Turkish Journal of Hematology最新文献

{"title":"Amplification of the <i>BCR::ABL1</i> Fusion Gene: A Rare Phenomenon in B-cell Acute Lymphoblastic Leukemia.","authors":"Debadrita Ray,&nbsp;Praveen Sharma,&nbsp;Arihant Jain,&nbsp;Sreejesh Sreedharanunni","doi":"10.4274/tjh.galenos.2023.2023.0212","DOIUrl":"10.4274/tjh.galenos.2023.2023.0212","url":null,"abstract":"clustered manner (Figure 1B) in 70% of the interphase nuclei, consistent with the amplification of the BCR::ABL1 fusion gene. The patient died within a month of the diagnosis. Additional Philadelphia (Ph) chromosomes are frequently seen in B-ALL and in cases of disease progression in chronic myeloid leukemia (CML). However, multiple extra copies of the BCR::ABL1 fusion gene are rare genetic occurrences in CML signifying disease progression and imatinib resistance. This results from a second Ph chromosome, double minutes, isoderivative chromosome 22, and isodicentric Ph chromosome [1,2]. Although extra copies of the BCR::ABL1 fusion gene were reported previously in T-ALL, they have never been documented in B-ALL [3]. This signal pattern highlights the need for FISH or other conventional cytogenetic approaches over reverse-transcriptase polymerase chain reaction studies to confirm disease progression.","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"204-205"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/53/72/TJH-40-204.PMC10476253.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10157690","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"“Rod-Like” Cytoplasmic Crystals of Peripheral Lymphocytes in Chronic Lymphocytic Leukemia","authors":"Jihong Hao,&nbsp;Jie Gao,&nbsp;Yiping Yang,&nbsp;Ziyi An,&nbsp;Xiaoqiang Lian","doi":"10.4274/tjh.galenos.2023.2023.0051","DOIUrl":"10.4274/tjh.galenos.2023.2023.0051","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"202-203"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/73/fa/TJH-40-202.PMC10476252.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10160356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"<i>JAK2</i> p.V617F Variants in Non-Blood DNA from Patients with Polycythemia Vera","authors":"Naoki Mori,&nbsp;Mari Ohwashi-Miyazaki,&nbsp;Kentaro Yoshinaga,&nbsp;Masayuki Shiseki,&nbsp;Junji Tanaka","doi":"10.4274/tjh.galenos.2023.2023-0159","DOIUrl":"10.4274/tjh.galenos.2023.2023-0159","url":null,"abstract":"The p.V617F mutation of the Janus kinase 2 ( JAK2 ) gene has been identified in 95% of patients with polycythemia vera (PV) and in half of patients with essential thrombocythemia (ET)","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"220-222"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/66/56/TJH-40-220.PMC10476263.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10160358","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute and Persistent Remission of Aggressive Natural Killer Cell Leukemia in an Older Patient Induced by Chidamide Combined with Cyclophosphamide, Vindesine, Prednisone, and Etoposide Therapy","authors":"Qingqing Lin,&nbsp;Renzhi Pei,&nbsp;Ying Lu","doi":"10.4274/tjh.galenos.2023.2023.0227","DOIUrl":"10.4274/tjh.galenos.2023.2023.0227","url":null,"abstract":"Aggressive natural killer cell leukemia (ANKL) is a fulminant disease with a median overall survival of 2 months [1,2]. Although induction therapy with an L-asparaginase-based combined chemotherapy regimen followed by allogeneic hematologic stem cell transplantation improves clinical survival, the overall success of this approach appears rather limited [3,4]. This limitation is even more pronounced in older patients who are unable to tolerate intensive chemotherapy. Histone deacetylase inhibitors have been identified by Dufva et al. [5] as ideal drug candidates in the management of ANKL. Here we report a case of an older patient with ANKL who was treated successfully with chidamide combined with conventional chemotherapy with no significant toxicity arising.","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"225-226"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9f/f1/TJH-40-225.PMC10476257.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10214531","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fungal Infections and Myeloma: Supports","authors":"İrfan Yavaşoğlu","doi":"10.4274/tjh.galenos.2023.2023-0255","DOIUrl":"10.4274/tjh.galenos.2023.2023-0255","url":null,"abstract":"a","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"206-207"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a2/18/TJH-40-206.PMC10476259.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10159322","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Level of Awareness of Acquired Hemophilia A Among Physicians in Türkiye: A Survey Study","authors":"Ahmet Muzaffer Demir,&nbsp;Muhlis Cem Ar,&nbsp;Fahri Şahin,&nbsp;Merve Altunbaş","doi":"10.4274/tjh.galenos.2023.2023.0236","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023.0236","url":null,"abstract":"<p><p>This survey study aimed to evaluate the level of awareness and knowledge of acquired hemophilia A (AHA) among physicians from various specialties. Data were collected by administering a questionnaire containing questions about two patient profiles to evaluate the approaches of physicians from different specialties. The study included a total of 945 physicians. The most common suspected diagnosis for the clinical patient profile was immune thrombocytopenia, followed by hemophilia. For the laboratory patient profile, the most common suspected diagnosis was hemophilia. While only 9.4% of the physicians stated that they had good knowledge of this disease, only 21.6% wanted to learn more about AHA. Most of the participating physicians do not rank AHA first in the differential diagnosis of a patient with clinical and laboratory findings of AHA. Appropriate educational activities to raise awareness of AHA will help reduce disease-related morbidity and mortality.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"197-201"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1d/e1/TJH-40-197.PMC10476256.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10157691","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hemophagocytic Lymphohistiocytosis in <i>RUNX1::RUNX1T1</i> Positive AML with Blast Count Below 20%","authors":"Xingqin Huang,&nbsp;Fang Long,&nbsp;Yun Zhang,&nbsp;Xiaopeng Gao,&nbsp;Ting Li","doi":"10.4274/tjh.galenos.2023.2023.0155","DOIUrl":"10.4274/tjh.galenos.2023.2023.0155","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"210-212"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/27/34/TJH-40-210.PMC10476245.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10513011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autoinflammation and Myelofibrosis: Report of a Case","authors":"Prasad R. Koduri,&nbsp;Amina Shaik,&nbsp;Durga Rao Vegulada","doi":"10.4274/tjh.galenos.2023.2023.0064","DOIUrl":"10.4274/tjh.galenos.2023.2023.0064","url":null,"abstract":"Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease of unknown etiology [1,2]. We describe the case of a young woman with AOSD who presented with extreme anemia and was diagnosed with secondary myelofibrosis. Treatment with prednisolone resulted in durable complete clinical and hematological response. She remains in excellent health, with normal blood counts over the course of 4 years of follow-up.","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"223-224"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9d/d8/TJH-40-223.PMC10476249.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10513009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Circ_0001946 Promotes the Development of Acute Myeloid Leukemia by Upregulating PDL1","authors":"Guohui Li,&nbsp;Conghui Zhu,&nbsp;Dan Qiao,&nbsp;Renan Chen","doi":"10.4274/tjh.galenos.2023.2022.0484","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2022.0484","url":null,"abstract":"<p><strong>Objective: </strong>Circ_0001946 has been identified as an oncogenic factor, and the aim of this study was to explore the detailed roles and putative targets of circ_0001946 in acute myeloid leukemia (AML).</p><p><strong>Materials and methods: </strong>Levels of circ_0001946 were examined in AML tissues and cells. Furthermore, the regulatory functions of circ_0001946 in AML were explored. The expression of circ_0001946 was evaluated in AML samples and a matched para-carcinoma control, as well as in AML cell lines and a human bone marrow stromal cell line using reverse transcription-quantitative polymerase chain reaction. Cell proliferation was examined using a CCK-8 kit, and migration/invasion was measured by transwell assay. Furthermore, interactions between associated molecules were assessed using RNA pulldown, and the mRNA stability of the relevant gene was examined by mRNA stability assay.</p><p><strong>Results: </strong>Our data indicated that circ_0001946 was upregulated in AML specimens/cells. Additionally, overexpression of circ_0001946 promoted the proliferation, migration, and invasion of AML cells and, vice versa, these biological processes were suppressed by knockdown of circ_0001946. Furthermore, PDL1 is a potential downstream molecule of circ_0001946 in AML and its stability was improved by circ_0001946. The expression of PDL1 was increased in AML specimens and positively correlated with circ_0001946 expression. Moreover, biological behavioral alterations in AML cells induced by oe-circ_0001946 were abrogated by sh-PDL1 and the effects of sh-circ_0001946 were enhanced by treatment with sh-PDL1.</p><p><strong>Conclusion: </strong>Taken together, these data suggest that levels of circ_0001946 are elevated in AML and that circ_0001946 could promote the growth of AML cells. Furthermore, PDL1 is a novel downstream molecule of circ_0001946 in AML. Circ_0001946/PDL1 signaling may play crucial roles in tumor progression in AML and could be a novel candidate for targeted treatments for AML patients.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"154-161"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/89/3c/TJH-40-154.PMC10476255.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10157594","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correlation of Peripheral Chimeric Antigen Receptor T-cell (CAR-T Cell) mRNA Expression Levels with Toxicities and Outcomes in Patients with Diffuse Large B-cell Lymphoma","authors":"Christian Messerli,&nbsp;Gertrud Wiedemann,&nbsp;Naomi Porret,&nbsp;Michael Nagler,&nbsp;Katja Seipel,&nbsp;Barbara Jeker,&nbsp;Urban Novak,&nbsp;Sacha Zeerleder,&nbsp;Ulrike Bacher,&nbsp;Thomas Pabst","doi":"10.4274/tjh.galenos.2023.2023.0136","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023.0136","url":null,"abstract":"<p><p>Cytokine-release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS) are significant complications in patients with relapsed/refractory diffuse large B-cell lymphoma undergoing chimeric antigen receptor T-cell (CAR-T cell) therapy. However, it remains unclear whether CAR-T cell expression itself is clinically relevant. We assessed CAR-T cell mRNA expression and DNA concentration by digital droplet PCR in peripheral blood from 14 sequential CAR-T cell recipients. Patients were grouped according to CAR-T cell peak expression. Patients with high CAR-T cell peak expression (8 patients; 57%) had higher rates of ICANS (p=0.0308) and intensive care unit admission (p=0.0404), longer durations of hospitalization (p=0.0077), and, although not statistically significant, a higher rate of CRS (p=0.0778). There was a correlation of CAR-T cell mRNA expression with DNA concentration, but CAR-T cell expression levels failed to correlate to response or survival. Our data suggest that higher CAR-T cell peak mRNA expression is associated with increased risk for ICANS and possibly CRS, requiring further investigation in larger studies.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"40 3","pages":"187-196"},"PeriodicalIF":2.6,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/41/23/TJH-40-187.PMC10476258.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10513008","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}