[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第3页

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.397

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.143

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[Improvement of extensive epidermolysis due to severe acute graft-versus-host disease through long-term multidisciplinary skin care]. [通过长期多学科皮肤护理改善严重急性移植物抗宿主病引起的广泛表皮松解]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.100

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":"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.","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}

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[Dermatopathic lymphadenopathy in chronic skin graft-versus-host disease]. 慢性皮肤移植物抗宿主病中的皮肤病淋巴结病。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.127

Kana Kato, Takeshi Kobayashi, Tsunekazu Hishima, Kaori Kondo, Yasutaka Sadaga, Chika Kato, Satoshi Sakai, Yasuhiro Kambara, Hiroki Fujiwara, Kou Teshima, Kazuya Asano, Yoshimi Nabe, Kazuya Kurihara, Atsushi Jinguji, Masashi Shimabukuro, Fumihiko Ouchi, Kazuki Inai, Naoki Shingai, Takashi Toya, Hiroaki Shimizu, Yuho Najima, Yoshiki Okuyama, Noriko Doki

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[Diffuse large B-cell lymphoma with central pontine myelinolysis without rapid changes in serum sodium levels]. [弥漫性大b细胞淋巴瘤伴脑桥中央髓鞘溶解，血清钠水平无快速变化]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.30

Aika Kitamura, Makoto Osada, Hisako Kunieda, Yuiko Tsukada, Soya Iwamoto, Koichi Oki, Takahide Kikuchi, Kohei Yamazaki

{"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":"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.","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}

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.207

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[Donor T cell exhaustion and immune tolerance after allogeneic hematopoietic cell transplantation]. [同种异体造血细胞移植后供体T细胞衰竭和免疫耐受]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.339

Hajime Senjo, Daigo Hashimoto

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[Precision diagnosis and genetic counseling for early-onset hereditary thrombosis]. 【早发性遗传性血栓的精准诊断与遗传咨询】。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.348

Shouichi Ohga

{"title":"[Precision diagnosis and genetic counseling for early-onset hereditary thrombosis].","authors":"Shouichi Ohga","doi":"10.11406/rinketsu.66.348","DOIUrl":"https://doi.org/10.11406/rinketsu.66.348","url":null,"abstract":"The rapid global adoption of genomic medicine has recently deepened understanding of the triggers and predisposing factors of thromboembolism. This led to a greater diagnostic significance of heritable thrombophilia, even among infants, children, adolescents, and young adults in Asian countries, whose genetic predisposition had previously received little attention. With the advent of direct oral anticoagulants and replacement agents, the treatment and preventive management of thromboembolism have become a common challenge across all age groups and ethnic backgrounds. In the setting of advanced perinatal and pediatric medicine in Japan, the focus has remained on thrombophilia in patients with early-onset thrombosis (EOT) and clinical guidelines were established in 2024. Heritable thrombophilia is easier to understand when categorized into \"high-frequency and low-risk\" and \"low-frequency and high-risk\" variants of anticoagulant genes. The high penetrance of heritable protein C deficiency and the concurrent thromboembolic events observed in mother-child pairs with EOT during the perinatal period highlight the need for genetic counseling with appropriate treatment and preventive management. This review describes age-dependent diagnosis and management of EOT based on its molecular epidemiology in Japan, and discusses genetic counseling for family members in the precision medicine era.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"348-354"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227929","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}

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[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)]. [基于京都血液学临床研究组（KOTOSG） 15年临床研究的弥漫性大b细胞淋巴瘤的临床问题和观点]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.153

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

{"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":"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.","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}

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[Novel treatment strategies for polycythemia vera: focus on ropeginterferon alfa-2b]. 真性红细胞增多症的新治疗策略：关注ropeg干扰素α -2b。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.258

Keita Kirito

{"title":"[Novel treatment strategies for polycythemia vera: focus on ropeginterferon alfa-2b].","authors":"Keita Kirito","doi":"10.11406/rinketsu.66.258","DOIUrl":"https://doi.org/10.11406/rinketsu.66.258","url":null,"abstract":"The current main treatment goal of polycythemia vera (PV) is preventing thromboembolic and hemorrhagic complications. However, these therapeutic strategies cannot delay progression of PV. Therefore, there is an unmet need for next-generation therapeutic strategies to slow disease progression and improve survival in patients with PV. One of the most promising agents for modifying the disease course of myeloproliferative neoplasms is interferon alpha, which has been used to treat PV since the 1980s. Notably, it achieved cytogenetic or molecular responses in some patients. However, conventional interferon was not widely used due to its high incidence of adverse events and poor tolerance. Ropeginterferon alfa-2b (ropeg) is a unique, site-selective polyethylene glycol-conjugated form of recombinant interferon alpha. A phase 1/2 study of ropeg for PV patients showed low toxicity and improved tolerability, along with significant molecular response. A phase 3 randomized study (PROUD-PV/CONTINUATION-PV) revealed that treatment with ropeg continuously decreased the JAK2V617F allele burden compared to hydroxyurea and improved event-free survival (with events defined as thromboembolic events, disease progression, or death). A phase 2 study in Japanese patients with PV further confirmed the efficacy of ropeg.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 4","pages":"258-266"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144060701","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}

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