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Article: Chronic Neutrophilic Leukemia: A Comprehensive Review of Clinical Characteristics, Genetic Landscape and Management.

Thomopoulos, Thomas P / Symeonidis, Argiris / Kourakli, Alexandra / Papageorgiou, Sotirios G / Pappa, Vasiliki

2022 Volume 12, Page(s) 891961

Abstract: Chronic neutrophilic leukemia (CNL) represents a rare disease, that has been classified among the BCR/ABL-negative myeloproliferative neoplasms. The disease is characterized by marked leukocytosis with absolute neutrophilia and its clinical presentation ... ...

Abstract	Chronic neutrophilic leukemia (CNL) represents a rare disease, that has been classified among the BCR/ABL-negative myeloproliferative neoplasms. The disease is characterized by marked leukocytosis with absolute neutrophilia and its clinical presentation may vary from asymptomatic to highly symptomatic with massive splenomegaly and constitutional symptoms. CNL prognosis remains relatively poor, as most patients succumb to disease complications or transform to acute myeloid leukemia. Recent studies have demonstrated that
Language	English
Publishing date	2022-04-14
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2649216-7
ISSN	2234-943X
ISSN	2234-943X
DOI	10.3389/fonc.2022.891961
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Article: Allogeneic Hematopoietic Stem Cell Transplantation for Mixed or Overlap Myelodysplastic/Myeloproliferative Disorders.

Symeonidis, Argiris / Chondropoulos, Spiros / Verigou, Evgenia / Lazaris, Vasileios / Kourakli, Alexandra / Tsirigotis, Panagiotis

Frontiers in oncology

2022 Volume 12, Page(s) 884723

Abstract: Chronic myelomonocytic leukemia (CMML) and the remaining, less frequent hybrid, mixed, or overlap myelodysplastic syndromes/myeloproliferative neoplasms (MDSs/MPNs) are difficult to treat neoplastic hematological disorders, exhibiting substantial ... ...

Abstract	Chronic myelomonocytic leukemia (CMML) and the remaining, less frequent hybrid, mixed, or overlap myelodysplastic syndromes/myeloproliferative neoplasms (MDSs/MPNs) are difficult to treat neoplastic hematological disorders, exhibiting substantial clinical and prognostic heterogeneity, for which clear therapeutic guidelines or effective treatment options are still missing. CMML has an overall survival ranging from a few months to several years. Although patients with proliferative or dysplastic features may benefit from hydroxyurea and hypomethylating agent treatment, respectively, none of these treatments can establish long-term remission and prevent the inevitable transformation to acute leukemia. Novel targeted treatment approaches are emerging but are still under investigation. Therefore, currently, allogeneic stem cell transplantation (allo-SCT) remains the only treatment modality with a curative potential, but its widespread application is limited, due to significant morbidity and mortality associated with the procedure, especially in the elderly and in patients with comorbidities. Recognition of patient eligibility for allo-SCT is crucial, and the procedure should be addressed to patients with a good performance status without severe comorbidities and mainly to those in intermediate- to high-risk category, with a suitable stem cell donor available. The issues of best timing for performing transplantation, patient and donor eligibility, the type of conditioning regimen, and the outcomes after various allo-SCT procedures are the topics of this review.
Language	English
Publishing date	2022-08-05
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2649216-7
ISSN	2234-943X
ISSN	2234-943X
DOI	10.3389/fonc.2022.884723
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Article: A Cross-Sectional, Multicentric, Disease-Specific, Health-Related Quality of Life Study in Greek Transfusion Dependent Thalassemia Patients.

Klonizakis, Philippos / Roy, Noémi / Papatsouma, Ioanna / Mainou, Maria / Christodoulou, Ioanna / Pantelidou, Despina / Kokkota, Smaro / Diamantidis, Michael / Kourakli, Alexandra / Lazaris, Vasileios / Andriopoulos, Dimitrios / Tsapas, Apostolos / Klaassen, Robert J / Vlachaki, Efthymia

Healthcare (Basel, Switzerland)

2024 Volume 12, Issue 5

Abstract: The assessment of health-related quality of life (HRQoL) in thalassemia offers a holistic approach to the disease and facilitates better communication between physicians and patients. This study aimed to evaluate the HRQoL of transfusion-dependent ... ...

Abstract	The assessment of health-related quality of life (HRQoL) in thalassemia offers a holistic approach to the disease and facilitates better communication between physicians and patients. This study aimed to evaluate the HRQoL of transfusion-dependent thalassemia (TDT) patients in Greece. This was a multicentric, cross-sectional study conducted in 2017 involving 283 adult TDT patients. All participants completed a set of two QoL questionnaires, the generic SF-36v2 and the disease-specific TranQol. Demographic and clinical characteristics were used to predefine patient subgroups. Significant factors identified in the univariate analysis were entered into a multivariate analysis to assess their effect on HRQoL. The SF-36 scores of TDT patients were consistently lower compared to the general population in Greece. The mean summary score of TranQol was relatively high (71 ± 14%), exceeding levels observed in national surveys in other countries. Employment emerged as the most significant independent factor associated with better HRQoL, whereas age had the most significant negative effect. This study represents the first comprehensive QoL assessment of a representative sample of the TDT population in Greece. The implementation of TranQol allowed for the quantification of HRQoL in Greece, establishing a baseline for future follow-up, and identifying more vulnerable patient subgroups.
Language	English
Publishing date	2024-02-22
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2721009-1
ISSN	2227-9032
ISSN	2227-9032
DOI	10.3390/healthcare12050524
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Article ; Online: Common cardiovascular biomarkers can independently predict outcome of patients with Myelodysplastic syndromes.

Mitroulis, Ioannis / Papadopoulos, Vasileios / Lamprianidou, Eleftheria / Mirtschink, Peter / Liapis, Konstantinos / Zafeiropoulou, Kalliopi / Kourakli, Alexandra / Moysiadis, Theodoros / Papoutselis, Menelaos / Vrachiolias, George / Symeonidis, Argiris / Kotsianidis, Ioannis

Blood cancer journal

2023 Volume 13, Issue 1, Page(s) 64

MeSH term(s)	Humans ; Biomarkers ; Myelodysplastic Syndromes/diagnosis ; Prognosis
Chemical Substances	Biomarkers
Language	English
Publishing date	2023-05-03
Publishing country	United States
Document type	Letter ; Research Support, Non-U.S. Gov't
ZDB-ID	2600560-8
ISSN	2044-5385 ; 2044-5385
ISSN (online)	2044-5385
ISSN	2044-5385
DOI	10.1038/s41408-023-00844-4
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Article ; Online: Genome-wide analysis toward the epigenetic aetiology of myelodysplastic syndrome disease progression and pharmacoepigenomic basis of hypomethylating agents drug treatment response.

Siamoglou, Stavroula / Boers, Ruben / Koromina, Maria / Boers, Joachim / Tsironi, Anna / Chatzilygeroudi, Theodora / Lazaris, Vasileios / Verigou, Evgenia / Kourakli, Alexandra / van IJcken, Wilfred F J / Gribnau, Joost / Symeonidis, Argiris / Patrinos, George P

Human genomics

2023 Volume 17, Issue 1, Page(s) 37

Abstract: Myelodysplastic syndromes (MDS) consist of a group of hematological malignancies characterized by ineffective hematopoiesis, cytogenetic abnormalities, and often a high risk of transformation to acute myeloid leukemia (AML). So far, there have been only ... ...

Abstract	Myelodysplastic syndromes (MDS) consist of a group of hematological malignancies characterized by ineffective hematopoiesis, cytogenetic abnormalities, and often a high risk of transformation to acute myeloid leukemia (AML). So far, there have been only a very limited number of studies assessing the epigenetics component contributing to the pathophysiology of these disorders, but not a single study assessing this at a genome-wide level. Here, we implemented a generic high throughput epigenomics approach, using methylated DNA sequencing (MeD-seq) of LpnPI digested fragments to identify potential epigenomic targets associated with MDS subtypes. Our results highlighted that PCDHG and ZNF gene families harbor potential epigenomic targets, which have been shown to be differentially methylated in a variety of comparisons between different MDS subtypes. Specifically, CpG islands, transcription start sites and post-transcriptional start sites within ZNF124, ZNF497 and PCDHG family are differentially methylated with fold change above 3,5. Overall, these findings highlight important aspects of the epigenomic component of MDS syndromes pathogenesis and the pharmacoepigenomic basis to the hypomethylating agents drug treatment response, while this generic high throughput whole epigenome sequencing approach could be readily implemented to other genetic diseases with a strong epigenetic component.
MeSH term(s)	Humans ; DNA Methylation/genetics ; Epigenomics ; Epigenesis, Genetic ; Myelodysplastic Syndromes/drug therapy ; Myelodysplastic Syndromes/genetics ; Myelodysplastic Syndromes/pathology ; Disease Progression ; CpG Islands/genetics ; DNA-Binding Proteins/genetics
Chemical Substances	ZNF124 protein, human (148733-47-5) ; DNA-Binding Proteins
Language	English
Publishing date	2023-04-25
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2147618-4
ISSN	1479-7364 ; 1479-7364
ISSN (online)	1479-7364
ISSN	1479-7364
DOI	10.1186/s40246-023-00483-7
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Article ; Online: Real-world complication burden and disease management paradigms in transfusion-related β-thalassaemia in Greece: Results from ULYSSES, an epidemiological, multicentre, retrospective cross-sectional study.

Kattamis, Antonis / Voskaridou, Ersi / Delicou, Sophia / Klironomos, Evangelos / Lafiatis, Ioannis / Petropoulou, Foteini / Diamantidis, Michael D / Lafioniatis, Stylianos / Evliati, Loukia / Kapsali, Eleni / Karvounis-Marolachakis, Kiki / Timotheatou, Despoina / Deligianni, Chrysoula / Viktoratos, Panagiotis / Kourakli, Alexandra

EJHaem

2023 Volume 4, Issue 3, Page(s) 569–581

Abstract: Patients with transfusion-dependent beta (β)-thalassaemia experience a broad range of complications. ULYSSES, an epidemiological, multicentre, retrospective cross-sectional study, aimed to assess the prevalence and severity of treatment and disease ... ...

Abstract	Patients with transfusion-dependent beta (β)-thalassaemia experience a broad range of complications. ULYSSES, an epidemiological, multicentre, retrospective cross-sectional study, aimed to assess the prevalence and severity of treatment and disease complications, capture disease management and identify predictors of complications in patients with transfusion-dependent β-thalassaemia, treated in routine settings in Greece. Eligible patients were adults diagnosed with β-thalassaemia ≥12 months before enrolment and having received ≥6 red blood cell (RBC) units (excluding elective surgery) with no transfusion-free period ≥35 days in the 24 weeks before enrolment. Primary data were collected at a single visit and through chart review. Between Oct 21, 2019, and Jun 15, 2020, 201 eligible patients [median (interquartile range, IQR) age 45.7 (40.2-50.5) years; 75.6% > 40 years old; 64.2% female] were enrolled, a mean (standard deviation) of 42.9 (7.8) years after diagnosis. Median (IQR) age at diagnosis and RBC transfusion initiation were 0.8 (0.4-2.8) and 1.3 (1.0-5.0) years, respectively. From diagnosis to enrolment, patients had developed a median of six (range: 1-55) complications; 19.6% were grade ≥3. The most represented complications were endocrine/metabolic/nutrition disorders (91.5%), surgical/medical procedures (67.7%) and blood/lymphatic system disorders (64.7%). Real-world data generated by ULYSSES underscore the substantial complication burden of transfusion-dependent β-thalassaemia patients, routinely managed in Greece.
Language	English
Publishing date	2023-05-23
Publishing country	United States
Document type	Journal Article
ISSN	2688-6146
ISSN (online)	2688-6146
DOI	10.1002/jha2.695
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Article ; Online: Predisposing factors for advanced liver fibrosis in patients with sickle cell disease.

Manganas, Konstantinos / Delicou, Sophia / Xydaki, Aikaterini / Kourakli, Alexandra / Evliati, Loukia / Vlachaki, Efthymia / Klironomos, Evangelos / Diamantidis, Michail / Lafiatis, Ioannis / Kattamis, Antonios / Koskinas, John

British journal of haematology

2023 Volume 202, Issue 6, Page(s) 1192–1198

Abstract: Sickle cell disease (SCD) is one of the most common monogenic disorders worldwide and liver complications are common in this group of patients. Our study aims to highlight the prevalence of chronic liver complications and the main predisposing factors ... ...

Abstract	Sickle cell disease (SCD) is one of the most common monogenic disorders worldwide and liver complications are common in this group of patients. Our study aims to highlight the prevalence of chronic liver complications and the main predisposing factors for advanced liver fibrosis in SCD patients. For this purpose, 219 patients from eight Thalassemia and Sickle Cell Units across Greece enrolled in our study and history of liver related disease complications was recorded, as well as a full laboratory and imaging analysis concerning their liver function. 13.6% of the patients had advanced liver fibrosis. The presence of liver fibrosis was significantly correlated with advanced age, male gender, cholelithiasis and higher LDH, γ-GT, INR, direct and indirect bilirubin levels. These patients had exhibited significantly more episodes of liver crises and acute intrahepatic cholestasis. No correlation was observed with right heart failure or previous viral hepatitis. Patients with advanced liver fibrosis were receiving a more intensive transfusion therapy for a longer period of time and had higher Liver Iron Concentration levels. Our study shows that liver complications and cirrhosis is a significant cause of morbidity in patients with SCD and it is primarily associated with intravascular hemolysis and vaso-occlusive phenomena and secondarily with iron overload.
MeSH term(s)	Humans ; Male ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/therapy ; Liver Cirrhosis/etiology ; Blood Transfusion/methods ; Liver Diseases/complications ; Liver
Language	English
Publishing date	2023-07-12
Publishing country	England
Document type	Journal Article
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1111/bjh.18970
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Article: Ruptured infectious ICA pseudoaneurysm into the sphenoid sinus after maxillofacial infection, successfully treated by selective embolization.

Panagiotopoulos, Vasileios / Theofanopoulos, Andreas / Kourakli, Alexandra / Symeonidis, Anargyros / Krisela, Valera / Mastronikolis, Nicholas S / Zampakis, Petros

Surgical neurology international

2021 Volume 12, Page(s) 191

Abstract: Background: Intracranial infectious aneurysms are cerebral aneurysms caused by pathogen-induced inflammation undermining the arterial wall. We present a rare case of inflammatory pseudoaneurysm of cavernous internal carotid artery (ICA).: Case ... ...

Abstract	Background: Intracranial infectious aneurysms are cerebral aneurysms caused by pathogen-induced inflammation undermining the arterial wall. We present a rare case of inflammatory pseudoaneurysm of cavernous internal carotid artery (ICA). Case description: A 51-year-old female with a recent diagnosis of acute lymphoblastic leukemia developed maxillofacial infection with Conclusion: Conclusively, ruptured internal carotid infectious aneurysms are rare but potentially fatal causes of epistaxis when extended into the sphenoid sinus. Selective coiling is feasible and can provide definitive treatment of these lesions.
Language	English
Publishing date	2021-04-26
Publishing country	United States
Document type	Case Reports
ISSN	2229-5097
ISSN	2229-5097
DOI	10.25259/SNI_52_2021
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Article: Case Report: Kidney Transplantation in a Patient With Acquired Agammaglobulinemia and SLE. Issues and Challenges.

Pavlakou, Paraskevi / Papasotiriou, Marios / Ntrinias, Theodoros / Kourakli, Alexandra / Bratsiakou, Adamantia / Goumenos, Dimitrios S / Papachristou, Evangelos

Frontiers in medicine

2021 Volume 8, Page(s) 665475

Abstract: Lupus nephritis in the context of Systemic Lupus Erythematosus (SLE) is characterized by an unpredicted course with remissions and flare-ups. Among others, it remains a significant cause of end-stage kidney disease (ESKD) in relatively young patients. ... ...

Abstract	Lupus nephritis in the context of Systemic Lupus Erythematosus (SLE) is characterized by an unpredicted course with remissions and flare-ups. Among others, it remains a significant cause of end-stage kidney disease (ESKD) in relatively young patients. Therapeutic regimens with newer immunosuppressive agents have been introduced in order to control SLE clinical manifestations more efficiently and limit organ damage induced by immune complex formation and sustained inflammation. Treatment is usually long-term, and the cumulative impact of immunosuppression is expressed through the increased frequency of infections and neoplasms. However, if the observed immunity dysregulation is secondary and pharmaceutically induced or there is a pre-existing, primary immunodeficiency that shares common pathogenetic pathways with SLE's autoimmunity is not always clear. Herein, we present the case of a 39-year-old woman, that reached ESKD due to lupus nephritis. After an upper respiratory cytomegalovirus (CMV) infection and concomitant CMV reactivations the investigation revealed significant immunodeficiency. Not long after the initiation of intravenous immunoglobulin (IVIG) administration, patient received a cadaveric kidney transplant. IVIG was continued along with standard immunosuppression so that both recurrent infections and allograft rejection are avoided. Patient is closely monitored, and her post-transplant course is remarkably satisfying so far. ESKD patients with immunodeficiency syndromes should not be excluded by definition from kidney transplantation.
Language	English
Publishing date	2021-03-12
Publishing country	Switzerland
Document type	Case Reports
ZDB-ID	2775999-4
ISSN	2296-858X
ISSN	2296-858X
DOI	10.3389/fmed.2021.665475
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Article ; Online: Rare lobular capillary hemangioma associated with azacitidine in high-risk myelodysplastic syndrome patient.

Grafanaki, Katerina / Kourakli, Alexandra / Skeparnias, Ilias / Spiliopoulos, Theofanis / Koumoundourou, Dimitra / Bravou, Vasiliki / Alexopoulos, Angelos / Symeonidis, Argiris / Georgiou, Sophia

Dermatologic therapy

2021 Volume 34, Issue 2, Page(s) e14884

MeSH term(s)	Antimetabolites, Antineoplastic/therapeutic use ; Azacitidine/therapeutic use ; Granuloma, Pyogenic ; Humans ; Myelodysplastic Syndromes/diagnosis ; Myelodysplastic Syndromes/drug therapy
Chemical Substances	Antimetabolites, Antineoplastic ; Azacitidine (M801H13NRU)
Language	English
Publishing date	2021-02-25
Publishing country	United States
Document type	Letter
ZDB-ID	1354801-3
ISSN	1529-8019 ; 1396-0296
ISSN (online)	1529-8019
ISSN	1396-0296
DOI	10.1111/dth.14884
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