[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第8页

[Ruptured mycotic cerebral aneurysm in an adult T-cell leukemia/lymphoma patient undergoing allogeneic stem cell transplantation]. [一名接受同种异体干细胞移植的成人 T 细胞白血病/淋巴瘤患者的霉菌性脑动脉瘤破裂]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.84

Satoshi Koi, Hiroaki Shimizu, Yasutaka Sadaga, Kaori Kondo, Chika Kato, Satoshi Sakai, Yasuhiro Kambara, Ryosuke Konuma, Yuya Atsuta, Masashi Shimabukuro, Atsushi Jinguji, Yuzuru Hosoda, Daishi Onai, Atsushi Hamamura, Naoki Shingai, Takashi Toya, Yuho Najima, Takeshi Kobayashi, Yuichi Matsuzawa, Hideo Arai, Noritaka Sekiya, Kyoko Haraguchi, Yoshiki Okuyama, Noriko Doki

{"title":"[Ruptured mycotic cerebral aneurysm in an adult T-cell leukemia/lymphoma patient undergoing allogeneic stem cell transplantation].","authors":"Satoshi Koi, Hiroaki Shimizu, Yasutaka Sadaga, Kaori Kondo, Chika Kato, Satoshi Sakai, Yasuhiro Kambara, Ryosuke Konuma, Yuya Atsuta, Masashi Shimabukuro, Atsushi Jinguji, Yuzuru Hosoda, Daishi Onai, Atsushi Hamamura, Naoki Shingai, Takashi Toya, Yuho Najima, Takeshi Kobayashi, Yuichi Matsuzawa, Hideo Arai, Noritaka Sekiya, Kyoko Haraguchi, Yoshiki Okuyama, Noriko Doki","doi":"10.11406/rinketsu.65.84","DOIUrl":"10.11406/rinketsu.65.84","url":null,"abstract":"A 63-year-old man with adult T-cell leukemia-lymphoma underwent allogeneic bone marrow transplantation from an HLA-matched unrelated donor. On day 17 after transplantation, chest computed tomography (CT) showed nodules in the lower lobes of both lungs, and invasive pulmonary aspergillosis (IPA) was suspected. Treatment with liposomal amphotericin B was started, and improvement of infectious lesions was confirmed with CT on day 28. The antifungal agent was changed to voriconazole on day 52 because of progressive renal dysfunction. Disorders of consciousness and paralysis of the left upper and lower extremities developed on day 61. Brain CT showed subcortical hemorrhage in the right parietal and occipital lobes, and the patient died on day 62. An autopsy revealed filamentous fungi, suspected to be Aspergillus, in the pulmonary nodules and a ruptured cerebral aneurysm. Although IPA occurs in 10% of transplant recipients, vigilant monitoring for mycotic cerebral aneurysms is required to prevent hematogenous dissemination of Aspergillus, which is associated with a high mortality rate.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140051218","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}

引用次数: 0

[Successful immunosuppressive therapy in female hemophilia A developing inhibitor after perioperative administration of factor VIII products]. [女性 A 型血友病患者在围手术期使用 VIII 因子产品后出现抑制因子，成功接受免疫抑制治疗]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.90

Maki Yamaguchi, Yusuke Takaki, Yoshitaka Yamasaki, Shuki Oya, Takayuki Nakamura, Satoshi Morishige, Kazutoshi Aoyama, Fumihiko Mouri, Ryuta Takase, Yoko Matsuo, Koichi Osaki, Koji Nagafuji, Takashi Okamura

{"title":"[Successful immunosuppressive therapy in female hemophilia A developing inhibitor after perioperative administration of factor VIII products].","authors":"Maki Yamaguchi, Yusuke Takaki, Yoshitaka Yamasaki, Shuki Oya, Takayuki Nakamura, Satoshi Morishige, Kazutoshi Aoyama, Fumihiko Mouri, Ryuta Takase, Yoko Matsuo, Koichi Osaki, Koji Nagafuji, Takashi Okamura","doi":"10.11406/rinketsu.65.90","DOIUrl":"10.11406/rinketsu.65.90","url":null,"abstract":"A 62-year-old woman was diagnosed as a hemophilia A carrier (factor VIII activity 35%) on preoperative examination of an ovarian tumor. A total of 35,600 units of recombinant factor VIII products was administered perioperatively. On postoperative day 95, a subcutaneous hematoma formed and immunosuppressive therapy with prednisolone was started based on an APTT of 66 seconds, factor VIII (FVIII) activity of 3%, and FVIII inhibitor of 1 BU/ml. During this treatment, the patient was hospitalized due to ankle joint bleeds and required hemostatic treatment, but the inhibitor disappeared and FVIII activity recovered to 30% after postoperative day 438 with cyclophosphamide. F8 analysis revealed the patient carried a heterozygosity of p.Arg391Cys, which has previously been categorized as cross-reacting material (CRM)-positive severe hemophilia A. No high-risk mutations for inhibitor development were found. We also report the results of a desmopressin acetate hydrate test administered to the patient to prepare for future treatment in case of hemorrhage, since high-dose FVIII administration may have been a factor in inhibitor development.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140051220","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}

引用次数: 0

[Building a future society that helps female scientists and physicians thrive]. [建设未来社会，帮助女科学家和女医生茁壮成长]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.764

Miyoko O Watanabe

{"title":"[Building a future society that helps female scientists and physicians thrive].","authors":"Miyoko O Watanabe","doi":"10.11406/rinketsu.65.764","DOIUrl":"https://doi.org/10.11406/rinketsu.65.764","url":null,"abstract":"Japan is now at a major historical turning point with its shrinking population. While conventional wisdom is no longer applicable, we have an excellent opportunity for a new social transformation. Promoting diversity is an effective way to achieve this, but this requires recognition of the intergenerational gap in diversity. The medical community has its own unique problems, but also broader problems shared with other fields. In medicine specifically, there is an urgent need to improve the working environment of physicians and realize sustainable medicine and patient-centered care. To solve these problems, reforms are needed that encompass not only the medical community, but also the entire citizenry as patients. More broadly, the main issue is the promotion and development of women leaders. We must refer to examples in Europe and the United States, and implement reforms in work styles. The medical community, which solves the essential problem of maintaining and improving human health, is well positioned to create a new society that coexists with artificial intelligence based on scientific evidence to ensure a bright future beyond this historical turning point.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142134698","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}

引用次数: 0

[Developments of high-throughput sequencing-based diagnosis of congenital thrombocytopenia/platelet disorders in a registry study]. [一项登记研究中基于高通量测序诊断先天性血小板减少症/血小板疾病的进展]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.747

Akira Ishiguro, Toru Uchiyama, Atsushi Sakamoto, Shinji Kunishima

{"title":"[Developments of high-throughput sequencing-based diagnosis of congenital thrombocytopenia/platelet disorders in a registry study].","authors":"Akira Ishiguro, Toru Uchiyama, Atsushi Sakamoto, Shinji Kunishima","doi":"10.11406/rinketsu.65.747","DOIUrl":"https://doi.org/10.11406/rinketsu.65.747","url":null,"abstract":"Congenital thrombocytopenia/platelet disorders are heterogeneous disorders of platelet number and/or function. Pathogenic variants in the genes implicated in megakaryocyte differentiation and platelet formation cause thrombocytopenia in these patients. Recent advances have elucidated several causative genes for these disorders, but identifying the underlying causative genes remains challenging. Patients with these disorders often receive inappropriate treatments, including glucocorticoids and splenectomy, for chronic immune thrombocytopenia (ITP). In Japan, we have developed a diagnostic system using high-throughput DNA sequencing with a multigene panel and established a registry. Between 2018 and 2023, 245 patients were enrolled and analyzed. Pathogenic variants in 17 genes (42 MYH9, 19 ANKRD26, 17 ITGA2B/ITGB3, 8 ACTN1, 8 WAS, 6 ETV6, 6 VWF, 5 CYCS, and 14 others) were identified in 125 patients (51.0%). An additional 29 patients (11.8%) had suspected pathogenic variants under investigation. We also found that immature platelet fraction (IPF%) is useful in the differential diagnosis because the median IPF% in MYH9 disorders, 48.7%, was significantly higher than in all other groups (chronic ITP, 13.4%; controls, 2.6%). The results of this study provide new insight into congenital thrombocytopenia/platelet disorders.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142134703","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}

引用次数: 0

[JAK inhibitors for myelofibrosis: ruxolitinib and momelotinib]. [治疗骨髓纤维化的 JAK 抑制剂：鲁索利替尼和莫美罗替尼]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.798

Hiroshi Kosugi

{"title":"[JAK inhibitors for myelofibrosis: ruxolitinib and momelotinib].","authors":"Hiroshi Kosugi","doi":"10.11406/rinketsu.65.798","DOIUrl":"https://doi.org/10.11406/rinketsu.65.798","url":null,"abstract":"Myelofibrosis should be diagnosed according to the WHO classification (2022, 5th Ed.) and International Consensus Conference 2022 criteria. Testing for driver mutations in the three genes JAK2, CALR, and MPL is recommended to ensure a definitive diagnosis. Ruxolitinib is the only JAK inhibitor currently approved in Japan, but momelotinib is under regulatory review. The MOMENTUM study showed similar spleen volume reduction at 24 weeks and MFSAF-TSS reduction as the COMFORT study of ruxolitinib. Momelotinib acts on ACVR1 and, therefore, improves anemia through suppression of hepcidin. Anemia and/or transfusion dependency are known to be associated with overall survival duration. Consequently, supportive care measures such as ESA and danazol in lieu of transfusion should be considered in addition to JAK inhibitor selection. Mean survival after discontinuation of JAK inhibitors is 11 to 14 months. Pacritinib (not approved in Japan) is suitable for MF patients with thrombocytopenia. JAK inhibitor selection and supportive care by ESA or danazol in lieu of transfusion should be considered. Many classes of drugs other than JAK inhibitors for myelofibrosis are under investigation.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142134706","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}

引用次数: 0

[Successful bridging therapy with alectinib prior to allogeneic stem cell transplantation for refractory ALK-positive anaplastic large cell lymphoma]. [ALK阳性难治性无性大细胞淋巴瘤异基因干细胞移植前使用阿来替尼的桥接疗法获得成功]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.158

Asuka Kono, Keisuke Tanaka, Tomohito Shimada, Kana Bando, Atsushi Takahata, Satoshi Koi, Masahide Yamamoto, Takehiko Mori, Shigeo Toyota

{"title":"[Successful bridging therapy with alectinib prior to allogeneic stem cell transplantation for refractory ALK-positive anaplastic large cell lymphoma].","authors":"Asuka Kono, Keisuke Tanaka, Tomohito Shimada, Kana Bando, Atsushi Takahata, Satoshi Koi, Masahide Yamamoto, Takehiko Mori, Shigeo Toyota","doi":"10.11406/rinketsu.65.158","DOIUrl":"https://doi.org/10.11406/rinketsu.65.158","url":null,"abstract":"Although alectinib is effective for relapsed or refractory ALK-positive anaplastic large cell lymphoma (ALCL) and has a favorable safety profile, its role as a bridging therapy for allogeneic hematopoietic stem cell transplantation (allo-HSCT) and the role of allo-HSCT itself in this setting are unknown. A 35-year-old man with ALK-positive ALCL experienced relapse after first-line therapy with CHOP. Brentuximab vedotin led to partial response and high-dose chemotherapy combined with autologous HSCT was performed. However, disease progressed 15 months after transplantation, and alectinib was initiated. Complete response (CR) was achieved after three months of treatment, and alectinib was continued for 5 months. After cessation of alectinib, allogeneic bone marrow transplantation from an HLA 1-locus mismatched unrelated donor was performed after conditioning with fludarabine, busulfan, and total body irradiation. GVHD prophylaxis consisted of tacrolimus and short-term methotrexate. The post-transplant course was unremarkable except for grade I acute GVHD. The lymphoma has not recurred for 2 years after allo-HSCT without resuming alectinib. The clinical course of our case suggests that alectinib bridging therapy and allo-HSCT are effective in relapsed/refractory ALK-positive ALCL.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140869682","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}

引用次数: 0

[Evidence of GVHD/GVL in allogeneic hematopoietic stem cell transplantation from sex-mismatched donors]. [性别不匹配供体的异体造血干细胞移植中 GVHD/GVL 的证据]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.265

Hideki Nakasone

{"title":"[Evidence of GVHD/GVL in allogeneic hematopoietic stem cell transplantation from sex-mismatched donors].","authors":"Hideki Nakasone","doi":"10.11406/rinketsu.65.265","DOIUrl":"https://doi.org/10.11406/rinketsu.65.265","url":null,"abstract":"Hematopoietic cell transplantation (HCT) is considered a curative treatment for hematological malignancies. However, HCT recipients often face complications such as graft-versus-host disease (GVHD) and disease relapse. Clinical factors like age and HLA disparity are recognized as risks for GVHD. Notably, sex-mismatched HCT, particularly with female donors and male recipients (F→M), is reported to increase the risk of chronic GVHD. This adverse effect of F→M HCT is thought to result from allogeneic immune response against minor histocompatibility antigens encoded on the Y-chromosome of a male recipient (HY-antigens). Indeed, antibodies against HY-antigens (HY-Abs) were detected three months after F→M HCT, and the cumulative number of HY-Abs was significantly associated with increased risks of chronic GVHD and non-relapse mortality. This review focuses on F→M HCT, shedding light on its impact in several clinical settings and presenting clinical evidence of its allogeneic response, encompassing GVHD and graft-versus-leukemia (GVL) effects. Additionally, potential clinical options to mitigate adverse effects in F→M HCT will be discussed. Further investigation is required to improve clinical outcomes and understand allogenic immunological reconstitution after F→M HCT.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140856865","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}

引用次数: 0

[Clinical and genetic features of MDS associated with VEXAS syndrome]. [与 VEXAS 综合征相关的 MDS 的临床和遗传特征]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.255

Hiroyoshi Kunimoto

引用次数: 0

[Recent advances in bone marrow pathology for myeloproliferative neoplasms diagnosis]. [骨髓病理学在骨髓增生性肿瘤诊断中的最新进展]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.778

Masafumi Ito

引用次数: 0

[Treatment strategies for low-risk polycythemia vera]. [低风险红细胞增多症的治疗策略]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.810

Shuichi Ota

引用次数: 0