{"title":"[Intravascular large B-cell lymphoma presenting as subacute progressive myelopathy].","authors":"Kiwamu Doi, Yosuke Nonohara, Soichiro Sakamoto, Toshiyuki Kitano","doi":"10.11406/rinketsu.66.12","DOIUrl":"https://doi.org/10.11406/rinketsu.66.12","url":null,"abstract":"<p><p>The clinical manifestations of intravascular large B-cell lymphoma (IVLBCL) are highly variable and include constitutional B symptoms, neurological findings, and skin lesions. We report the case of a 64-year-old male patient who presented with myelopathy as a sole manifestation of IVLBCL. He had experienced progressive bilateral leg weakness along with bladder and rectal dysfunction for several months. He did not have non-neurological symptoms such as fever, night sweats, and weight loss at presentation. Blood tests, cerebrospinal fluid analysis, random skin biopsy, and bone marrow biopsy showed no evidence of lymphoproliferative disease. Subsequently, the patient developed a fever, and his LDH and soluble interleukin-2 receptor levels were elevated. PET/CT showed ground-glass opacity of the lung with associated FDG avidity. Transbronchial lung biopsy confirmed a diagnosis of IVLBCL. The patient received 6 cycles of R-CHOP combined with 2 cycles of high-dose methotrexate. His symptoms gradually improved with the chemotherapy, and PET/CT after completion of the treatment showed a complete metabolic response.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"12-17"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383795","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":"[Good's syndrome complicated with pure white cell aplasia].","authors":"Yukiko Nishizaki, Yuki Osada, Hikari Kanai-Sudo, Taro Mizuki, Sakae Tanosaki, Ken Suzuki","doi":"10.11406/rinketsu.66.106","DOIUrl":"https://doi.org/10.11406/rinketsu.66.106","url":null,"abstract":"<p><p>A 58-year-old man was admitted to our department with a fever that started 1 week prior. His white blood cell count was 600/µl, with 0% neutrophils, and erythrocyte and platelet counts were in the normal range. The bone marrow was hypoplastic with 5.8% myeloblasts, but the number of granulocytes differentiated from promyelocytes was markedly decreased, with an M/E ratio of 0.18. The erythroblasts and megakaryocytes were of normal karyotype with no morphological abnormalities. Good's syndrome complicated by pure white cell aplasia (PWCA) was diagnosed. G-CSF was administered for eight days, but produced no response. Ten days after starting cyclosporine, neutrophils recovered and cyclosporine was tapered off. On admission, pharyngitis and enteritis were observed, which resolved with antibiotics, antifungal agents, G-CSF, and immunoglobulin supplementation. The anterior mediastinal mass was removed, and was diagnosed as thymoma type A. PWCA is rarely observed in Good's syndrome, and recurrence of PWCA after thymectomy poses a challenge in its treatment.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"106-110"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569229","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":"https://doi.org/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":"[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":"https://doi.org/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":"https://doi.org/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}
{"title":"","authors":"","doi":"10.11406/rinketsu.66.190","DOIUrl":"https://doi.org/10.11406/rinketsu.66.190","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 3","pages":"190-191"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143774967","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":"[Refractory acute monocytic leukemia with cutaneous infiltration complicated by sterile pustules after CPX-351 administration].","authors":"Takahiro Nishiyama, Takuya Maki, Tomohiro Yamada, Kohei Usui, Yusuke Yamaga, Shoichiro Okazaki","doi":"10.11406/rinketsu.66.165","DOIUrl":"10.11406/rinketsu.66.165","url":null,"abstract":"<p><p>CPX-351 (Vyxeos<sup>®</sup>), a liposomal formulation used in the treatment of acute myeloid leukemia, has been associated with cutaneous adverse events, particularly purpura, in previous clinical trials. We present the case of a 73-year-old woman with refractory acute monocytic leukemia and leukemia cutis, who was treated with CPX-351 as salvage induction therapy. Following treatment, the extramedullary lesions resolved; however, some skin lesions developed into pustular eruptions. These pustules appeared during the neutropenic phase, and cultures ruled out infection, leading to the diagnosis of sterile pustulosis. Supportive care, including skin care measures and application of antibiotic ointment, allowed for continued treatment, and the pustules resolved after crusting. To our knowledge, this is the first reported case of sterile pustulosis associated with CPX-351 treatment, highlighting the importance of early detection and appropriate management of skin complications for the successful continuation of therapy.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 3","pages":"165-169"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143775054","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":"[Progressive multifocal leukoencephalopathy developed 3 years after related HLA-haploidentical peripheral blood stem cell transplantation].","authors":"Kumiko Ando, Kazuo Nakamichi, Kiyoharu Hirose, Yuichi Taneyama, Harumi Kakuda, Hidemasa Ochiai","doi":"10.11406/rinketsu.66.36","DOIUrl":"https://doi.org/10.11406/rinketsu.66.36","url":null,"abstract":"<p><p>The patient was a 22-year-old man. 8 years ago, he developed T-cell lymphoblastic lymphoma and relapsed during treatment. He underwent HLA-haploidentical peripheral blood stem cell transplantation (PBSCT) from a related donor and achieved remission. After transplantation, he developed severe chronic graft-versus-host disease (GVHD) with systemic involvement, requiring long-term administration of several immunosuppressive drugs. About 3 years and 9 months after the transplant, he was experiencing depression, anorexia, and weight loss. Brain MRI showed hyperintense lesions in T2-weighted imaging extending from the right cerebellar hemisphere to the middle cerebellar peduncle with slight enhancement, and lymphoma recurrence was also suspected. Finally, a quantitative real-time PCR test was positive for JC virus (JCV) in the cerebrospinal fluid (CSF) at 61 copies/ml, leading to a diagnosis of progressive multifocal leukoencephalopathy (PML). We considered the patient to have prolonged secondary immunodeficiency after transplantation, and gradually reduced his immunosuppressive drugs. After that, the JCV in CSF became less sensitive to detection. This case highlights the importance of monitoring for PML as a potential late complication after hematopoietic stem cell transplantation, and provides the valuable insight that improvement was achieved only through dose reduction and discontinuation of immunosuppressive drugs.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"36-41"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383981","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.66.1","DOIUrl":"https://doi.org/10.11406/rinketsu.66.1","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"1"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384229","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":"[Combination therapy with efgartigimod, fostamatinib, eltrombopag and prednisolone for a patient with refractory immune thrombocytopenia].","authors":"Toru Kida, Takayuki Ozawa, Tomoya Takahashi, Ayano Esaki, Hiroaki Masaie, Seiji Tadokoro, Satoru Kosugi","doi":"10.11406/rinketsu.66.117","DOIUrl":"https://doi.org/10.11406/rinketsu.66.117","url":null,"abstract":"<p><p>A 50-year-old man with immune thrombocytopenic purpura (ITP) was initially treated with prednisolone after 10 years of observation, but did not respond. Treatment with the thrombopoietin receptor agonist (TPO-RA) eltrombopag failed as well. After a transient partial response with fostamatinib, platelet counts decreased again, and the patient showed a severe bleeding tendency. Additional treatment with rituximab or TPO-RAs also failed, and thus efgartigimod was added to fostamatinib. The patient finally maintained a partial response with a combination of efgartigimod, fostamatinib, eltrombopag and prednisolone. Although new agents for ITP (fostamatinib and efgartigimod) have recently been introduced in Japan, there is little clinical experience with combination therapies incorporating these agents. This case suggests that four-drug combination might be beneficial.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"117-120"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569191","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}