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

[Delayed diagnosis of hydroa vacciniforme-like lymphoproliferative disorder in a patient with skin rashes]. [皮疹患者的疫苗样水痘样淋巴增生性疾病的延迟诊断]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.209

Yuka Umeki, Mako Ikeda, Wataru Nakahara, Mizuki Asako, Yoshiki Matsuoka, Takuya Terakawa, Hitomi Matsunaga, Rei Tadokoro, Masahiro Sekiguchi, Takahiro Matsui, Kazumasa Oka, Kohei Higuchi, Akihisa Sawada, Masami Inoue, Shuji Ueda

{"title":"[Delayed diagnosis of hydroa vacciniforme-like lymphoproliferative disorder in a patient with skin rashes].","authors":"Yuka Umeki, Mako Ikeda, Wataru Nakahara, Mizuki Asako, Yoshiki Matsuoka, Takuya Terakawa, Hitomi Matsunaga, Rei Tadokoro, Masahiro Sekiguchi, Takahiro Matsui, Kazumasa Oka, Kohei Higuchi, Akihisa Sawada, Masami Inoue, Shuji Ueda","doi":"10.11406/rinketsu.64.209","DOIUrl":"https://doi.org/10.11406/rinketsu.64.209","url":null,"abstract":"Chronic active Epstein-Barr virus (CAEBV) infection is characterized by persistent EBV infection and can lead to fatal conditions such as hemophagocytic syndrome and malignant lymphoma through the clonal expansion of EBV-infected T or natural killer (NK) cells. Hydroa vacciniforme lymphoproliferative disorder (HV) and hypersensitivity to mosquito bites (HMB) have been identified as skin diseases in EBV-associated T- or NK-cell lymphoproliferative diseases. We present the case of a 33-year-old man. The patient had frequent episodes of a facial rash for three years before he visited our hospital, he visited several dermatologists but did not receive a diagnosis of HV. He was referred to the hematology department of our hospital for assessment of atypical lymphocytes in peripheral blood. Based on routine blood and bone marrow test we were unable to diagnose HV. However, when the patient's liver function deteriorated six months later, we considered the possibility of HV after reevaluating the skin rash. After performing EBV-related tests, we were able to definitively diagnose CAEBV with HV. It is crucial to be able to connect clinical observations to EBV-related tests when diagnosing CAEBV. Hematologists must be knowledgeable of the EBV-associated skin conditions of HV and HMB.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 3","pages":"209-213"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9612091","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

[Anti-MDA5 antibody-positive rapidly progressive interstitial lung disease after allogeneic hematopoietic cell transplantation successfully treated with triple immunosuppressive therapy]. 【抗mda5抗体阳性的异基因造血细胞移植后快速进行性间质性肺病经三联免疫抑制治疗成功】。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.250

Yuhei Nakamura, Shunto Kawamura, Shimpei Matsumi, Kazuhisa Matsumoto, Satona Tanaka, Takuto Ishikawa, Akari Matsuoka, Tomohiro Meno, Masakatsu Kawamura, Junko Takeshita, Nozomu Yoshino, Kazuki Yoshimura, Yukiko Misaki, Ayumi Gomyo, Yosuke Okada, Masaharu Tamaki, Machiko Kusuda, Yu Akahoshi, Kazuaki Kameda, Hidenori Wada, Shun-Ichi Kimura, Hideki Nakasone, Shinichi Kako, Hiroshi Date, Yoshinobu Kanda

{"title":"[Anti-MDA5 antibody-positive rapidly progressive interstitial lung disease after allogeneic hematopoietic cell transplantation successfully treated with triple immunosuppressive therapy].","authors":"Yuhei Nakamura, Shunto Kawamura, Shimpei Matsumi, Kazuhisa Matsumoto, Satona Tanaka, Takuto Ishikawa, Akari Matsuoka, Tomohiro Meno, Masakatsu Kawamura, Junko Takeshita, Nozomu Yoshino, Kazuki Yoshimura, Yukiko Misaki, Ayumi Gomyo, Yosuke Okada, Masaharu Tamaki, Machiko Kusuda, Yu Akahoshi, Kazuaki Kameda, Hidenori Wada, Shun-Ichi Kimura, Hideki Nakasone, Shinichi Kako, Hiroshi Date, Yoshinobu Kanda","doi":"10.11406/rinketsu.64.250","DOIUrl":"https://doi.org/10.11406/rinketsu.64.250","url":null,"abstract":"A 34-year-old man with KMT2A-MLLT1 acute myeloid leukemia in first complete remission underwent allogeneic peripheral blood stem cell transplantation from his HLA-matched sister after conditioning with busulfan/cyclophosphamide. He did not have severe graft-versus-host disease, but he developed interstitial pneumonia six months after transplantation when his oral cyclosporine A (CsA) dose was reduced to 10 mg/day. He was given prednisolone (PSL), which temporarily improved his respiratory condition, but he quickly deteriorated when PSL was reduced. Anti-MDA5 antibody was found to be positive after additional testing, and a three-drug combination of intravenous cyclophosphamide+PSL+CsA was initiated for anti-MDA5 antibody positive rapidly progressive interstitial lung disease, which was effective for interstitial pneumonia. He received a successful living-donor lung transplant from his younger brother and sister. We present a case of rapidly progressive anti-MDA5 antibody positive interstitial lung disease in which the patient's respiratory condition improved after triple therapy and subsequent living-donor lung transplantation.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 4","pages":"250-254"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9384942","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

[HLA-haploidentical stem cell transplantation]. hla -单倍体干细胞移植。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.515

Junichi Sugita

{"title":"[HLA-haploidentical stem cell transplantation].","authors":"Junichi Sugita","doi":"10.11406/rinketsu.64.515","DOIUrl":"https://doi.org/10.11406/rinketsu.64.515","url":null,"abstract":"HLA-haploidentical stem cell transplantations using posttransplant cyclophosphamide (PTCy-haplo) rapidly increased worldwide. In Japan, the number of HLA-haploidentical stem cell transplantation cases exceeded related HLA-matched transplants in 2020. Recent retrospective studies using Japanese registry data have reported comparable transplantation outcomes between PTCy-haplo and HLA-matched unrelated and cord blood transplantations. PTCy-haplo was initially developed in the bone marrow transplantation setting after non-myeloablative conditioning but has recently become widely used in peripheral blood stem cell transplantation and myeloablative conditioning. Peripheral blood stem cell transplantation increases the occurrence of graft-versus-host disease but may have more improved transplant outcomes compared with bone marrow transplantation. Other factors, such as the number of infused cluster of differentiation 34-positive cells, donor age, HLA class II mismatch, HLA-B leader, and reduced PTCy dosage, may also contribute to the outcome of PTCy-haplo transplantations. Furthermore, PTCy has been reportedly effective in related/unrelated HLA-matched transplantation and HLA-mismatched unrelated transplantations. A prospective phase II trial using PTCy in patients who underwent HLA-matched and 1-2 allele-mismatched transplantation is ongoing in Japan. Patient enrollment has already been completed, and the results will be revealed soon. Using PTCy to sufficiently reduce the occurrence of graft-versus-host disease will make performing allogeneic transplants with a higher safety level possible.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 6","pages":"515-523"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9803683","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: 1

[Thoracic SMARCA4-deficient undifferentiated tumor resembling malignant lymphoma]. [胸腔smarca4缺陷未分化肿瘤，类似恶性淋巴瘤]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.271

Chie Asou, Tomoya Maeda, Ou Yamaguchi, Daisuke Okamura, Maho Ishikawa, Mika Kohri, Kunihiro Tsukasaki, Akira Matsuda, Norio Asou, Tsugumi Satoh, Hidekazu Kayano, Naoki Takahashi

{"title":"[Thoracic SMARCA4-deficient undifferentiated tumor resembling malignant lymphoma].","authors":"Chie Asou, Tomoya Maeda, Ou Yamaguchi, Daisuke Okamura, Maho Ishikawa, Mika Kohri, Kunihiro Tsukasaki, Akira Matsuda, Norio Asou, Tsugumi Satoh, Hidekazu Kayano, Naoki Takahashi","doi":"10.11406/rinketsu.64.271","DOIUrl":"https://doi.org/10.11406/rinketsu.64.271","url":null,"abstract":"We present a case of thoracic SMARCA4-deficient undifferentiated tumor that needed to be differentiated from malignant lymphoma owing to multiple lymph node swelling and marrow involvement. A 52-year-old man developed multiple lymphadenopathies along with anorexia, general fatigue, fever, and sweating 2 months prior to admission. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) scan revealed a mass lesion on the right upper lung, generalized lymph node swelling, and bone metastasis, indicating the presence of suspicious lung cancer; therefore, he was referred to our hospital. Malignant lymphoma was suspected at the time of admission because of elevated levels of lactate dehydrogenase (11,977 U/l) and soluble interleukin 2 receptor (2,152 U/ml) as well as marrow infiltration of large abnormal cells. On day 11, the patient died from rapid respiratory failure. Histological and immunohistochemical features of the pleural effusion cell block led to the diagnosis of thoracic SMARCA4-deficient undifferentiated tumor. Thoracic SMARCA4-deficient undifferentiated tumor was recently introduced in the 2021 World Health Organization classification of lung tumors, with most patients being young adults with a history of heavy smoking and poor prognosis. Because of the multiple lymph node swelling and marrow involvement, this undifferentiated tumor should be distinguished from malignant lymphoma.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 4","pages":"271-276"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9811565","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

[New concepts in multiple myeloma by returning to cytogenetics]. [从细胞遗传学角度探讨多发性骨髓瘤的新概念]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.411

Taku Tsukamoto

{"title":"[New concepts in multiple myeloma by returning to cytogenetics].","authors":"Taku Tsukamoto","doi":"10.11406/rinketsu.64.411","DOIUrl":"https://doi.org/10.11406/rinketsu.64.411","url":null,"abstract":"Multiple myeloma (MM) is characterized by genomic instability, which causes multiple genetic and chromosomal alterations and leads to disease progression and therapeutic resistance. Overlapping mechanisms, including defective genome repair machinery such as the loss of TP53 activity, as well as chromosomal segregation error represented by the abnormality of mitotic checkpoint kinases such as BUB1, cell cycle dysregulation, and tumor environment, cause structural and numerical chromosomal abnormalities. Cytogenetic abnormalities are important prognostic factors, and they are also linked to the use of proteasome inhibitors, immunomodulatory drugs, monoclonal antibodies, and the BCL2 inhibitor venetoclax. We developed new diagnostic modalities for chromosomal analysis to improve the sensitivity and convenience of chromosomal diagnosis. The immunophenotyped-suspension-multiplex (ISM)-fluorescence in situ hybridization (FISH) can use imaging flow to examine three IGH-related chromosomal translocations at the same time. We also created a new FISH method called amplified FISH (amFISH) to detect microdeletion involving narrow chromosomal regions (approximately<100 kb), such as the microdeletions of 1p32 (CDKN2C) or of 14q32 (TRAF3), by using a fluorescent antibody to amplify the signals of small probes. Even in the era of clinical sequencing, these convenient modalities may hasten the cytogenetics-oriented therapeutic approach for MM.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 5","pages":"411-417"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9631074","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

[Molecular targeted therapy for acute myeloid leukemia]. [分子靶向治疗急性髓性白血病]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.345

Yuichi Ishikawa

{"title":"[Molecular targeted therapy for acute myeloid leukemia].","authors":"Yuichi Ishikawa","doi":"10.11406/rinketsu.64.345","DOIUrl":"https://doi.org/10.11406/rinketsu.64.345","url":null,"abstract":"Acute myeloid leukemia (AML) is a heterogeneous disease, and the accumulation of various chromosomal and genetic abnormalities is considerably involved in its pathogenesis and prognosis. Recently, the disease classification based on molecular abnormalities and novel molecular-targeting therapies has been developed. In Europe and the United States, several agents have been approved for AML and incorporated into guidelines as the standard treatment depending on comorbid genetic mutations combined with conventional chemotherapy or monotherapy since 2017. The combination therapy of FLT3 inhibitor midostaurin and intensive chemotherapy has improved the prognosis of patients with FLT3-ITD-positive AML, which has been considered a poor prognosis for a long period. In addition to small-molecule compounds, various novel therapies for AML are under clinical investigation, including antibody therapies targeting CD47 and TIM-3, bispecific antibodies, and CAR-T-cell therapies. Considering the treatment strategies with diverse therapeutic modalities, the pathogenesis and clonal selection process of refractory AML, including the surrounding environment of residual leukemia cells, should be clarified. The combination of new therapies and chemotherapies is highly expected to improve the prognosis of patients with AML in the near future.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 5","pages":"345-354"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9631071","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

[Polatuzumab vedotin, bendamustine, and rituximab in patients with relapsed/refractory diffuse large B-cell lymphoma, including the outcome as a bridging treatment to CAR-T cell therapy or allogeneic hematopoietic stem cell transplant]. [Polatuzumab vedotin，苯达莫司汀和利妥昔单抗在复发/难治性弥漫性大b细胞淋巴瘤患者中的应用，包括作为CAR-T细胞治疗或同种异体造血干细胞移植的桥接治疗的结果]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.586

Yuka Morita, Yu Yagi, Yusuke Kanemasa, Yuki Sasaki, Kento Ishimine, Yudai Hayashi, Mano Mino, An Ohigashi, Taichi Tamura, Shohei Nakamura, Toshihiro Okuya, Takuya Shimizuguchi, Naoki Shingai, Takashi Toya, Hiroaki Shimizu, Yuho Najima, Takeshi Kobayashi, Kyoko Haraguchi, Noriko Doki, Yoshiki Okuyama, Kazuteru Ohashi, Tatsu Shimoyama

{"title":"[Polatuzumab vedotin, bendamustine, and rituximab in patients with relapsed/refractory diffuse large B-cell lymphoma, including the outcome as a bridging treatment to CAR-T cell therapy or allogeneic hematopoietic stem cell transplant].","authors":"Yuka Morita, Yu Yagi, Yusuke Kanemasa, Yuki Sasaki, Kento Ishimine, Yudai Hayashi, Mano Mino, An Ohigashi, Taichi Tamura, Shohei Nakamura, Toshihiro Okuya, Takuya Shimizuguchi, Naoki Shingai, Takashi Toya, Hiroaki Shimizu, Yuho Najima, Takeshi Kobayashi, Kyoko Haraguchi, Noriko Doki, Yoshiki Okuyama, Kazuteru Ohashi, Tatsu Shimoyama","doi":"10.11406/rinketsu.64.586","DOIUrl":"https://doi.org/10.11406/rinketsu.64.586","url":null,"abstract":"Pola-BR (polatuzumab vedotin, bendamustine, and rituximab) therapy received approval for relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL) in Japan in March 2021. There have been few reports on the efficacy and safety of Pola-BR therapy in Japanese clinical practice. A retrospective analysis was performed on twenty-nine patients with R/R DLBCL who received Pola-BR therapy at our institution (intent to cellular immunotherapy cohort: 20 patients, stand-alone treatment cohort: nine patients). The overall response rate was 69.0% (complete response 27.6%). The median progression-free survival was 5.1 months, with a 9.5-month median overall survival. In the intent to cellular immunotherapy cohort, 11 of 19 patients received chimeric antigen receptor T-cell (CAR-T) infusions, and one patient received allogeneic stem cell transplantation. Four patients received Pola-BR therapy, including bendamustine before leukapheresis, and all produced CAR-T products successfully. 3 of the 28 patients experienced grade3 or higher adverse events, and two required treatment discontinuation. Our single institution, a real-world cohort of R/R DLBCL patients showed high efficacy outcomes and a tolerable toxicity profile for Pola-BR therapy, which is comparable to previous studies. More cases are needed to determine its impact on CAR-T therapy and stem cell transplantation.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 7","pages":"586-595"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10412869","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

[Pathogenesis and treatment of immune dysregulation associated with myelodysplastic syndromes]. [骨髓增生异常综合征相关免疫失调的发病机制和治疗]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.753

Hideaki Nakajima

{"title":"[Pathogenesis and treatment of immune dysregulation associated with myelodysplastic syndromes].","authors":"Hideaki Nakajima","doi":"10.11406/rinketsu.64.753","DOIUrl":"10.11406/rinketsu.64.753","url":null,"abstract":"Myelodysplastic syndromes (MDS) are a clonal disorder based on genomic mutations in hematopoietic stem cells. They are categorized as lower-risk MDS, characterized by peripheral cytopenia; and higher-risk MDS, characterized by progression to acute myeloid leukemia. Previous studies reported that inflammation and immune activation are deeply involved in the pathogenesis of lower-risk MDS. Recent studies elucidated the molecular basis for the activation of inflammatory pathways via dysregulated innate immune system and the resultant cell-death acceleration in lower-risk MDS. Conversely, immunosuppression and immune escape are substantially involved in the pathogenesis and disease progression of higher-risk MDS. VEXAS syndrome is an autoinflammatory disease characterized by clonal hematopoiesis with somatic mutation of UBA1 in hematopoietic stem and progenitor cells and has attracted broad attention as a lower-risk MDS model caused by systemic inflammation. Although therapeutic effects of immunosuppressants are observed for a limited number of patients with lower-risk MDS with inflammation, an optimal treatment should be developed based on their pathology.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 8","pages":"753-763"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10183273","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 : 2023-01-01 DOI: 10.11406/rinketsu.64.678

引用次数: 0

[Large granular lymphocytic leukemia and its association with immune dysregulation]. [大颗粒淋巴细胞白血病及其与免疫失调的关系]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.791

Fumihiro Ishida

{"title":"[Large granular lymphocytic leukemia and its association with immune dysregulation].","authors":"Fumihiro Ishida","doi":"10.11406/rinketsu.64.791","DOIUrl":"https://doi.org/10.11406/rinketsu.64.791","url":null,"abstract":"Large granular lymphocytic (LGL) leukemia is a chronic lymphoproliferative disease of cytotoxic T cells or NK cells with LGL morphology and frequently complicated cytopenia and/or different autoimmune diseases, which often require medical interventions, although LGL leukemia itself is seldom lethal. Immunologic dysregulations in LGL leukemia contribute to the development of complications, for example, neutropenia with the involvement of Fas ligand system and, in pure red cell aplasia, which is a common complication among the patients of East Asian origin, impairing erythroid developments by cytotoxic T cells. Rheumatoid arthritis (RA) is the most prevalent nonhematological consequence, and Felty syndrome, a rare form of RA, and T-LGL leukemia have a lot in common. When patients have LGL leukemia-associated complications, immunosuppressive medication is a mainstay of treatment. Characteristic mutational features in STAT3, STAT5B, CCL22, and other genes in specific subtypes of LGL leukemia have been detected, that would be associated with immunologically mediated molecular pathogenesis in LGL leukemia, and these new findings may help in creating optimal diagnostic approaches or novel therapies for LGL leukemia.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 8","pages":"791-798"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10183272","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