{"title":"","authors":"","doi":"10.11406/rinketsu.67.70","DOIUrl":"https://doi.org/10.11406/rinketsu.67.70","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 1","pages":"70-71"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146101039","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":"[Gastric intramural hematoma during treatment with aspirin and anagrelide in a patient with essential thrombocythemia].","authors":"Atsuhiro Takagi, Satoru Mukai, Akihiro Umakoshi, Junpei Rikitake, Ritsuko Nakai, Masahisa Nakamura, Kazuo Hatanaka","doi":"10.11406/rinketsu.67.238","DOIUrl":"https://doi.org/10.11406/rinketsu.67.238","url":null,"abstract":"<p><p>A 62-year-old man was diagnosed with JAK2 mutation positive essential thrombocythemia (ET) after developing cerebral infarction. He was treated with anagrelide and aspirin, with anagrelide increased to 5.5 mg/day, but platelet count remained around 700,000/µl. In July 2024, he presented to the emergency department with abdominal pain and vomiting. CT angiography showed a large gastric intramural hematoma as well as pseudoaneurysm from the right gastroepiploic artery. Following transcatheter arterial embolization, his symptoms improved, the hematoma decreased in size, and his condition stabilized. Gastric intramural hematoma is an extremely rare condition, often secondary to factors like anticoagulant therapy. Reports of cases diagnosed after resection of a suspected tumor highlight the importance of accurate diagnosis. Although thrombosis prevention is crucial in ET, anagrelide is associated with a higher risk of bleeding events than other therapies. To our knowledge, this is the first reported case of gastric intramural hematoma associated with ET and anagrelide.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 3","pages":"238-243"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147576929","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":"[Sustained hematologic complete response after discontinuation of ropeginterferon alfa-2b in patients with polycythemia vera].","authors":"Masaki Maruta, Kento Mori, Koji Oka, Yuya Masuda, Junichi Kato, Tatsuya Konishi, Shogo Nabe, Yukihiro Miyazaki, Toshiki Ochi, Jun Yamanouchi, Katsuto Takenaka","doi":"10.11406/rinketsu.67.205","DOIUrl":"https://doi.org/10.11406/rinketsu.67.205","url":null,"abstract":"<p><p>We report two cases of polycythemia vera (PV) treated with ropeginterferon alfa-2b (ropeg-IFN) in which hematologic control was maintained without therapy after treatment discontinuation. The patients achieved complete response (CR) at 14 and 22 weeks after treatment initiation, respectively. After discontinuation of ropeg-IFN, Patient 1 has maintained hematocrit (Ht) <45% for 3 years and 3 months, while patient 2 has maintained CR for 1 year and 5 months. Clinical trials have demonstrated that ropeg-IFN reduces the JAK2 V617F allele burden, which is recognized as an independent risk factor for disease progression and thrombosis, apart from traditional factors such as age and prior cardiovascular events. To improve the prognosis of PV, it is essential to accumulate clinical evidence on trends in JAK2 V617F allele burden and the conditions required to maintain hematologic control after discontinuation of ropeg-IFN therapy in real-world settings.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 3","pages":"205-208"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147576156","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":"[Sustained molecular response after discontinuation of ropeginterferon alfa-2b in a young patient with polycythemia vera].","authors":"Daigo Akahane, Meiko Kuriyama, Kai Osone, Yuya Arai, Shohei Wakamatsu, Shunsuke Ohtuki, Mitsuru Moriyama, Michiyo Asano, Seiichiro Katagiri, Seiichiro Yoshizawa","doi":"10.11406/rinketsu.67.209","DOIUrl":"https://doi.org/10.11406/rinketsu.67.209","url":null,"abstract":"<p><p>Ropeginterferon alfa-2b (ropeg-IFN), a treatment that allows for prolonged dosing intervals through site-specific PEGylation, has been approved for the treatment of polycythemia vera (PV) in patients for whom existing therapies are insufficient or unsuitable. In this case report, we describe a woman in her thirties who was treated with ropeg-IFN. Blood tests showed increased levels of all three blood cell lineages, and PV was diagnosed following bone marrow examination. The patient was enrolled in a clinical study in which she received ropeg-IFN because phlebotomy could not be performed due to difficulty with securing peripheral vascular access. Complete hematologic response (CHR) was achieved 12 weeks after the start of administration, and molecular response (MR) with a JAK2 V617F allele burden of <1% was achieved after approximately 156 weeks of continuous administration of ropeg-IFN. Alopecia (grade 1) occurred as an adverse event, but resolved after ropeg-IFN dose reduction. The patient maintained CHR and MR for 2 years following treatment discontinuation. We also discuss the potential benefit of ropeg-IFN in low-risk PV patients who were not previously considered candidates for aggressive cytoreductive therapy.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 3","pages":"209-213"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147576426","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":"[Successful treatment of cardiac tamponade due to/pericardial infiltration with pericardial drainage and intrapericardial chemotherapy in a patient with acute myeloid leukemia].","authors":"Hiroaki Zushi, Hiroyuki Kuroda, Kosuke Nishibori, Kana Nagashima, Yuji Kanisawa, Shouya Ino, Kazuyuki Murase, Koichi Takada, Masayoshi Kobune","doi":"10.11406/rinketsu.67.97","DOIUrl":"10.11406/rinketsu.67.97","url":null,"abstract":"<p><p>A 66-year-old man with acute myeloid leukemia with myelodysplasia-related changes (AML-MR) was admitted in April 2024 after presenting with increased peripheral blood blasts. At the initial visit, he had atrial fibrillation but no pericardial effusion. Although treatment with venetoclax and azacitidine reduced peripheral blood blasts, a concurrent cardiac tamponade was observed in May of the same year. Pericardial drainage was performed, and cytology and flow cytometry of the pericardial fluid confirmed a diagnosis of pericardial infiltration due to AML. Pericardial effusion resolved with repeated intrapericardial injections of cytarabine, allowing discontinuation of pericardial drainage. Remission induction therapy with daunorubicin and cytarabine was subsequently administered. Palliative care for AML was given during the cardiac treatment, and pericardial effusion did not occur prior to the patient's death in February 2025.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"97-103"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358031","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":"[Key points in selecting first-line therapy for chronic myeloid leukemia].","authors":"Shinya Kimura","doi":"10.11406/rinketsu.67.130","DOIUrl":"10.11406/rinketsu.67.130","url":null,"abstract":"<p><p>Chronic myeloid leukemia (CML) is driven by the BCR::ABL1 fusion gene, and the introduction of tyrosine kinase inhibitors (TKIs) has dramatically improved long-term survival. In Japan, five agents-imatinib (Glivec<sup>®</sup>), dasatinib (Sprycel<sup>®</sup>), nilotinib (Tasigna<sup>®</sup>), bosutinib (Bosulif<sup>®</sup>), and STAMP inhibitor asciminib (Scemblix<sup>®</sup>)-are currently approved for first-line therapy. Each has distinct efficacy and toxicity profiles, requiring individualized treatment decisions based on age, comorbidities, cardiovascular risk, fertility, adherence, and financial factors. Recent advances have shifted treatment goals from disease control to treatment-free remission (TFR), with imatinib, dasatinib, and nilotinib supported by robust clinical trial data. Emerging strategies, such as low-dose regimens and step-up dosing of TKI, highlight the importance of balancing efficacy with tolerability and quality of life. Looking forward, immunologic determinants of TFR and novel therapeutic approaches, including targeting CML stem cells with agents such as asciminib or demethylating drugs, may offer prospects for cure. This review summarizes the current evidence and practical considerations in selecting first-line therapy for chronic-phase CML, with a focus on optimizing long-term outcomes and advancing toward individualized and potentially curative strategies.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"130-141"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358033","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.67.183","DOIUrl":"https://doi.org/10.11406/rinketsu.67.183","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 3","pages":"183-184"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147576875","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}
Makiko Ogawa, Yuki Osada, Aina Tomori, Taro Mizuki, Sakae Tanosaki, Ken Suzuki
{"title":"[Pure red cell aplasia in a patient with cold agglutinin disease-associated lymphoproliferative disorder].","authors":"Makiko Ogawa, Yuki Osada, Aina Tomori, Taro Mizuki, Sakae Tanosaki, Ken Suzuki","doi":"10.11406/rinketsu.67.118","DOIUrl":"10.11406/rinketsu.67.118","url":null,"abstract":"<p><p>A 51-year-old woman had been noted to have anemia since 2019 and developed Raynaud's phenomenon during winter or in air-conditioned environments from 2021. In January 2024, she experienced palpitations, tinnitus, dyspnea on exertion, and fatigue. Twenty-three days later, she was urgently admitted to hospital with severe anemia (Hb 2.8 g/dl). She was diagnosed with primary cold agglutinin disease (CAD) and CAD-associated lymphoproliferative disorder (LPD). While she was under observation, undergoing red blood cell transfusion with avoidance of cold exposure, her anemia progressed without worsening of hemolysis and she was diagnosed with pure red cell aplasia (PRCA). Cyclosporine A (CsA) was administered for 10 days without response, and treatment was switched to bendamustine-rituximab (BR) for LPD. Although her LPD improved, her PRCA persisted; CsA was therefore resumed, leading to improvement in the PRCA. In October, her CAD-LPD relapsed with hemolysis, and BR therapy with continued CsA treatment again achieved improvement in the CAD-LPD without worsening of the PRCA. This rare case of primary CAD-associated LPD complicated by PRCA highlights the importance of accurate disease assessment and appropriate treatment strategies.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"118-123"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358058","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":"[Primary myelofibrosis with Factor V Leiden diagnosed following portal vein thrombosis].","authors":"Akihiko Numata, Takahiro Shima, Takashi Jiromaru, Sachiko Kanaji, Koji Kato, Koichi Akashi","doi":"10.11406/rinketsu.67.104","DOIUrl":"10.11406/rinketsu.67.104","url":null,"abstract":"<p><p>Primary myelofibrosis (PMF), a subtype of myeloproliferative neoplasms, is frequently associated with thrombosis, particularly in the presence of the JAK2 V617F mutation. In contrast, Factor V Leiden (FVL) is a hereditary thrombophilic mutation commonly observed in Caucasian populations, and clinical encounters with FVL carriers are extremely rare in Japan. Here, we report a case of an American patient who presented with portal hypertension and esophageal variceal bleeding, which led to the diagnosis of PMF. Based on family history, the patient was also diagnosed as a carrier of FVL. Treatment with ruxolitinib resulted in a marked improvement in splenomegaly and symptoms related to portal hypertension. Additionally, the patient safely underwent invasive surgery for a fracture with appropriate perioperative thrombosis risk management. This case represents a rare instance of PMF with FVL mutation encountered in Japan. In cases of atypical-site thrombosis, particularly among Caucasian patients, comprehensive evaluation including MPN driver mutations and other thrombophilic factors is essential to avoid underdiagnosis.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"104-108"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358048","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":"[Key points in second-line therapy for chronic myeloid leukemia].","authors":"Takaaki Ono","doi":"10.11406/rinketsu.67.142","DOIUrl":"10.11406/rinketsu.67.142","url":null,"abstract":"<p><p>Tyrosine kinase inhibitors (TKIs) have markedly improved the prognosis of chronic myeloid leukemia (CML). In Japan, in addition to the four established first-line TKIs, asciminib is now approved as an initial therapy, expanding the treatment options. Nevertheless, more than 10% of patients treated with asciminib over 48 weeks, and approximately 20-30% of those receiving other TKIs over five years, require second-line therapy because of resistance or intolerance. As first-line choices diversify, selecting the optimal second-line regimen has become increasingly complex. For intolerance, switching should be guided by the adverse-event profile with attention to potential cross-intolerance. For resistance, assessment of BCR::ABL1 mutations is essential, and second-line agents should be chosen according to the initial TKI and mutation sensitivity. This article summarizes the criteria and timing for switching to second-line therapy and key considerations for selecting and managing second-line TKIs, and briefly reviews the evidence for asciminib and ponatinib in second-line and later settings.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"142-151"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358050","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}