{"title":"Identifying Risk Factors and Improving Preventive Strategies for Febrile Neutropenia in Children with Leukemia Receiving Ciprofloxacin Prophylaxis","authors":"Zühre Kaya, Nurettin Alıcı, Serap Kırkız, Ülker Koçak","doi":"10.4274/tjh.galenos.2023.2023.0116","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023.0116","url":null,"abstract":"<p><p>The purpose of this study was to identify risk factors and improve preventive strategies for febrile neutropenia (FEN) in children with leukemia who were receiving ciprofloxacin prophylaxis. The study included 100 children with leukemia [n=80 with acute lymphoblastic leukemia and n=20 with acute myeloblastic leukemia (AML)]. Patients were divided into two groups based on whether they had three or fewer FEN episodes (Group 1) or more than three FEN episodes (Group 2). Group 1 contained 63 (63%) of the 100 patients, while Group 2 contained 37 (37%). Older age (≥7 years), leukemia type, prolonged neutropenia (>10 days), and the presence of neutropenia and hypogammaglobulinemia at diagnosis were all risk factors for having more than three FEN episodes. Our findings suggest that, in addition to ciprofloxacin prophylaxis, identifying risk factors and improving preventive strategies could help reduce FEN episodes in children with leukemia.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"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/7c/7e/TJH-40-183.PMC10476261.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10511966","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":"Etiology and Factors Affecting Severe Complications and Mortality of Febrile Neutropenia in Children with Acute Leukemia","authors":"İrem Ceren Erbaş, Ayşe Çakıl Güzin, Şilem Özdem Alataş, Hatice Karaoğlu Asrak, İlknur Akans, Şefika Akyol, Canan Özlü, Özlem Tüfekçi, Şebnem Yılmaz, Hale Ören, Nurşen Belet","doi":"10.4274/tjh.galenos.2023.2023.0185","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023.0185","url":null,"abstract":"<p><strong>Objective: </strong>Febrile neutropenia (FN) is an important complication that causes high rates of morbidity and mortality in patients with malignancies. We aimed to investigate the etiology, epidemiological distribution and its change over the years, clinical courses, and outcomes of FN in children with acute leukemia.</p><p><strong>Materials and methods: </strong>We retrospectively analyzed the demographic data, clinical characteristics, laboratory results, severe complications, and mortality rates of pediatric patients with FN between January 2010 and December 2020.</p><p><strong>Results: </strong>In 153 patients, a total of 450 FN episodes (FNEs) occurred. Eighty-four (54.9%) of these patients were male, the median age of the patients was 6.5 (range: 3-12.2) years, and 127 patients (83%) were diagnosed with acute lymphoblastic leukemia. Fever with a focus was found in approximately half of the patients, and an etiology was identified for 38.7% of the patients. The most common fever focus was bloodstream infection (n=74, 16.5%). Etiologically, a bacterial infection was identified in 22.7% (n=102), a viral infection in 13.3% (n=60), and a fungal infection in 5.8% (n=26) of the episodes. Twenty-six (23.2%) of a total of 112 bacteria were multidrug resistant (MDR) The rate of severe complications was 7.8% (n=35) and the mortality rate was 2% (n=9). In logistic regression analysis, refractory/relapsed malignancies and high C-reactive protein (CRP) at first admission were found to be the most important independent risk factors for mortality. Prolonged neutropenia after chemotherapy, diagnosis of acute myeloid leukemia, identification of fever focus or etiological agents, invasive fungal infections, polymicrobial infections, and need for intravenous immunoglobulin treatment increased the frequency of severe complications.</p><p><strong>Conclusion: </strong>We found that there was no significant change in the epidemiological distribution or frequency of resistant bacteria in our center in the last 10 years compared to previous years. Prolonged duration of fever, relapsed/refractory malignancies, presence of fever focus, and high CRP level were significant risk factors for poor clinical course and outcome.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"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/ec/1d/TJH-40-143.PMC10476243.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10215099","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":"Aleukemic Variant of Mast Cell Leukemia with del (7)(q31): Rare Case Report of an Elderly Chinese Man","authors":"Xiaoying Song, Yiping Yang, Zhanlong Wang, Jihong Hao","doi":"10.4274/tjh.galenos.2023.2022.0456","DOIUrl":"10.4274/tjh.galenos.2023.2022.0456","url":null,"abstract":"81-year-old","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"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/e2/35/TJH-40-216.PMC10476254.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10511965","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":"Clinical Impact of <i>JAK2V617F</i> Allele Burden in Philadelphia-Negative Myeloproliferative Neoplasms","authors":"İpek Yönal-Hindilerden, Ezgi Şahin, Fehmi Hindilerden, Aynur Dağlar-Aday, Meliha Nalçacı","doi":"10.4274/tjh.galenos.2023.2023.0169","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023.0169","url":null,"abstract":"<p><strong>Objective: </strong>The impact of <i>JAK2V617F</i> allele burden on clinical course in Philadelphia-negative (Ph-negative) myeloproliferative neoplasms (MPNs) is not clear. We analyzed the clinical impact of <i>JAK2V617F</i> allele burden in a relatively large series of patients with Ph-negative MPNs and long-term follow-up.</p><p><strong>Materials and methods: </strong>A total of 228 patients with Ph-negative MPNs, including 118 with essential thrombocythemia (ET), 84 with primary myelofibrosis (PMF), and 26 with polycythemia vera (PV), were analyzed. The JAK2 MutaScreen assay was used to quantify <i>JAK2V617F</i> allele burden in genomic DNA.</p><p><strong>Results: </strong>In PV cases, high <i>JAK2V617F</i> allele burden was associated with a trend towards inferior overall survival. In ET, high <i>JAK2V617F</i> allele burden was associated with lower hemoglobin and hematocrit levels, higher lactate dehydrogenase (LDH) levels, larger spleen size, and increased bleeding and mortality rates. In PMF, high <i>JAK2V617F</i> allele burden was associated with higher leukocyte counts and larger spleen size. In the entire cohort, high allele burden was associated with higher leukocyte and lower platelet counts, higher LDH levels, larger spleen size, higher percentage of bleeding events, higher death rate, and inferior overall survival.</p><p><strong>Conclusion: </strong>Our results suggest that high <i>JAK2V617F</i> allele burdens are associated with more severe disease in PV and ET. In PMF, high <i>JAK2V617F</i> allele burdens were associated with more pronounced myeloproliferative phenotypes. In Ph-negative MPNs, high allele burdens were associated with more aggressive phenotypes. Our data with a long follow-up period support the possibility of <i>JAK2V617F</i> allele burden being used as a marker for predicting clinical phenotype in cases of Ph-negative MPNs.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"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/1f/55/TJH-40-174.PMC10476250.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10215666","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}
Jihong Hao, Jie Gao, Yiping Yang, Ziyi An, Xiaoqiang Lian
{"title":"“Rod-Like” Cytoplasmic Crystals of Peripheral Lymphocytes in Chronic Lymphocytic Leukemia","authors":"Jihong Hao, Jie Gao, Yiping Yang, Ziyi An, Xiaoqiang Lian","doi":"10.4274/tjh.galenos.2023.2023-0051","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023-0051","url":null,"abstract":"could be observed in the cytoplasm or covering the nuclei in lymphocytes (Figures 1A-1D). Abdominal computed tomography showed multiple enlarged lymph nodes in the abdomen and groin. Flow cytometry analysis revealed the expression of CD19, CD20, CD5, CD22, CD23, CD24, CD38, lambda, FCM7 (dim, 38.6%), and CD200 (high). Previous studies have suggested that the formation of crystals may be related to dysfunctional immunoglobulin (Ig) synthesis [1,2,3,4]. In our case, the levels of IgG, IgA, and IgM were all reduced, with the most significant reduction seen for IgG at 4.96 g/L. While some observations of similar rod-like structures have been reported in cases of B-cell lymphoproliferative diseases, intracellular rod-like crystals in mature lymphocytes in CLL are extremely rare in peripheral blood [5,6,7]. Here, the crystals in the lymphocytes were present after anti-leukemia therapy. Whether the formation of these crystals may be related to chemotherapy drugs needs to be confirmed.","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2023-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47490944","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"In Vitro FVIII-Encoding Transgenic Mesenchymal Stem Cells Maintain Successful Coagulation in FVIII-Deficient Plasma Mimicking Hemophilia A","authors":"Cansu Hemşinlioğlu, Elif Sibel Aslan, Cihan Taştan, Didem Çakırsoy, Raife Dilek Turan, Utku Seyis, Muhammer Elek, Gözde Sir Karakuş, Ömur Selin Günaydın, Selen Abanuz, Derya Dilek Kançağı, Bulut Yurtsever, Koray Yalçın, Murat Kasap, Ercüment Ovalı","doi":"10.4274/tjh.galenos.2023.2022.0318","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2022.0318","url":null,"abstract":"Hemophilia A is an X-linked recessive bleeding disorder caused by a deficiency of plasma coagulation factor VIII (FVIII), and it accounts for about 80%-85% of all cases of hemophilia. Plasma-derived therapies or recombinant FVIII concentrates are used to prevent and treat the bleeding symptoms along with FVIII-mimicking antibodies. Recently, the European Medicines Agency granted conditional marketing approval for the first gene therapy for hemophilia A. The aim of this study was to determine the effectiveness of coagulation in correcting FVIII deficiency with FVIII-secreting transgenic mesenchymal stem cells (MSCs).A lentiviral vector encoding a B domain-deleted FVIII cDNA sequence with CD45R0 truncated (CD45R0t) surface marker was designed to develop a transgenic FVIII-expressing primary cell line by transducing MSCs. The efficacy and functionality of the FVIII secreted from the MSCs was assessed with anti-FVIII ELISA, CD45R0t flow cytometry, FVIII western blot, and mixing test analysis in vitro.The findings of this study showed that the transgenic MSCs maintained persistent FVIII secretion. There was no significant difference in FVIII secretion over time, suggesting stable FVIII expression from the MSCs. The functionality of the FVIII protein secreted in the MSC supernatant was demonstrated by applying a mixing test in coagulation analysis. In the mixing test analysis, FVIII-deficient human plasma products were mixed with either a saline control or FVIII-secreted MSC supernatant. The mean FVIII level of the saline control group was 0.41±0.03 IU/dL, whereas the mean level was 25.41±33.38 IU/dL in the FVIII-secreting MSC supernatant mixed group (p<0.01). The mean activated partial thromboplastin time (aPTT) of the saline control group was 92.69±11.38 s, while in the FVIII-secreting MSC supernatant mixed group, the mean aPTT level decreased to 38.60±13.38 s (p<0.001).The findings of this in vitro study suggest that the new method presented here is promising as a possible treatment for hemophilia A. Accordingly, a study of FVIII-secreting transgenic MSCs will next be initiated in a FVIII-knockout animal model.Hemofili A, pıhtılaşma faktörü VIII’in (FVIII) eksikliğine bağlı gelişen, hemofili hastalarının yaklaşık %80-85’ini oluşturan, X’e bağlı resesif geçiş gösteren bir kanama bozukluğudur. Kanama semptomlarını önlemek ve tedavi etmek için plazma kaynaklı tedaviler ya da rekombinant FVIII konsantreleri ile FVIII’i taklit eden monoklonal antikorlar kullanılmaktadır. Son zamanlarda EMA, hemofili A’nın ilk gen tedavisi için koşullu pazarlama onayı vermiştir. Bu çalışmada, hemofili A hastalığından sorumlu olan FVIII eksikliğini düzeltebilmek amacıyla FVIII salgılayan transgenik mezenkimal kök hücreler (MKH) ile koagülasyon etkinliğinin değerlendirilmesi amaçlanmaktadır.CD45R0 truncated (CD45R0t) yüzey belirteci ile B domaini silinmiş FVIII cDNA dizisini kodlayan bir lentiviral vektör, MKH’leri transdükte ederek transgenik FVIII ekspresyonu sağlayan bir","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135479067","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Ibrutinib-Induced Pancreatitis in Patients with Waldenstrom Macroglobulinemia","authors":"Nurhilal Büyükkurt, Barış Soydaş","doi":"10.4274/tjh.galenos.2023.2023-0109","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023-0109","url":null,"abstract":"Waldenstrom macroglobulinemia (WM) is a rare disorder [1]. The combination of rituximab and ibrutinib has been associated with prolonged progression-free survival in previously treated and untreated cases of WM [2]. Unfortunately, however, adverse effects due to off-target interactions are disadvantages of ibrutinib. In this context, we would like to present a case of pancreatitis in a patient undergoing ibrutinib treatment. The mechanism of pancreatitis may be direct toxicity, hypertriglyceridemia, or immune-mediated injury to the liver, pancreas, or blood supply by direct injection [3]. Although a single case of pancreatitis was reported in a phase 1/1b study that included 48 patients, ibrutinib-associated pancreatitis has not yet been reported in the literature as a real-life clinical experience [4].","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2023-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/95/ab/TJH-40-141.PMC10240158.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9581080","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}
Yusuf Yaşar, Zühre Kaya, Esra Güney, Serap Kirkiz, Ülker Koçak
{"title":"Assessment of the Knowledge, Attitudes, Anxiety, and Coping Strategies of Pediatric Patients and Parents after Leukemia Diagnosis in Türkiye","authors":"Yusuf Yaşar, Zühre Kaya, Esra Güney, Serap Kirkiz, Ülker Koçak","doi":"10.4274/tjh.galenos.2023.2023.0043","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2023.0043","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2023-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ae/a9/TJH-40-139.PMC10240164.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9937202","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":"In Vitro FVIII-Encoding Transgenic Mesenchymal Stem Cells Maintain Successful Coagulation in FVIII-Deficient Plasma Mimicking Hemophilia A","authors":"Cansu Hemşinlioğlu, Elif Sibel Aslan, Cihan Taştan, Didem Çakırsoy, Raife Dilek Turan, Utku Seyis, Muhammer Elek, Gözde Sir Karakuş, Ömur Selin Günaydın, Selen Abanuz, Derya Dilek Kançağı, Bulut Yurtsever, Koray Yalçın, Murat Kasap, Ercüment Ovalı","doi":"10.4274/tjh.galenos.2023.2022-0318","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2023.2022-0318","url":null,"abstract":"<p><strong>Objective: </strong>Hemophilia A is an X-linked recessive bleeding disorder caused by a deficiency of plasma coagulation factor VIII (FVIII), and it accounts for about 80%-85% of all cases of hemophilia. Plasma-derived therapies or recombinant FVIII concentrates are used to prevent and treat the bleeding symptoms along with FVIII-mimicking antibodies. Recently, the European Medicines Agency granted conditional marketing approval for the first gene therapy for hemophilia A. The aim of this study was to determine the effectiveness of coagulation in correcting FVIII deficiency with FVIII-secreting transgenic mesenchymal stem cells (MSCs).</p><p><strong>Materials and methods: </strong>A lentiviral vector encoding a B domain-deleted FVIII cDNA sequence with CD45R0 truncated (CD45R0t) surface marker was designed to develop a transgenic FVIII-expressing primary cell line by transducing MSCs. The efficacy and functionality of the FVIII secreted from the MSCs was assessed with anti-FVIII ELISA, CD45R0t flow cytometry, FVIII western blot, and mixing test analysis in vitro.</p><p><strong>Results: </strong>The findings of this study showed that the transgenic MSCs maintained persistent FVIII secretion. There was no significant difference in FVIII secretion over time, suggesting stable FVIII expression from the MSCs. The functionality of the FVIII protein secreted in the MSC supernatant was demonstrated by applying a mixing test in coagulation analysis. In the mixing test analysis, FVIII-deficient human plasma products were mixed with either a saline control or FVIII-secreted MSC supernatant. The mean FVIII level of the saline control group was 0.41±0.03 IU/dL, whereas the mean level was 25.41±33.38 IU/dL in the FVIII-secreting MSC supernatant mixed group (p<0.01). The mean activated partial thromboplastin time (aPTT) of the saline control group was 92.69±11.38 s, while in the FVIII-secreting MSC supernatant mixed group, the mean aPTT level decreased to 38.60±13.38 s (p<0.001).</p><p><strong>Conclusion: </strong>The findings of this in vitro study suggest that the new method presented here is promising as a possible treatment for hemophilia A. Accordingly, a study of FVIII-secreting transgenic MSCs will next be initiated in a FVIII-knockout animal model.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2023-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/cb/34/TJH-40-118.PMC10240160.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9954042","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}