{"title":"[Diagnosis and treatment of ITP].","authors":"Jun Yamanouchi","doi":"10.11406/rinketsu.66.1132","DOIUrl":"https://doi.org/10.11406/rinketsu.66.1132","url":null,"abstract":"<p><p>Immune thrombocytopenia (ITP) is defined as acquired thrombocytopenia with a platelet count of less than 100,000/ml and is caused by increased platelet destruction and decreased platelet production by immune mechanisms. ITP is essentially a diagnosis of exclusion, made when other thrombocytopenic diseases are ruled out. The goal of ITP treatment is not to return platelet counts to normal, but to improve bleeding symptoms and prevent serious bleeding. Indications for treatment should be based on platelet counts, severity of bleeding symptoms, patient characteristics, and lifestyle.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 9","pages":"1132-1136"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208657","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 significance of irregular antibodies].","authors":"Naomi Shimizu","doi":"10.11406/rinketsu.66.949","DOIUrl":"https://doi.org/10.11406/rinketsu.66.949","url":null,"abstract":"<p><p>Antibodies directed against red blood cell antigens other than those of the ABO system are referred to as irregular antibodies. Among these, IgG-type immune antibodies that are active at 37°C are considered to have high clinical significance. Irregular antibodies are typically detected using the indirect anti-globulin test (IAT), which offers high sensitivity for IgG detection. Although detection of irregular antibodies in the IAT is considered clinically significant, not all IAT-positive antibodies cause hemolytic transfusion reactions (HTR) or hemolytic disease of the fetus and newborn (HDFN). IgG is classified into four subclasses-IgG1 through IgG4. In this study, the characteristics of irregular antibodies were analyzed according to their subclasses using flow cytometry, and a monocyte phagocytosis assay was performed. The results revealed that both the IgG3-only and the IgG1+IgG3 combination types were strongly associated with adverse hemolytic effects and had high clinical relevance. Furthermore, in cases of HDFN, higher levels of IgG3 on fetal red blood cells correlated with increased phagocytosis rates and more severe disease progression. These findings suggest that IgG subclass analysis may serve as an important indicator for predicting the severity of HTR and HDFN.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 9","pages":"949-958"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208659","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 development of genetically modified T cell therapies].","authors":"Keisuke Watanabe","doi":"10.11406/rinketsu.66.1233","DOIUrl":"https://doi.org/10.11406/rinketsu.66.1233","url":null,"abstract":"<p><p>Genetically modified T cell therapies using a chimeric antigen receptor (CAR) or a modified T cell receptor (TCR) have emerged as an attractive approach to cancer treatment, especially for hematological malignancies, and are becoming an indispensable therapeutic option in clinical practice. CAR- and TCR-T cell development continues to face many challenges, including relapsed/refractory disease, adverse events, manufacturing failures, and cost. Nevertheless, several significant achievements have been made, including favorable clinical responses in CAR-T cell trials and the first approval of a TCR-T cell product for synovial sarcoma by the FDA. This review article will describe the current state and future outlook of gene modified T cell therapy development, and will briefly discuss the drug discovery ecosystem for cell therapy platforms, with a particular focus on Japan.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 9","pages":"1233-1240"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208633","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}
{"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":"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}
{"title":"","authors":"","doi":"10.11406/rinketsu.66.207","DOIUrl":"https://doi.org/10.11406/rinketsu.66.207","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 4","pages":"207"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144065097","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":"[Donor T cell exhaustion and immune tolerance after allogeneic hematopoietic cell transplantation].","authors":"Hajime Senjo, Daigo Hashimoto","doi":"10.11406/rinketsu.66.339","DOIUrl":"https://doi.org/10.11406/rinketsu.66.339","url":null,"abstract":"<p><p>Allogeneic hematopoietic cell transplantation (allo-HCT) is a curative treatment for hematologic malignancies. However, graft-versus-host disease (GVHD) and disease relapse negatively impact prognosis. Chronic GVHD significantly affects both prognosis and quality of life, making its prevention crucial. The current standard GVHD prophylaxis, using calcineurin inhibitors (CNIs) and methotrexate (MTX), is effective against acute GVHD but its effect on chronic GVHD is limited. The mechanism by which CNIs fail to prevent chronic GVHD has long been unclear. Recently, we conducted single-cell RNA sequencing of donor T cells after mouse HCT and found that CNIs inhibit donor T-cell exhaustion while inducing effector-like exhausted T cells. These cells contribute to the development of chronic GVHD after allo-HCT and play a role in enhancing graft-versus-leukemia (GVL) effects after post-HCT PD-1 blockade. We are currently accumulating and analyzing patient samples to validate our findings from the mouse HCT models.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"339-347"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227926","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}