{"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":"https://doi.org/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":"[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":"https://doi.org/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":"[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":"https://doi.org/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":"[Diffuse large B-cell lymphoma with central pontine myelinolysis without rapid changes in serum sodium levels].","authors":"Aika Kitamura, Makoto Osada, Hisako Kunieda, Yuiko Tsukada, Soya Iwamoto, Koichi Oki, Takahide Kikuchi, Kohei Yamazaki","doi":"10.11406/rinketsu.66.30","DOIUrl":"https://doi.org/10.11406/rinketsu.66.30","url":null,"abstract":"<p><p>Central pontine myelinolysis (CPM) typically arises from the rapid correction of hyponatremia. A literature search showed that CPM secondary to malignant lymphoma is rare, with only 11 reported cases. An 80-year-old woman presented with anorexia as her primary symptom and was subsequently diagnosed with diffuse large B-cell lymphoma (DLBCL) following an axillary lymph node biopsy. Shortly after treatment initiation, she experienced convulsions and altered consciousness. Brain MRI revealed a symmetrical lesion in the pons, but gadolinium enhancement was not observed and cerebrospinal fluid cytology showed negative results. Despite the absence of a history of hyponatremia, we diagnosed CPM secondary to malignant lymphoma due to the consistent MRI findings and previous similar case reports. Chemotherapy for DLBCL was continued, leading to gradual improvement of the pontine lesion and the patient's neurological status. Given the rarity of CPM secondary to malignant lymphoma, we present a summary of previously reported cases.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"30-35"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383711","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":"https://doi.org/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}
{"title":"[Clinical issues and perspectives in diffuse large B-cell lymphoma based on 15 years of clinical studies by the Kyoto Hematology Clinical Research Group (KOTOSG)].","authors":"Eri Kawata, Takahiro Fujino, Tsutomu Kobayashi, Haruya Okamoto, Taku Tsukamoto, Shinsuke Mizutani, Yuji Shimura, Daichi Nishiyama, Yuri Kamitsuji, Shin-Ichi Fuchida, Mitsushige Nakao, Ryoichi Takahashi, Hiroto Kaneko, Hitoji Uchiyama, Koichi Hirakawa, Nobuhiko Uoshima, Junya Kuroda","doi":"10.11406/rinketsu.66.153","DOIUrl":"10.11406/rinketsu.66.153","url":null,"abstract":"<p><p>Since 2010, the Kyoto Hematology Clinical Research Group (KOTOSG), a multicenter clinical research group, has dedicated itself to clinical studies on hematological diseases based on daily clinical practice. These studies have provided information about patient characteristics, treatment outcomes, and adverse events in actual clinical practice, and have improved the standard of local medical care by uncovering differences between facilities. In addition, study results have clarified ongoing problems that pose the next challenges for both clinical and basic research. The disease most widely studied was diffuse large B-cell lymphoma (DLBCL). Studies related to DLBCL covered various topics: (1) development of a new prognostic index that can identify highly refractory patients with high accuracy, such as the Kyoto Prognostic Index (KPI) for newly diagnosed DLBCL and KPI-R for relapsed/refractory DLBCL, (2) identification of cytogenetic and clinicopathological characteristics of patients with a poor prognosis, and (3) issues with second cancers after successful treatment. In this article, we review the results of clinical studies on DLBCL by KOTOSG, comparing them with those of past clinical studies in DLBCL, mainly those conducted in Japan. We hope these studies will help to advance the field of clinical hematology.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 3","pages":"153-164"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143774976","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}
{"title":"[Outcome of allogeneic hematopoietic stem cell transplantation for myelofibrosis].","authors":"Takumi Nishikawa, Masuho Saburi, Kuniaki Maehara, Keiichi Uraisami, Hiroyuki Takata, Yasuhiko Miyazaki, Eiichi Ohtsuka","doi":"10.11406/rinketsu.66.111","DOIUrl":"https://doi.org/10.11406/rinketsu.66.111","url":null,"abstract":"<p><p>We retrospectively reviewed outcomes of four patients (median age: 54.5 years) who received allogeneic stem cell transplantation for myelofibrosis at our hospital. Transplantation consisted of unrelated bone marrow transplantation (BMT) (n=1), related peripheral blood stem cell transplantation (PBSCT) (n=1), and haploidentical PBSCT with post-transplant cyclophosphamide (PTCy-haplo) (n=2). The conditioning regimen was fludarabine combined with busulfan 12.8 mg/kg or melphalan 140 mg/m<sup>2</sup>. All patients received a JAK2 inhibitor, which was gradually tapered and then discontinued before conditioning. The three PBSCT recipients achieved engraftment, and are alive in remission. The BMT recipient died of graft failure. PTCy-haplo, myeloablative conditioning, and JAK2 inhibitors may be useful for transplantation in patients with myelofibrosis.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"111-116"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569243","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.66.54","DOIUrl":"https://doi.org/10.11406/rinketsu.66.54","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"54-69"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384255","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}