{"title":"[Prophylactic efficacy of tixagevimab/cilgavimab in patients with hematological neoplasms: a single-center study].","authors":"Hirofumi Nakano, Shiori Nakashima, Yui Imai, Tomoyuki Uchida, Morihiro Inoue, Masao Hagihara","doi":"10.11406/rinketsu.66.3","DOIUrl":"10.11406/rinketsu.66.3","url":null,"abstract":"<p><p>We retrospectively analyzed the efficacy of tixagevimab/cilgavimab (Tix/Cil) in 142 patients (total of 157 injections) with hematological disorders. Fifteen patients (9.5%) were infected with coronavirus disease (COVID-19), and 3 of these remained infected even after repeated administration of Tix/Cil. Malignant lymphoma and multiple myeloma were the most frequent underlying disorders (frequencies of 18.9% and 17.5%, respectively). Whole genome sequencing of the Omicron variant was performed in 11 patients, and revealed sensitivity to Tix/Cil in only 2. In both cases, the severity of COVID-19 was moderate I or II. Since April 2023, when a Tix/Cil-resistant variant became dominant (frequency >70%), the incidence of breakthrough infections increased from 4% to 35%, and none responded to Tix/Cil. Vaccination, together with daily precautions against infection, is the current approach used to prevent COVID-19, particularly in patients with lymphoid malignancies, because Tix/Cil is no longer effective as prophylaxis.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"3-6"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383987","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.66.271","DOIUrl":"https://doi.org/10.11406/rinketsu.66.271","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 4","pages":"271-293"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144032601","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":"[Diffuse large B-cell lymphoma presenting with anti-MAG/SGPG IgM gammopathy and neurolymphomatosis at onset].","authors":"Masaki Yoshida, Kuniko Takano, Yasuko Mori, Yosuke Kodama, Mizuki Arimatsu, Manami Iwanaga, Masanori Sakata, Kazuki Okuhiro, Shuhei Honda, Yui Moroga, Masao Ogata","doi":"10.11406/rinketsu.66.305","DOIUrl":"https://doi.org/10.11406/rinketsu.66.305","url":null,"abstract":"<p><p>Anti-MAG/SGPG antibody is an IgM antibody against myelin-associated glycoprotein and sulfate-3-glucuronyl paragloboside, which constitute the myelin sheath, and peripheral neuropathy associated with this antibody causes symptoms such as sensory disturbances and ataxia. Anti-MAG/SGPG antibodies are known to be associated with IgM monoclonal gammopathy derived from plasma cell tumors. Chemotherapy with rituximab has been shown to yield neurological improvement in patients with peripheral neuropathy positive for anti-MAG/SGPG antibodies. Here we report a very rare case of diffuse large B-cell lymphoma presenting with neurolymphomatosis along with sensorimotor neuropathy associated with anti-MAG/SGPG antibody-positive IgM monoclonal gammopathy.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"305-311"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227925","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":"[Hemostatic therapy for hemorrhagic cholecystitis in a patient with hemophilia A with inhibitors].","authors":"Yumi Oshima, Makiko Mizuguchi, Yasunobu Okamoto, Kumiko Kagawa, Hironobu Shibata, Yusuke Arakawa, Toshihiro Omoya, Hiroyoshi Watanabe, Shuji Ozaki","doi":"10.11406/rinketsu.66.312","DOIUrl":"https://doi.org/10.11406/rinketsu.66.312","url":null,"abstract":"<p><p>The patient was a 47-year-old man. He was diagnosed with hemophilia A in childhood, and received replacement therapy with FVIII products. However, this became ineffective due to inhibitor production, and he was treated with emicizumab. He developed fever and vomiting, and abdominal CT revealed an enlarged gallbladder and gallstones. Acute cholecystitis was diagnosed, and he was hospitalized and treated conservatively with antibiotics. On the 12th day of illness, the anemia progressed and a high-density area was observed in the gallbladder by CT scan, leading to a diagnosis of hemorrhagic cholecystitis. Since the inhibitor titer was low at 3.6 BU/ml, neutralization therapy with FVIII products was attempted. FVIII activity increased to 155%, and emergency laparoscopic subtotal cholecystectomy was performed successfully. After surgery, FVIII replacement therapy was continued and FVIII activity was maintained, but on the 19th day of illness, it decreased to 16%, and inhibitor levels rose again. Emicizumab is effective in preventing bleeding in hemophilia A with inhibitors, but the risk of bleeding is high if tissue damage leads to severe inflammation or necrosis. Neutralization therapy with FVIII products might be effective in hemorrhagic emergencies and minimally invasive surgery for hemophilia A patients with inhibitors when emicizumab is used to reduce inhibitor levels.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"312-317"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227928","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.66.355","DOIUrl":"https://doi.org/10.11406/rinketsu.66.355","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"355-368"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227920","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.66.134","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":"[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":"[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}