (GVHD) prophylaxis. After engraftment, she developed acute GVHD involving the skin, gut, and liver. Even after treatment with glucocorticoids, human mesenchymal stem cells, and ruxolitinib, skin GVHD progressed and caused extensive epidermolysis and erosions with persistent bleeding. The patient was started on a daily skin care regimen, which included washing, application of dimethyl isopropylazulene and betamethasone ointment, and topical trafermin and hydrogel wound dressing for bleeding sites. A multi-disciplinary team consisting of hematologists, plastic surgeons, and nurses, and physical therapists, psychiatrists/clinical psychologists, and palliative care providers for physical, mental, and pain supportive care managed the patient's care. After 4 months of treatment under this team, complete epithelial regeneration was achieved. This case demonstrates the efficacy of local skin care and multi-disciplinary collaboration in acute GHVD causing extensive skin damage.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"100-105"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569232","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}