Clinical Hematology International最新文献

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Risks and benefits of cannabis as a pain control modality in patients with sickle cell disease 将大麻作为镰状细胞病患者止痛方式的风险和益处
Clinical Hematology International Pub Date : 2023-12-18 DOI: 10.46989/001c.90837
J. Jacobs, Brian D. Adkins, Laura D Stephens, Jennifer S Woo, Garrett Booth
{"title":"Risks and benefits of cannabis as a pain control modality in patients with sickle cell disease","authors":"J. Jacobs, Brian D. Adkins, Laura D Stephens, Jennifer S Woo, Garrett Booth","doi":"10.46989/001c.90837","DOIUrl":"https://doi.org/10.46989/001c.90837","url":null,"abstract":"","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138995019","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
Chimeric Antigen Receptor T-cell Therapy for Chronic Lymphocytic Leukemia: What is the supporting evidence so far? 慢性淋巴细胞白血病的嵌合抗原受体 T 细胞疗法:目前有哪些支持性证据?
Clinical Hematology International Pub Date : 2023-11-28 DOI: 10.46989/001c.88382
R. Mohty, Shaykha Alotaibi, M. Gadd, Yan Luo, Ricardo D. Parrondo, Hong Qin, M. Kharfan-Dabaja
{"title":"Chimeric Antigen Receptor T-cell Therapy for Chronic Lymphocytic Leukemia: What is the supporting evidence so far?","authors":"R. Mohty, Shaykha Alotaibi, M. Gadd, Yan Luo, Ricardo D. Parrondo, Hong Qin, M. Kharfan-Dabaja","doi":"10.46989/001c.88382","DOIUrl":"https://doi.org/10.46989/001c.88382","url":null,"abstract":"While acknowledging that newer therapies have improved survival rates in chronic lymphocytic leukemia (CLL), patients with high-risk disease features are at an increased risk of treatment failure. Allogeneic hematopoietic cell transplantation (allo-HCT) was traditionally offered as front-line consolidation in high-risk CLL; however, with the emergence of targeted therapies like Bruton tyrosine kinase (BTK) and B-cell lymphoma 2 (BCL-2) inhibitors, the role of allo-HCT has been relegated to later stages of the disease. Patients with relapsed/refractory (R/R) CLL who have failed both BTK and BCL-2 inhibitors represent a therapeutic challenge owing to a poor prognosis. Chimeric antigen receptor T-cell (CAR T) therapies targeting CD19 have improved response rates and overall survival in various types of R/R B-cell non-Hodgkin lymphomas. For CLL, no approved CAR T-cell therapies are yet available. Emerging data appear to show a therapeutic benefit of CAR T-cell therapy in patients with R/R CLL, even after failing an allo-HCT.","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139217981","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
Outcome of Stem Cell Transplantation in HTLV-1-Associated North American Adult T-Cell Leukemia/Lymphoma. 干细胞移植治疗htlv -1相关北美成人t细胞白血病/淋巴瘤的疗效
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00032-y
Abdul-Hamid Bazarbachi, Daniel Reef, Hiba Narvel, Riya Patel, Rama Al Hamed, Sindhu Vikash, Karun Neupane, Eleftheria Atalla, Astha Thakkar, Shafia Rahman, Urvi Shah, Diego Adrianzen-Herrera, Ryann Quinn, Sumaira Zareef, Emma Rabinovich, Alyssa De Castro, Felisha Joseph, Kailyn Gillick, Jennat Mustafa, Fariha Khatun, Amanda Lombardo, Latoya Townsend-Nugent, Michelly Abreu, Nicole Chambers, Richard Elkind, Yang Shi, Yanhua Wang, Olga Derman, Kira Gritsman, Ulrich Steidl, Mendel Goldfinger, Noah Kornblum, Aditi Shastri, Ioannis Mantzaris, Liza Bachier-Rodriguez, Nishi Shah, Dennis Cooper, Amit Verma, Bihui Hilda Ye, Murali Janakiram, Roberto Alejandro Sica
{"title":"Outcome of Stem Cell Transplantation in HTLV-1-Associated North American Adult T-Cell Leukemia/Lymphoma.","authors":"Abdul-Hamid Bazarbachi,&nbsp;Daniel Reef,&nbsp;Hiba Narvel,&nbsp;Riya Patel,&nbsp;Rama Al Hamed,&nbsp;Sindhu Vikash,&nbsp;Karun Neupane,&nbsp;Eleftheria Atalla,&nbsp;Astha Thakkar,&nbsp;Shafia Rahman,&nbsp;Urvi Shah,&nbsp;Diego Adrianzen-Herrera,&nbsp;Ryann Quinn,&nbsp;Sumaira Zareef,&nbsp;Emma Rabinovich,&nbsp;Alyssa De Castro,&nbsp;Felisha Joseph,&nbsp;Kailyn Gillick,&nbsp;Jennat Mustafa,&nbsp;Fariha Khatun,&nbsp;Amanda Lombardo,&nbsp;Latoya Townsend-Nugent,&nbsp;Michelly Abreu,&nbsp;Nicole Chambers,&nbsp;Richard Elkind,&nbsp;Yang Shi,&nbsp;Yanhua Wang,&nbsp;Olga Derman,&nbsp;Kira Gritsman,&nbsp;Ulrich Steidl,&nbsp;Mendel Goldfinger,&nbsp;Noah Kornblum,&nbsp;Aditi Shastri,&nbsp;Ioannis Mantzaris,&nbsp;Liza Bachier-Rodriguez,&nbsp;Nishi Shah,&nbsp;Dennis Cooper,&nbsp;Amit Verma,&nbsp;Bihui Hilda Ye,&nbsp;Murali Janakiram,&nbsp;Roberto Alejandro Sica","doi":"10.1007/s44228-023-00032-y","DOIUrl":"https://doi.org/10.1007/s44228-023-00032-y","url":null,"abstract":"<p><p>Adult T-cell leukemia/lymphoma (ATLL) remains challenging to treat and has dismal outcome. Allogeneic stem-cell transplantation (allo-SCT) has promising results, but data remain scarce. In this single-center retrospective analysis of 100 patients with ATLL from north America (67 acute, 22 lymphomatous), 17 underwent allo-SCT and 5 autologous SCT (ASCT), with a median follow-up of 65 months. Post-transplant 3-years relapse incidence (RI) and non-relapse mortality (NRM) were 51% and 37%, respectively, and 3-year progression-free survival (PFS) and overall survival (OS) were 31% and 35%, respectively. ASCT 1-year RI was 80% compared to 30% in allo-SCT (p = 0.03). After adjusting for immortal-time bias, allo-SCT had significantly improved OS (HR = 0.4, p = 0.01). In exploratory multivariate analysis, patients achieving first complete response and Karnofsky score ≥ 90 had significantly better outcomes, as did Black patients, compared to Hispanics, who had worse outcome. In transplanted patients, 14 died within 2 years, 4 of which ASCT recipients. Our data are the largest ATLL transplant cohort presented to date outside of Japan and Europe. We show that allo-SCT, but not ASCT, is a valid option in select ATLL patients, and can induce long term survival, with 40% of patients alive after more than 5 years.</p>","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10241770/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9588767","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Venetoclax: A New Partner in the Novel Treatment Era for Acute Myeloid Leukemia and Myelodysplastic Syndrome. Venetoclax:急性髓系白血病和骨髓增生异常综合征新治疗时代的新伙伴。
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00041-x
Jean El-Cheikh, Ghassan Bidaoui, Mustafa Saleh, Nour Moukalled, Iman Abou Dalle, Ali Bazarbachi
{"title":"Venetoclax: A New Partner in the Novel Treatment Era for Acute Myeloid Leukemia and Myelodysplastic Syndrome.","authors":"Jean El-Cheikh,&nbsp;Ghassan Bidaoui,&nbsp;Mustafa Saleh,&nbsp;Nour Moukalled,&nbsp;Iman Abou Dalle,&nbsp;Ali Bazarbachi","doi":"10.1007/s44228-023-00041-x","DOIUrl":"https://doi.org/10.1007/s44228-023-00041-x","url":null,"abstract":"<p><strong>Background: </strong>Acute Myeloid Leukemia (AML) and Myelodysplastic Syndrome (MDS) are two closely related blood cancers that are more frequent in older adults. AML is the most common type of adult acute leukemia, and MDS is characterized by ineffective blood cell production and abnormalities in the bone marrow and blood. Both can be resistant to treatment, often due to dysfunction in the process of apoptosis, the body's natural mechanism for cell death. Venetoclax, an orally-administered medication that selectively targets the BCL-2 protein, has shown promise in enhancing treatment sensitivity in some hematological malignancies by reducing the apoptotic threshold. This review aims to evaluate the effectiveness of venetoclax in treating AML and MDS, as well as potential mechanisms of resistance to the medication.</p><p><strong>Methods: </strong>A literature search was conducted utilizing PUBMED to capture all relevant research articles on the use of venetoclax as a therapy for both diseases. The MeSH terms \"acute myeloid leukemia\", \"myelodysplastic syndrome\" and \"venetoclax\" were searched. Furthermore, Clinicaltrials.gov was accessed to ensure the inclusion of all ongoing clinical trials.</p><p><strong>Results: </strong>Although Venetoclax showed modest results as a single-agent therapy in AML, venetoclax-based combination therapies? mainly with hypomethylating agents or low-dose cytarabine? yielded significantly positive results. Preliminary results oN the use of venetoclax-based combination therapy with HMA, mainly azacitidine, in unfit high-risk MDS also yielded optimistic results. Identification of mutations for which various drugs have been approved has spurred active investigation of venetoclax in combination trials.</p><p><strong>Conclusion: </strong>Venetoclax-based combination therapies have been shown to induce rapid responses and increase overall survival in AML patients unfit for intensive chemotherapy. These therapies are also yielding positive preliminary results in high-risk MDS patients in phase I trials. Resistance to venetoclax and drug-related toxicity are two main obstacles that need to be overcome to reap the full benefits of this therapy.</p>","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10241752/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9593164","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
'Standing on the Shoulders of the Giants': Dr. Giampaolo Merlini. “站在巨人的肩膀上”:詹保罗·梅里尼博士。
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00039-5
Mohamad Mohty, Giampaolo Merlini
{"title":"'Standing on the Shoulders of the Giants': Dr. Giampaolo Merlini.","authors":"Mohamad Mohty,&nbsp;Giampaolo Merlini","doi":"10.1007/s44228-023-00039-5","DOIUrl":"https://doi.org/10.1007/s44228-023-00039-5","url":null,"abstract":"","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10241735/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9960914","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Racial Differences in Spinal Cord Compression Related Hospitalizations in Patients with Multiple Myeloma. 多发性骨髓瘤患者脊髓压迫相关住院治疗的种族差异
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00027-9
Samer Al Hadidi, Deepa Dongarwar, Carolina Schinke, Sharmilan Thanendrarajan, Maurizio Zangari, John D Shaughnessy, Frits van Rhee
{"title":"Racial Differences in Spinal Cord Compression Related Hospitalizations in Patients with Multiple Myeloma.","authors":"Samer Al Hadidi,&nbsp;Deepa Dongarwar,&nbsp;Carolina Schinke,&nbsp;Sharmilan Thanendrarajan,&nbsp;Maurizio Zangari,&nbsp;John D Shaughnessy,&nbsp;Frits van Rhee","doi":"10.1007/s44228-023-00027-9","DOIUrl":"https://doi.org/10.1007/s44228-023-00027-9","url":null,"abstract":"","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10241736/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9589064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Co-occurrence of Multiple Sclerosis and Severe Aplastic Anemia: A Report of Two Cases Successfully Treated with Allogeneic Hematopoietic Stem Cell Transplantation. 多发性硬化症合并严重再生障碍性贫血:异基因造血干细胞移植成功治疗2例报告。
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00028-8
Alfadil Haroon, Syed Osman Ahmed, Mahmoud Aljurf, Etedal AbuElbasher, Hazzaa Alzahrani
{"title":"Co-occurrence of Multiple Sclerosis and Severe Aplastic Anemia: A Report of Two Cases Successfully Treated with Allogeneic Hematopoietic Stem Cell Transplantation.","authors":"Alfadil Haroon,&nbsp;Syed Osman Ahmed,&nbsp;Mahmoud Aljurf,&nbsp;Etedal AbuElbasher,&nbsp;Hazzaa Alzahrani","doi":"10.1007/s44228-023-00028-8","DOIUrl":"https://doi.org/10.1007/s44228-023-00028-8","url":null,"abstract":"","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10241739/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9586846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Disseminated Invasive Mucormycosis Infection Following Autologous Stem Cell Transplantation for Diffuse Large B-Cell Lymphoma. 自体干细胞移植治疗弥漫性大b细胞淋巴瘤后播散性侵袭性毛霉病感染。
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00031-z
Edward R Scheffer Cliff, Gemma Reynolds, Andrew Grigg
{"title":"Disseminated Invasive Mucormycosis Infection Following Autologous Stem Cell Transplantation for Diffuse Large B-Cell Lymphoma.","authors":"Edward R Scheffer Cliff,&nbsp;Gemma Reynolds,&nbsp;Andrew Grigg","doi":"10.1007/s44228-023-00031-z","DOIUrl":"https://doi.org/10.1007/s44228-023-00031-z","url":null,"abstract":"<p><p>Invasive fungal infections (IFI) are challenging to predict, diagnose and treat, and are associated with a particularly high mortality among patients with hematological malignancies. They are relatively uncommon in patients with lymphoma, compared with those with acute leukemia or undergoing allogeneic transplantation. We present a patient, autografted for recurrent lymphoma, with fever and refractory diarrhea persisting post engraftment, eventually attributable to disseminated mucor infection. This case illustrates the challenge of timely diagnosis and initiation of treatment for IFI in lymphoma patients, who do not routinely receive antifungal prophylaxis, and the importance of aggressive investigation and symptom-directed tissue sampling for evidence of IFI in febrile immunocompromised hosts not responding to broad-spectrum antibiotics.</p>","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9905013/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9581801","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Clinical and Prognostic Significance of Ribonucleotide Reductase Subunits RRM1 and RRM2 mRNA Levels in Patients with Chronic Lymphocytic Leukemia. 慢性淋巴细胞白血病患者核糖核苷酸还原酶亚基RRM1和RRM2 mRNA水平的临床及预后意义
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00033-x
Sevastianos Chatzidavid, Christina-Nefeli Kontandreopoulou, Panagiotis T Diamantopoulos, Nefeli Giannakopoulou, Panagiota Katsiampoura, Christos Stafylidis, Georgios Dryllis, Marie-Christine Kyrtsonis, Maria Dimou, Panayiotis Panayiotidis, Nora-Athina Viniou
{"title":"The Clinical and Prognostic Significance of Ribonucleotide Reductase Subunits RRM1 and RRM2 mRNA Levels in Patients with Chronic Lymphocytic Leukemia.","authors":"Sevastianos Chatzidavid,&nbsp;Christina-Nefeli Kontandreopoulou,&nbsp;Panagiotis T Diamantopoulos,&nbsp;Nefeli Giannakopoulou,&nbsp;Panagiota Katsiampoura,&nbsp;Christos Stafylidis,&nbsp;Georgios Dryllis,&nbsp;Marie-Christine Kyrtsonis,&nbsp;Maria Dimou,&nbsp;Panayiotis Panayiotidis,&nbsp;Nora-Athina Viniou","doi":"10.1007/s44228-023-00033-x","DOIUrl":"https://doi.org/10.1007/s44228-023-00033-x","url":null,"abstract":"<p><p>Ribonucleotide Reductase (RNR) converts ribonucleotides to deoxyribonucleotides required for DNA replication and repair. RNR consists of subunits M1 and M2. It has been studied as a prognostic factor in several solid tumors and in chronic hematological malignancies, but not in chronic lymphocytic leukemia (CLL). Peripheral blood samples were collected from 135 CLL patients. M1/M2 gene mRNA levels were measured and expressed as a RRM1-2/GAPDH ratio. M1 gene promoter methylation was studied in a patients' subgroup. M1 mRNA expression was higher in patients without anemia (p = 0.026), without lymphadenopathy (p = 0.005) and 17p gene deletion (p = 0.031). Abnormal LDH (p = 0.022) and higher Rai stage (p = 0.019) were associated with lower M1 mRNA levels. Higher M2 mRNA levels were found in patients without lymphadenopathy (p = .048), Rai stage 0 (p = 0.025) and Trisomy 12 (p = 0.025). The correlation between RNR subunits and clinic-biological characteristics in CLL patients demonstrate RNR's potential role as a prognostic factor.</p>","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10241768/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9589562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Adult Onset Langerhans Cell Histiocytosis: Clinical Characteristics and Treatment Outcomes. 成人起病朗格汉斯细胞组织细胞增多症:临床特征和治疗结果。
Clinical Hematology International Pub Date : 2023-06-01 DOI: 10.1007/s44228-023-00034-w
Eren Arslan Davulcu, Nur Soyer, Zühal Demirci, Ajda Güneş, Filiz Vural, Fahri Şahin, Mahmut Töbü, Serra Kamer, Mine Hekimgil, Güray Saydam
{"title":"Adult Onset Langerhans Cell Histiocytosis: Clinical Characteristics and Treatment Outcomes.","authors":"Eren Arslan Davulcu,&nbsp;Nur Soyer,&nbsp;Zühal Demirci,&nbsp;Ajda Güneş,&nbsp;Filiz Vural,&nbsp;Fahri Şahin,&nbsp;Mahmut Töbü,&nbsp;Serra Kamer,&nbsp;Mine Hekimgil,&nbsp;Güray Saydam","doi":"10.1007/s44228-023-00034-w","DOIUrl":"https://doi.org/10.1007/s44228-023-00034-w","url":null,"abstract":"<p><strong>Purpose: </strong>Langerhans cell histiocytosis (LCH) is a rare disease that can affect all tissues and organs. Our study evaluated the clinical characteristics and treatment outcomes of adult-onset LCH patients in a tertiary center.</p><p><strong>Materials and methods: </strong>Adult patients diagnosed with LCH were retrospectively evaluated. Their initial symptoms, stratification according to disease involvement, treatment details, treatment responses, and overall and progression-free survival (PFS) were analyzed.</p><p><strong>Results: </strong>Thirty-three patients were included. There were 21 single system LCH, 10 multisystem LCH, and 2 pulmonary LCH patients. Patients with single system unifocal involvement were successfully treated with local therapies such as surgery and radiotherapy. Most of the multisystem LCH patients and patients with single system multifocal involvement were treated with systemic chemotherapy. Cladribine was the first choice in 10 out of 11 patients who received chemotherapy. Among all patients, the overall response rate (ORR) was 97%. Among those who had cladribine in the first-line the ORR was 81%. All these patients achieved a complete remission and were alive at the last visit. The median follow-up was 38 (range, 2-183) months. The median PFS has not yet been reached. Ten-year PFS was 90.9%.</p><p><strong>Conclusion: </strong>Besides successful local treatments with surgery and radiotherapy, our study provides information for front-line cladribine treatment.</p>","PeriodicalId":10368,"journal":{"name":"Clinical Hematology International","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10241753/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9580041","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
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