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BACKGROUND: Although plateletpheresis donation is commonly accepted as a safe procedure, its influence on platelet function, coagulation system and fibrinolysis is not completely elucidated. OBJECTIVES: In this study, we tried to assess the effects of plateletpheresis on donor's hemostasis system by measuring platelet activation, development of platelet-leukocyte aggregates, and coagulation activation. STUDY DESIGN: Prospective observational study. METHODS: We used flow cytometry to determine the levels of platelet-monocyte complexes (PMC) and platelet-neutrophil complexes (PNC). sP-selectin and prothrombin fragment (PF) 1â+â2 values were determined by ELISA. RESULTS: The PMC levels increased significantly seven days after apheresis in comparison with just after apheresis and 24âh after apheresis (pâ<â0.05). The PNC levels increased significantly seven days after apheresis compared to immediately after apheresis (pâ<â0.05). sP-selectin values decreased significantly immediately after apheresis (pâ<â0.05). While sP-selectin values increased seven days after apheresis in comparison with immediately after apheresis and 24âh after apheresis, but there were not statistically significant differences for sP-selectin levels (pâ>â0.05). PF1â+â2 levels decreased significantly immediately after apheresis compared to pre-apheresis (pâ<â0.05) and increased 24âh after apheresis and seven days after apheresis, but these differences were not statistically significant. CONCLUSION: We concluded that plateletpheresis affects platelet activation but does not cause any change in coagulation activation.
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Although Candida species are the most common cause of fungemia, non-Candida rare yeasts (NCY) have been increasingly reported worldwide. Although the importance of these yeast infections is recognized, current epidemiological information about these pathogens is limited, and they have variable antifungal susceptibility profiles. In this study, we aimed to evaluate the clinical characteristics for fungemia caused by NCY by comparing with candidemia. The episodes of NCY fungemia between January 2011 and August 2023 were retrospectively evaluated in terms of clinical characteristics, predisposing factor, and outcome. In addition, a candidemia group, including patients in the same period was conducted for comparison. Antifungal susceptibility tests were performed according to the reference method. A total of 85 patients with fungemia episodes were included: 25 with NCY fungemia and 60 with candidemia. Fluconazole had high minimal inhibitory concentration (MIC) values against almost all NCY isolates. The MIC values for voriconazole, posaconazole, and amphotericin B were ≤ 2 µg/ml, and for caspofungin and anidulafungin were ≥ 1 µg/ml against most of isolates. Hematological malignancies, immunosuppressive therapy, neutropenia and prolonged neutropenia, polymicrobial bacteremia/fungemia, preexposure to antifungal drugs, and breakthrough fungemia were associated with NCY fungemia, whereas intensive care unit admission, diabetes mellitus, urinary catheters, and total parenteral nutrition were associated with candidemia. In conclusion, the majority of fungemia due to NCY species was the problem, particularly in hematology units and patients with hematological malignancy. Preexposure to antifungal drugs likely causes a change in the epidemiology of fungemia in favor of non-albicans Candida and/or NCY.
Among all fungemia episodes, hematological malignancies, immunosuppressive therapy, neutropenia, and preexposure to antifungals were risk factors for non-Candida yeast fungemia; diabetes mellitus, urinary catheters, and total parenteral nutrition were risks for candidemia.
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Antifúngicos , Candida , Candidemia , Fungemia , Testes de Sensibilidade Microbiana , Centros de Atenção Terciária , Humanos , Estudos Retrospectivos , Centros de Atenção Terciária/estatística & dados numéricos , Antifúngicos/farmacologia , Antifúngicos/uso terapêutico , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Candida/efeitos dos fármacos , Candida/isolamento & purificação , Candida/classificação , Fungemia/microbiologia , Fungemia/epidemiologia , Fungemia/tratamento farmacológico , Adulto , Candidemia/microbiologia , Candidemia/epidemiologia , Candidemia/tratamento farmacológico , Leveduras/isolamento & purificação , Leveduras/efeitos dos fármacos , Leveduras/classificação , Idoso de 80 Anos ou mais , Fluconazol/farmacologia , Fluconazol/uso terapêutico , Adulto JovemRESUMO
Background: Although human leukocyte antigen (HLA) data for the Turkish population has been reported, there are no statistics on the HLA-DPB1 locus, which has recently received significant attention, particularly in hematopoietic stem cell transplantation. In addition, there is no study that has reported the 2-6 loci HLA haplotype distribution, 8-digit HLA allele frequency, and genotype frequency in the Turkish population. Aims: To evaluate the low and high resolution (2-4-8 digits) HLA-A, -B, -C, -DRB1, -DQB1, -DPB1 allele data using the data of 6100 healthy individuals from the Central Anatolian region of Turkey. Study Design: Retrospective cross-sectional study. Methods: All tests were performed using molecular HLA techniques: low-resolution DNA-based sequence-specific oligonucleotides, low/high-resolution DNA-based sequence-specific primer, and high-resolution next generation sequencing. A total of 6100 healthy donors with a minimum of 3 loci (HLA-A, -B, -DRB1) were analyzed for their HLA-A, -B, -C, -DRB1, -DQB1, and -DPB1 data. Pypop and HLA-net GENE[RATE] were used to analyze the data. Results: Among the HLA class I alleles, the following were the most frequently observed alleles: for HLA-A, A*02, A*24, A*03, and A*01; for HLA-B, B*35, B*51, and B*44; and for HLA-C, C*07, C*04, and C*12. Among the HLA class II alleles, the following alelles were the most frequently observed: for HLA-DRB1, DRB1*11, DRB1*04, and DRB1*13; for HLA-DQB1, DQB1*03, DQB1*05, and DQB1*06; and for HLA- DPB1, DPB1*04, DPB1*02, and DPB1*03. The most common alleles among HLA-DPB1 in the 4-digit evaluation were DPB1*04:01, DPB1*02:01, and DPB1*04:02. Among the HLA classes I and II, the following were the most frequently observed 8-digit alleles in HLA-A, -B, -C, -DRB1, -DQB1 and -DPB1 were A*02:01:01:01, B*49:01:01:01, C*04:01:01:06, DRB1*07:01:01:01, DQB1*03:01:01:02, and DPB1*02:01:02:05, respectively. The most common 6 loci haplotype was A*02~B*35~C*04~DRB1*11~DQB1*03~DPB1*04 (2.71%). Conclusion: In this study, low and high resolution HLA-DPB1 allele frequency, 6 locus haplotype frequency and genotype frequency were reported for the first time in Turkish population. These new data can be used to map HLA in our country and may provide ideas for potential future studies.
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Antígenos HLA-A , Antígenos de Histocompatibilidade Classe I , Humanos , Haplótipos , Turquia , Alelos , Frequência do Gene/genética , Estudos Retrospectivos , Estudos Transversais , Genótipo , Antígenos de Histocompatibilidade Classe I/genética , Cadeias HLA-DRB1/genética , Antígenos HLA-A/genética , DNARESUMO
OBJECTIVES: Acute hemolytic transfusion reaction (AHTR) due to ABO-incompatible erythrocyte concentrate (EC) is one of the most catastrophic complications of transfusion. Since the hemolysis is intravascular; hemoglobinemia and hemoglobinuria result in disseminated intravascular coagulation (DIC), acute renal failure, shock, and sometimes death. BACKGROUND: Treatment of AHTR is mostly supportive measures. Today there are no clear suggestions about plasma exchange (PE) in these patients. METHODS/MATERIALS: Here we report our experience with six patients diagnosed with AHTR due to ABO-incompatible EC transfusion. RESULTS: We performed PE in 5 of these patients. Although all of our patients were geriatric and most of them had significant comorbidities four out of five patients recovered without an incident. CONCLUSION: Although PE is considered a last-chance treatment when other measures fail in the literature, our experience above indicates that it must be evaluated in every patient with AHTR early in the course. If the patient has cardiac and renal comorbidities, large volume EC is transfused, DAT is negative, plasma color is red and there is macroscopic hemoglobinuria, we suggest performing PE.
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Hemólise , Reação Transfusional , Humanos , Idoso , Troca Plasmática , Hemoglobinúria , Transfusão de Plaquetas , Incompatibilidade de Grupos Sanguíneos , Eritrócitos , Sistema ABO de Grupos SanguíneosRESUMO
Subacute thyroiditis (SAT) is a thyroid disease associated with viral infections. Its relationship with major histocompatibility complex (MHC) antigens was shown before. SAT cases triggered by different types of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines have been reported. In this study, human leukocyte antigen (HLA) genotypes of 27 SAT patients (13 vaccine-associated (V-SAT) and 14 non-SARS-CoV-2-infection non-vaccine-associated (non-V-SAT)) were compared with those of 362 healthy donors. HLA analyses were performed with low-resolution DNA-based sequence-specific oligonucleotide or sequence-specific primer methods. Statistical analyses were performed using IBM SPSS Statistics 25 and Stata/MP 14.1 with the hapipf function. Allele and haplotype frequencies were estimated by PyPop and gene[RATE] tool programs. The allele frequencies of HLA-A*11, HLA-B*35, and HLA-C*04 were higher in the patient groups. Both the allele frequency of HLA-A*11 and the haplotype frequency of A*11-B*35-C*04 were higher in the V-SAT group. The A*11-B*35-C*04 haplotype, including all three loci of MHC class I genes, is shown to be associated with the disease for the first time, especially in the V-SAT group. This finding will contribute to a better understanding of the etiopathogenesis of vaccine-associated SAT and the role of HLA genotypes in the functioning mechanisms of the SARS-CoV-2 vaccines.
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Gaucher disease (GD) is a rare hereditary lysosomal storage disease that arises due to deficiency of glucocerebrosidase. Early diagnosis is very important for starting proper treatment and preventing complications. Splenomegaly, anemia, and thrombocytopenia are the most common findings in GD and so most patients are initially referred to hematologists. The Turkish Society of Hematology established its Rare Hematological Diseases Subcommittee in 2015. One of the main topics of this subcommittee was to increase and improve awareness and education of rare diseases among hematologists in Turkey. This review presents GD with an overview of its clinical features, pathophysiology, and treatment options for hematologists.
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Anemia , Doença de Gaucher , Esplenomegalia , Trombocitopenia , Anemia/etiologia , Doença de Gaucher/complicações , Doença de Gaucher/diagnóstico , Doença de Gaucher/terapia , Glucosilceramidase/deficiência , Humanos , Esplenomegalia/etiologia , Trombocitopenia/etiologia , TurquiaRESUMO
Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.
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Hiperplasia do Linfonodo Gigante , Adulto , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/terapia , Criança , Feminino , Humanos , Linfonodos/patologia , Masculino , Estudos Retrospectivos , Rituximab/uso terapêutico , Turquia/epidemiologiaRESUMO
Objective: Patients with solid malignancies are more vulnerable to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection than the healthy population. The outcome of SARS-CoV-2 infection in highly immunosuppressed populations, such as in patients with hematological malignancies, is a point of interest. We aimed to analyze the symptoms, complications, intensive care unit admissions, and mortality rates of patients with hematological malignancies infected with SARS-CoV-2 in Turkey. Materials and Methods: In this multicenter study, we included 340 adult and pediatric patients diagnosed with SARS-CoV-2 from March to November 2020. Diagnosis and status of primary disease, treatment schedules for hematological malignancies, time from last treatment, life expectancy related to the hematological disease, and comorbidities were recorded, together with data regarding symptoms, treatment, and outcome of SARS-CoV-2 infection. Results: Forty four patients were asymptomatic at diagnosis of SARS-CoV- 2 infection. Among symptomatic patients, fever, cough, and dyspnea were observed in 62.6%, 48.8%, and 41.8%, respectively. Sixty-nine (20%) patients had mild SARS-CoV-2 disease, whereas moderate, severe, and critical disease was reported in 101 (29%), 71 (20%), and 55 (16%) patients, respectively. Of the entire cohort, 251 (73.8%) patients were hospitalized for SARS-CoV-2. Mortality related to SARS-CoV-2 infection was 26.5% in the entire cohort; this comprised 4.4% of those patients with mild disease, 12.4% of those with moderate disease, and 83% of those with severe or critical disease. Active hematological disease, lower life expectancy related to primary hematological disease, neutropenia at diagnosis of SARS-CoV-2, ICU admission, and first-line therapy used for coronavirus disease-2019 treatment were found to be related to higher mortality rates. Treatments with hydroxychloroquine alone or in combination with azithromycin were associated with a higher rate of mortality in comparison to favipiravir use. Conclusion: Patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.
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COVID-19 , Neoplasias Hematológicas , Adulto , Amidas/administração & dosagem , Azitromicina/administração & dosagem , COVID-19/complicações , COVID-19/mortalidade , Criança , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/mortalidade , Neoplasias Hematológicas/terapia , Humanos , Hidroxicloroquina/administração & dosagem , Hidroxicloroquina/efeitos adversos , Pirazinas/administração & dosagem , SARS-CoV-2 , Turquia/epidemiologiaRESUMO
Deletion 13q [del(13q)] is a favorable prognostic marker if it is detected as a sole abnormality in chronic lymphocytic leukemia (CLL). However the clinical courses of cases with isolated del(13q) are quite heterogeneous. In our study, we investigated copy number variations (CNVs), loss of heterozygosity (LOH), and the size of del(13q) in 30 CLL patients with isolated del(13q). We used CGH+SNP microarrays in order to understand the cause of this clinical heterogeneity. We detected del(13q) in 28/30 CLL cases. The size of the deletion varied from 0.34 to 28.81 Mb, and there was no clinical effect of the deletion size. We found new prognostic markers, especially the gain of 16p13.3. These markers have statistically significant associations with short time to first treatment and advanced disease stage. Detecting both CNVs and LOH at the same time is an advantageous feature of aCGH+SNP. However, it is very challenging for the array analysis to detect mosaic anomalies. Therefore, it is very important to confirm the results by FISH. In our study, we detected approximately 9% mosaic del(13q) by microarray. In addition, the gain of 16p13.3 may affect the disease prognosis in CLL. However, additional studies with more patients are needed to confirm these results.
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Deleção Cromossômica , Cromossomos Humanos Par 13/genética , Cromossomos Humanos Par 16/genética , Leucemia Linfocítica Crônica de Células B/genética , Idoso , Variações do Número de Cópias de DNA/genética , Feminino , Humanos , Perda de Heterozigosidade/genética , Masculino , PrognósticoRESUMO
Castleman disease is a rare lymphoproliferative disease also known as angiofollicular lymph node hyperplasia. It is classified as hyaline vascular and plasmacytic variants histologically but characteristics of both types can coexist. Most unicentric cases of the disease are hyaline vascular while most multicentric cases are of the plasmacytic type. Although the pathogenesis is not completely understood, the role of interleukin (IL)-6 in unicentric disease and the roles of IL-6 and human herpes virus-8 in multicentric disease are well defined. Unicentric disease is typically localized and symptoms are minimal and treated locally. Multicentric disease is systemic and clinically characterized by generalized lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. Systemic therapies are primarily given. Several malignant diseases including lymphomas, POEMS syndrome, follicular dendritic cell sarcomas, paraneoplastic pemphigus, Kaposi sarcoma, and amyloidosis can be associated with Castleman disease. In this paper, recent information about Castleman disease, which is a rare disease, is summarized.
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Hiperplasia do Linfonodo Gigante , Doenças Raras , Hiperplasia do Linfonodo Gigante/patologia , Hiperplasia do Linfonodo Gigante/terapia , HumanosRESUMO
INTRODUCTION: Venetoclax is a selective B-cell lymphoma 2 (BCL2) inhibitor, which is approved to treat elderly patients with newly diagnosed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS) in combination with either low-dose cytarabine (ARA-C) or hypomethylating agents. We aimed to collect and share data among the efficacy and safety of venetoclax both as a monotherapy or in combination with other drugs used to treat high-risk MDS or AML. MATERIALS AND METHODS: A total of 60 patients with a median age of 67 (30-83) years from 14 different centers were included in the final analysis. Thirty (50%) of the patients were women; 6 (10%) of the 60 patients were diagnosed with high-risk MDS and the remaining were diagnosed with AML. RESULTS: The best objective response rate (complete remission [CR], complete remission with incomplete hematological recovery (CRi), morphological leukemia-free state [MLFS], partial response [PR]) was 35% in the entire cohort. Best responses achieved during venetoclax per patient number were as follows: 7 CR, 1 CRi, 8 MLFS, 5 PR, and stable disease. Median overall survival achieved with venetoclax was 5 months in patients who relapsed and not achieved in patients who were initially treated with venetoclax. Nearly all patients (86.7%) had experienced a grade 2 or more hematologic toxicity. Some 36.7% of these patients had received granulocyte colony stimulating factor (GCSF) support. Infection, mainly pneumonia (26.7%), was the leading nonhematologic toxicity, and fatigue, diarrhea, and skin reactions were the others reported. CONCLUSION: Our real-life data support the use of venetoclax in patients with both newly diagnosed and relapsed high-risk MDS and AML.
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Antineoplásicos/uso terapêutico , Compostos Bicíclicos Heterocíclicos com Pontes/uso terapêutico , Leucemia Mieloide Aguda/tratamento farmacológico , Síndromes Mielodisplásicas/tratamento farmacológico , Sulfonamidas/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Humanos , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/mortalidade , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/mortalidade , Indução de Remissão , Análise de Sobrevida , Resultado do Tratamento , TurquiaRESUMO
Background/aim: Hyperparathyroidism is an endocrine disorder characterized by hypercalcemia. Because of calcium's effects on parathyroid glands, bone, intestines, and kidneys, it has an important place in homeostasis. The results of studies regarding hyperparathyroidism hemostasis are conflicting. Thromboelastography helps to evaluate all steps of hemostatic system. Our aim in this study was to investigate the possible role of hemostatic mechanisms in the development of thrombosis in hyperparathyroid patients with the modified rotation thromboelastogram (ROTEM). Materials and methods: Twenty-two patients with primary hyperparathyroidism (PHPT) and 20 healthy controls were involved. This study was conducted in Eskisehir Osmangazi University Faculty of Medicine, Endocrinology and Hematology clinics for 2 years. The complete blood count, fibrinogen, D-dimer levels, prothrombin time, activated prothrombin time, and ROTEM parameters [clot formation time (CFT), clotting time (CT), and maximum clot formation (MCF)] were determined by two activated tests, INTEM and EXTEM analyses. A thromboelastographic evaluation was performed in the preoperative and postoperative (3 months after surgery) periods. Results: In INTEM assay, the CT (p = 0.012) and CFT (p = 0.07) values were increased in preoperative PHPT patients compared with the control group. Although there was a decrease in the postoperative CT and CFT values, no statistical difference was found. Conclusion: The prolongation of the CT and CFT values were consistent with a hypocoagulable state in patients with PHPT. Hyperparathyroidism causes a hypocoagulable state that can be successfully assessed by ROTEM. Hemostatic changes, do not seem to have an effect on increased cardiovascular mortality.
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Coagulação Sanguínea , Hemostáticos , Hiperparatireoidismo/complicações , Tromboelastografia/métodos , Testes de Coagulação Sanguínea , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , RotaçãoRESUMO
Glanzmann thrombasthenia is an inherited disease causing bleeding episodes due to platelet dysfunction. The standard treatment for moderate-severe bleeding is platelet transfusion. Recombinant factor VIIa (rFVIIa) is successfully used in bleeding episodes and invasive procedures. Here, we present a patient with Glanzmann thrombasthenia, whose bleeding episodes could only be controlled by rFVIIa. The patient is a 28 years old male, who has had frequent bleeding episodes unresponsive to local hemostatic agents and tranexamic acid and had an anaphylactoid reaction to platelet transfusion. We started the patient on a low-dose (20âµg/kg) rFVIIa once a week. The patient has no spontaneous bleeding since then. This is the first case report of a Glanzmann thrombasthenia patient on routine prophylaxis with low-dose rFVIIa.
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Fator VIIa/uso terapêutico , Hemorragia/etiologia , Hemorragia/prevenção & controle , Trombastenia/complicações , Adulto , Relação Dose-Resposta a Droga , Fator VIIa/administração & dosagem , Humanos , Masculino , Proteínas Recombinantes/administração & dosagem , Proteínas Recombinantes/uso terapêuticoRESUMO
Chronic myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem/progenitor cell characterized by thrombohemorrhagic complications and a tendency to transform into acute leukemia. The pathogenesis of thrombosis in MPN is complex and results from a multifaceted interplay of clinical and disease-related factors. Rotational thromboelastometry (ROTEM) provides the complete and rapid information about all stages of the coagulation process. Here, we assess ROTEM parameters as a screening of coagulation profile in patients with MPNs. In particular, higher mean maximum clot firmness values were found in Essential thrombocythemia and Polycythemia vera patients when compared to healthy controls. Rotational thromboelastometry may be able to detect MPN patients who are susceptible to thrombotic and/or hemorrhagic complications. The predictive value of ROTEM for thrombosis remains to be established to classify subsets of patients at prominent risk who may benefit from prophylaxis with antithrombotic drugs.
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Transtornos Mieloproliferativos/terapia , Tromboelastografia/métodos , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos Mieloproliferativos/patologiaRESUMO
The pathogenesis of chronic spontaneous urticaria (CSU) is incompletely understood. There is a growing interest in the role of the coagulation cascade in chronic urticaria. Rotational thromboelastometry (ROTEM) assay enables the global assessment of coagulation status. In the present study, we aimed to test the coagulation profile in children with CSU using ROTEM and correlate these parameters with those of a healthy group. A total of 24 children with active CSU (11 girls and 13 boys) 8 to 17 years of age and age-matched and sex-matched 30 healthy control participants were enrolled in the study. ROTEM assays (intrinsic thromboelastometry and extrinsic thromboelastometry) were used to measure and analyze coagulation time, clot formation time, and maximum clot firmness. The CSU patients and controls did not differ in age, sex, erythrocyte, neutrophil, and platelet counts. Also, ROTEM parameters did not show any difference between the 2 groups. ROTEM is increasingly being used as a tool for monitoring coagulation status. In this study, ROTEM parameters did not show any difference between CSU patients and the healthy group. Further studies are needed to confirm our findings on a larger number of CSU patients.
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Coagulação Sanguínea , Urticária Crônica/fisiopatologia , Tromboelastografia/métodos , Adolescente , Estudos de Casos e Controles , Feminino , Fibrinogênio/metabolismo , Seguimentos , Humanos , Masculino , Contagem de Plaquetas , PrognósticoRESUMO
Hereditary spherocytosis (HS) is a familial hemolytic disorder associated with a variety of mutations that lead to defects in red blood cell (RBC) membrane proteins. There is increasing evidence that hypercoagulability occurs in chronic hemolytic anemia. In this study, changes in the coagulation profile in children with HS were investigated using rotational thromboelastometry. A total of 21 children with HS and 28 healthy children were enrolled in the study between October 2010 and October 2018. Complete blood count, prothrombin time, activated partial thromboplastin time, and fibrinogen level were ascertained, while rotational thromboelastometry assays were used to measure and analyze coagulation time, clot formation time, and maximum clot firmness. There was no difference between the 2 groups in terms of age and sex. The values of hemoglobin and RBC in the patient group were statistically significantly lower than those in the control group (P<0.01, <0.0001, respectively), and the values of platelet count, mean corpuscular hemoglobin concentration, and RBC distribution width were statistically significantly higher than those in the control group (P<0.05, 0.001, <0.0001, respectively). There was no statistically significant difference between the 2 groups in terms of prothrombin time, activated partial thromboplastin time, fibrinogen levels, coagulation time, clot formation time, and maximum clot firmness values. In contrast to other chronic hemolytic anemias, no predisposition to hypercoagulability has been shown in the coagulation profile of children with HS without splenectomy.
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Esferocitose Hereditária/sangue , Adolescente , Contagem de Células Sanguíneas , Criança , Feminino , Fibrinogênio/metabolismo , Humanos , Masculino , Tempo de Tromboplastina Parcial , Tempo de Protrombina , TromboelastografiaRESUMO
OBJECTIVES: Circulating endothelial cells (CEC) are identified in conditions with vascular damage such as systemic vasculitis. Our aim was to investigate if EPC, CEC, and/or its subgroups activated CEC (aCEC) or resting CEC (rCEC) related with vascular involvement in Behçet's disease (BD). METHODS: In total 60 patients were included in this study, divided into 4 groups: 1) Behçet patients with a history of vascular involvement: vascular BD; 2) Behçet patients with mucocutaneus involvement: mucocutaneus BD; 3) patients with history of thrombosis due to other causes: thrombosis; 4) 20 healthy controls were also included: control group. Percentages of CEC, aCEC, rCEC and EPCs in peripheral blood mononuclear cells were measured by flow cytometry. RESULTS: CEC (3.75 (1.80-7.20), 1.80 (0.70-3.53), 3.50 (1.83-7.23), 2.45 (1.28- 4.60)) and aCEC (2.40 (1.28-4.28), 1.10 (0.77-2.20), 3.15 (1.48-7.20), 3.20 (1.15-9.80) levels were did not show a statistically significant difference between groups (p:0.077 and p:0.054, respectively). EPC and rCEC levels were higher in vascular BD and thrombosis groups than mucocutaneus BD and control groups (EPC:10.5 (7.20-18.3), 11.6 (7.30-20.9) vs. 7.15 (5.55-8.25), 10.2 (5.93-18.6), rCEC: 5.35 (3.13-7.90), 6.45 (4.60-10.8) vs. 4.95 (3.05-7.55), 3.40 (1.88-4.30), p:0.042 and p:0.007, respectively). CONCLUSIONS: CEC, EPC, aCEC and rCEC may have role in the assessment of vascular involvement in BD. Longitudinal studies would be needed to identify the utility of these cells for the follow up and risk stratification of BD patients with vascular involvement for recurrences or identify BD patients at risk of vascular involvement.
Assuntos
Síndrome de Behçet , Células Endoteliais , Síndrome de Behçet/sangue , Contagem de Células Sanguíneas , Estudos de Casos e Controles , Contagem de Células , Células Endoteliais/metabolismo , Feminino , Humanos , Leucócitos Mononucleares , MasculinoRESUMO
OBJECTIVE: Iron deficiency anemia (IDA) has been demonstrated to be a risk factor for thromboembolic events, although the pathogenesis of the development of thromboembolism in IDA is as yet unclear. The likelihood of children with IDA contracting hypercoagulability was evaluated in this cross-sectional study using rotational thromboelastometry (ROTEM). MATERIAL AND METHOD: A total of 57 children with IDA (median age 11 years; 37 female, 20 male) and 48 healthy children (median age 9.9 years; 23 female, 25 male) were enrolled in the study. Whole blood count, serum iron, transferrin saturation, ferritin level, prothrombin time (PT), activated partial thromboplastin time (aPTT), fibrinogen levels were ascertained, while ROTEM assays [intrinsic TEM (INTEM) and extrinsic TEM (EXTEM)] were used to measure and analyze coagulation time (CT), clot formation time (CFT), maximum clot firmness (MCF) and rate of maximum lysis (ML60%). This study conforms to ethical standards, has been approved by the appropriate Institutional Review Board. RESULTS: Hemoglobin, serum iron, transferrin saturation and ferritin levels were lower in the IDA group than in the control group (p < 0.001, for all), while the EXTEM and INTEM CT in the two groups were similar (p > 0.05). The EXTEM and INTEM MCF in the IDA group was higher than in the control group, while the INTEM CFT and rate of ML60% were lower than in the control group (p < 0.001, p < 0.001, p < 0.05, p < 0.001, respectively). CONCLUSION: The ROTEM results suggest that although the platelet count and coagulation tests were within normal ranges in IDA, the tendency to coagulate may have been increased.
Assuntos
Anemia Ferropriva/sangue , Trombofilia/sangue , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Tromboelastografia/métodosRESUMO
Autologous hematopoietic cell transplantation (AHCT) is an established treatment option for adult patients presenting with multiple myeloma (MM), Hodgkin lymphoma (HL) and various subtypes of non-Hodgkin lymphoma (NHL) in upfront and/or relapsed/refractory disease settings. Although there are recently published consensus guidelines addressing critical issues regarding autologous hematopoietic progenitor cell mobilization (HPCM), mobilization strategies of transplant centers show high variability in terms of routine practice. In order to understand the current institutional policies regarding HPCM in Turkey and to obtain the required basic data for preparation of a national positional statement on this issue, Turkish Hematology Research and Education Group (ThREG) conducted a web-based HPCM survey. The survey was designed to include multiple-choice questions regarding institutional practice of HPCM in adults presenting MM, HL, and NHL. The representatives of 27 adult HCT centers participated to the study. Here we report the results of this survey shedding light on the real-world experience in Turkey in terms of autologous HPCM mobilization strategies in patients presenting with MM and lymphoma.