{"title":"[Skin toxicities following auto PBSCT with thiotepa-based conditioning in adult patients: a single-center retrospective analysis].","authors":"Tomomi Oka, Momoko Nishikori, Hiroyuki Irie, Mitsumasa Watanabe, Toshiyuki Kitano, Chisaki Mizumoto, Junya Kanda, Kohei Yamashita, Takashi Nomura, Akifumi Takaori-Kondo","doi":"10.11406/rinketsu.66.413","DOIUrl":"https://doi.org/10.11406/rinketsu.66.413","url":null,"abstract":"<p><p>Autologous stem cell transplantation (ASCT) with thiotepa-based conditioning is known to be frequently associated with skin complications in children, but there are no comprehensive reports on skin reactions in adults. We retrospectively analyzed skin problems in 44 patients who underwent ASCT with thiotepa-based conditioning at our institution between January 2017 and April 2023. Skin problems were observed in 19 patients (43.1%), and included blisters or erythema of grade 2 or higher in 8 patients (18.1%). Edema was observed in 8 patients (42%), blisters in 8 patients (42%), and blisters and erythema in 3 patients (16%). These skin changes showed a late onset pattern, ranging from days 22 to 101 (median day 53) post ASCT. In all cases, these skin problems gradually improved with symptomatic treatment. No factors significantly associated with skin problems were identified. A better understanding of the mechanisms involved in the development of these skin problems in thiotepa-conditioned adult patients should lead to better prevention and treatment.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"413-419"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577220","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":"[Management of pregnancy and delivery in patients with inherited thrombophilia].","authors":"Eriko Morishita","doi":"10.11406/rinketsu.66.495","DOIUrl":"10.11406/rinketsu.66.495","url":null,"abstract":"<p><p>Inherited thrombophilia is a rare disorder that increases risk of thrombotic events. The risk of venous thromboembolism (VTE) increases during pregnancy and the postpartum period. Type I antithrombin (AT) deficiency in particular confers a higher risk compared to protein C (PC) or protein S (PS) deficiency. Comprehensive assessment of VTE risk is recommended for pregnant women with hereditary thrombophilia, and should include the type of thrombophilia (AT/PC/PS deficiency), subtype (type I/type II), site of the genetic mutation, prior history of VTE, family history, and other existing or pregnancy-specific risk factors. However, aspects of management during pregnancy, such as the necessity of prophylactic anticoagulation therapy, the appropriate dosing of heparin, and the role of AT replacement therapy, remain insufficiently defined due to limited research data. Heparin is the first-line drug for both prevention and treatment of VTE. In patients with a history of VTE, therapeutic-dose heparin is recommended throughout pregnancy and the postpartum period.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"495-503"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577208","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":"[Molecularly targeted therapy strategies for adult T-cell leukemia/lymphoma].","authors":"Koji Ando","doi":"10.11406/rinketsu.66.449","DOIUrl":"10.11406/rinketsu.66.449","url":null,"abstract":"<p><p>Adult T-cell leukemia/lymphoma (ATL) has a very poor prognosis with conventional multidrug chemotherapy. Lenalidomide, an oral anticancer drug classified as an immunomodulator, showed an overall response rate of 46% in a phase II clinical trial in relapsed ATL. The antibody therapy mogamulizumab showed an overall response rate of 50% in a phase II trial of relapsed C-C motif chemokine receptor 4-positive ATL. Brentuximab vedotin has yet to show clear evidence of efficacy due to the limited number of patients enrolled in phase II trials. Epigenetic therapy has also been investigated. The EZH1/2 inhibitor valemetostat showed a response rate of 48% in a phase II trial in relapsed/refractory aggressive ATL. The histone acetylation inhibitor tucidinostat also exhibited efficacy in ATL, with an objective response rate of 30.4%. This review focuses on the abovementioned molecular-targeted agents, which are all currently used in Japan.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"449-455"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577210","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":"[Novel therapeutic approaches for multiple myeloma].","authors":"Kyoko Yoshihara","doi":"10.11406/rinketsu.66.440","DOIUrl":"10.11406/rinketsu.66.440","url":null,"abstract":"<p><p>Multiple myeloma has conventionally been treated with three major classes of drugs, including immunomodulatory drugs, proteasome inhibitors and anti-CD38 antibodies, but T-cell redirection therapies (CAR-T cell therapy and bispecific antibodies) targeting B-cell maturation antigen (BCMA), which is highly expressed on the surface of myeloma cells, have now been adopted as a new modality. In Japan, CAR-T therapy is indicated as a third-line treatment for patients who have already received three classes of drugs, and bispecific antibodies are also indicated for patients who have already received three lines of therapy. Regimens combining antibody-drug conjugates targeting BCMA with bortezomib or pomalidomide have also demonstrated efficacy. GPRC5D is also attracting attention as a new target, and bispecific antibodies should soon become available for clinical use. Although these are very effective treatments, they are not curative. Therefore, determining how to sequence conventional treatments (combinations of the three classes) and these new treatments will be an important clinical challenge.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"440-448"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577212","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":"[Intramuscular abscess and multiple lymphadenitis due to Nocardia asiatica infection requiring differential diagnosis from smoldering adult T-cell leukemia/lymphoma].","authors":"Mahito Yamashiro, Tomo Nakajima, Yuka Irei, Riko Miyagi, Sakiko Kitamura, Keita Tamaki, Yukiko Nishi, Kazuho Morichika, Sawako Nakachi, Hiroki Maehara, Takuya Fukushima, Hiroaki Masuzaki","doi":"10.11406/rinketsu.66.121","DOIUrl":"10.11406/rinketsu.66.121","url":null,"abstract":"<p><p>A 72-year-old male engaged in horticulture developed a progressively enlarging painful mass in the left upper arm. The first biopsy revealed no malignancy or pathogenic bacteria. He completed an adequate course of antibiotics for infectious lymphadenitis, but showed no apparent improvement. Computed tomography demonstrated systemic lymphadenopathy, suggesting malignant lymphoma. Monoclonal integration of the HTLV-1 proviral genome in peripheral blood cells was confirmed by Southern blot analysis, but repeated lymph node biopsies identified no malignant cells. Based on these findings, a diagnosis of smoldering adult T-cell leukemia/lymphoma was made. After prolonged culture of the specimen, Nocardia sp. were detected and 16S rRNA sequencing confirmed infection by Nocardia asiatica. Combination therapy with sulfamethoxazole-trimethoprim and minocycline was started, and the systemic lymphadenopathy improved within 3 months. Although Nocardia asiatica infection manifesting as systemic lymphadenitis is extremely rare, it should be considered as a possibility in immunocompromised patients to ensure an accurate diagnosis.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"121-126"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569236","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":"[Chronic graft-versus-host disease manifesting as isolated myositis].","authors":"Ayako Yamamura, Takashi Hanada, Taichi Hirano, Kazuhito Tanaka, Nao Nishimura, Shinya Endo, Kenji Tokunaga, Yoshiki Mikami, Jun-Ichirou Yasunaga","doi":"10.11406/rinketsu.66.318","DOIUrl":"https://doi.org/10.11406/rinketsu.66.318","url":null,"abstract":"<p><p>A 26-year-old man underwent HLA-matched sibling allogeneic peripheral blood stem cell transplantation for acute myeloid leukemia with KMT2A rearrangement and maintained complete remission. On day338 post-transplant, he developed fever, myalgia, and markedly elevated creatinine kinase. We initially suspected drug-induced rhabdomyolysis and stopped the offending drugs, but his symptoms did not improve. Immunohistological findings with PD-1 staining in a muscle biopsy led to the diagnosis of graft versus host disease (GVHD)-associated myositis. The myositis resolved with prednisolone treatment based on the conventional chronic GVHD treatment protocol. Chronic GVHD with myositis alone is rare, and GVHD-associated myositis has been difficult to differentiate from other forms of myositis due to its lack of specific pathological features. Recently, PD-1 and HLA-DR expression have been reported as characteristic features of chronic GVHD-associated myositis. The pathological finding of PD-1-positive cell infiltration was useful in diagnosis and treatment in our case.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"318-323"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227923","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.66.369","DOIUrl":"https://doi.org/10.11406/rinketsu.66.369","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"369-395"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227921","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":"[CAR T-cell therapy and bispecific antibodies for relapsed/refractory large B-cell lymphoma].","authors":"Hideki Goto","doi":"10.11406/rinketsu.66.432","DOIUrl":"10.11406/rinketsu.66.432","url":null,"abstract":"<p><p>While many cases of large B-cell lymphoma (LBCL) can be cured with initial treatment, some patients experience relapse or are refractory to treatment. Chimeric antigen receptor (CAR) T-cell therapy and bispecific antibodies can be used for relapsed or chemotherapy-refractory LBCL. This review highlights the latest evidence regarding CAR T-cell therapy and bispecific antibodies for relapsed or refractory LBCL.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"432-439"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577204","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 with EPOCH followed by mogamulizumab for angioimmunoblastic T-cell lymphoma with myelofibrosis and pure red cell aplasia].","authors":"Ayako Yamamura, Kisato Nosaka, Hiro Tatetsu, Jun-Ichirou Yasunaga","doi":"10.11406/rinketsu.66.24","DOIUrl":"10.11406/rinketsu.66.24","url":null,"abstract":"<p><p>The patient was a 70-year-old woman. She visited a doctor for sore throat, facial edema, and lymphadenopathy. Lymph node biopsy was performed, and led to a diagnosis of AITL. When the patient was admitted to our department for treatment, she had severe anemia with Hb 4.2 g/dl, and bone marrow aspiration resulted in a dry tap. Bone marrow biopsy revealed infiltration of tumor cells, a severe decrease in erythroblastic cells, and bone marrow fibrosis. Although lymph node size was reduced by THP-COP, bone marrow biopsy showed residual infiltration of tumor cells. In addition, since low reticulocyte counts and anemia persisted, weekly red blood cell transfusions were required. Therefore, the treatment regimen was changed to EPOCH. After two cycles of EPOCH, anemia improved, and red cell transfusions were no longer required. Bone marrow examination after four cycles showed disappearance of lymphoma cells and no evidence of bone marrow fibrosis or erythroblasts. However, due to residual bone marrow infiltration of lymphoma cells, treatment with mogamulizumab was started. Complete remission was maintained for five years. This indicates that EPOCH and mogamulizumab are treatment options for AITL patients with bone marrow involvement, PRCA, and myelofibrosis.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"24-29"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384007","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":"[Therapy-related myeloid neoplasms with inv (16)(p13.1q22);CBFB::MYH11 during treatment for AL amyloidosis].","authors":"Kodai Kunisada, Mizuki Ogura, Yuki Oda, Moe Yogo, Tomomi Takei, Kota Sato, Taku Kikuchi, Yu Abe, Nobuhiro Tsukada, Tadao Ishida","doi":"10.11406/rinketsu.66.18","DOIUrl":"10.11406/rinketsu.66.18","url":null,"abstract":"<p><p>[Patient] A 65-year-old man. [History of present illness] An abnormal chest shadow was noted in March of 2011, and hoarseness was observed in November of 2017. Both times, AL amyloidosis was diagnosed by biopsy. The patient was admitted to our department for treatment in March 2018, and received 6 cycles of melphalan plus dexamethasone for systemic AL amyloidosis in May. His condition was good, but a blood test in August 2019 showed white blood cells 50,000/µl and 44.9% blasts in the peripheral blood, leading to a diagnosis of treatment-related acute leukemia (AML with inv (16)(p13.1q22);CBFB::MYH11). He achieved complete remission with standard treatment, but relapsed in May 2020, CNS relapsed in September 2020, CNS relapsed again in July 2021, and CNS relapsed a third time in May 2022. He underwent intensive chemotherapy, whole brain radiation therapy, 13 rounds of intrathecal injection, and five cycles of venetoclax plus azacitidine, but his general condition gradually worsened. He was transferred to best supportive care in November and died in June 2023. [Discussion] Although advances in treatment have extended survival in systemic AL amyloidosis, long-term follow-up for secondary cancer is important for patients with long-term exposure, as in this case.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"18-23"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384105","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}