[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第9页

[Diagnosis and treatment of iron deficiency anemia]. [缺铁性贫血的诊断和治疗]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.503

Hiroshi Kawabata

{"title":"[Diagnosis and treatment of iron deficiency anemia].","authors":"Hiroshi Kawabata","doi":"10.11406/rinketsu.65.503","DOIUrl":"10.11406/rinketsu.65.503","url":null,"abstract":"The causes of iron deficiency anemia include blood loss, increased demand, insufficient dietary intake, and disorders affecting iron absorption. In certain circumstances, atrophic gastritis, either autoimmune or due to Helicobacter pylori infection, may contribute. On very rare occasions, iron-refractory iron deficiency anemia can develop as a consequence of TMPRSS6 mutations. Iron deficiency anemia is diagnosed by identification of microcytic hypochromic anemia with low serum ferritin levels. In cases of chronic disorders such as chronic kidney disease, chronic heart failure, and chronic inflammatory disorders, the diagnosis may also incorporate transferrin saturation. Treatment of underlying diseases is recommended along with iron supplementation. While oral iron supplements are the first choice, intravenous iron may be considered when oral administration is impractical, iron absorption is impaired, or rapid iron replenishment is necessary. Recently, high-dose intravenous iron formulations became available in Japan, but their use requires caution due to potential risks of allergic reactions, hypophosphatemia/osteomalacia, iron overload, and vascular leakage. Notably, the benefits of high-dose intravenous iron for patients with heart failure and iron deficiency are recognized in the field of cardiology. This article provides an overview, incorporating recent developments in the field of iron deficiency anemia.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 6","pages":"503-513"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499872","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

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.53

引用次数: 0

[Kidney transplantation for end-stage renal disease after third allogeneic hematopoietic stem cell transplantation for Philadelphia chromosome-positive acute lymphoblastic leukemia]. [费城染色体阳性急性淋巴细胞白血病第三次异基因造血干细胞移植后终末期肾病的肾移植]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.7

Akihiko Nishijima, Naoki Shingai, Akihito Ohta, Kiyoko Suda, Kazuya Omoto, Shinya Ishida, Kosuke Yoshioka, Shuhei Kurosawa, Yutaro Hino, Yasushi Senoo, Aiko Igarashi, Gaku Oshikawa, Atsushi Hamamura, Takashi Toya, Hiroaki Shimizu, Yuho Najima, Takeshi Kobayashi, Kyoko Haraguchi, Yoshiki Okuyama, Kazuteru Ohashi, Noriko Doki

{"title":"[Kidney transplantation for end-stage renal disease after third allogeneic hematopoietic stem cell transplantation for Philadelphia chromosome-positive acute lymphoblastic leukemia].","authors":"Akihiko Nishijima, Naoki Shingai, Akihito Ohta, Kiyoko Suda, Kazuya Omoto, Shinya Ishida, Kosuke Yoshioka, Shuhei Kurosawa, Yutaro Hino, Yasushi Senoo, Aiko Igarashi, Gaku Oshikawa, Atsushi Hamamura, Takashi Toya, Hiroaki Shimizu, Yuho Najima, Takeshi Kobayashi, Kyoko Haraguchi, Yoshiki Okuyama, Kazuteru Ohashi, Noriko Doki","doi":"10.11406/rinketsu.65.7","DOIUrl":"10.11406/rinketsu.65.7","url":null,"abstract":"An 18-year-old man underwent allogenic bone marrow transplantation (BMT) for Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ALL). Ph+ALL relapsed 3 months after the first BMT, and the patient underwent a second BMT. However, Ph+ALL relapsed 4 months after the second BMT, and he received a haploidentical peripheral blood stem cell transplantation (haplo-PBSCT) from his father. Molecular complete remission was confirmed 29 days after haplo-PBSCT. However, the patient needed dialysis for end-stage renal disease due to thrombotic microangiopathy 3 years and 2 months after haplo-PBSCT. He received a kidney transplantation from his father 7 years and 10 months after haplo-PBSCT, and got off dialysis after the kidney transplantation. Immunosuppressive therapy with methylprednisolone, tacrolimus, and mycophenolate mofetil was started for kidney transplantation, but the dose of immunosuppressive agents was reduced successfully without rejection soon after kidney transplantation. The patient has maintained long-term remission since the haplo-PBSCT, and his kidney function was restored by the kidney transplantation from his father.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 1","pages":"7-12"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139708720","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

[Role of aberrant RNA splicing in acquired sideroblastic anemia]. [异常 RNA 剪接在获得性红细胞性贫血中的作用]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.222

Tetsuro Ochi

引用次数: 0

[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":"65 2","pages":"84-89"},"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":"65 2","pages":"90-94"},"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":"65 8","pages":"764-768"},"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":"65 8","pages":"747-755"},"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":"65 8","pages":"798-809"},"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

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.819

引用次数: 0