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1.
Eur J Clin Microbiol Infect Dis ; 43(1): 33-43, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37910269

RESUMEN

BACKGROUND: Diagnostic accuracy of galactomannan measurements is highly variable depending on the study population, diagnostic procedures, and treatment procedures. We aimed to evaluate the effect of posaconazole prophylaxis and empiric antifungal treatment upon diagnostic accuracy of GM measurements in bronchoalveolar lavage (BAL), bronchial lavage (BL), and serum in hematological malignancy population. METHODS: Patients hospitalized in a single tertiary care center with hematologic malignancies undergoing fiberoptic bronchoscopy (FOB) with a preliminary diagnosis of IPA were retrospectively included. RESULTS: In all the study population (n = 327), AUC for BAL, BL, and serum GM were as follows: 0.731 [0.666-0.790], 0.869 [0.816-0.912], and 0.610 [0.540-0.676] with BL samples having the best diagnostic value. GM measurements in patients under posaconazole prophylaxis (n = 114) showed similar diagnostic performance. While specificity was similar between patients with and without posaconazole prophylaxis, sensitivity of GM measurements was lower in patients with prophylaxis. Analyses with patient classified according to antifungal treatment at the time of FOB procedure (n = 166) showed a decreased diagnostic accuracy in serum GM and BAL GM measurements related with the duration of treatment. However, BAL, BL, and serum GM measurements presented similar sensitivity and specificity in higher cut-off values in longer durations of antifungal treatment. CONCLUSION: Our study shows that posaconazole prophylaxis and active short-term (3 days) antifungal treatment do not significantly affect overall diagnostic performance of GM measurements in bronchoalveolar lavage and bronchial lavage samples. However, using different cut-off values for patients receiving active treatment might be suggested to increase sensitivity.


Asunto(s)
Neutropenia Febril , Neoplasias Hematológicas , Hematología , Aspergilosis Pulmonar Invasiva , Neoplasias , Humanos , Antifúngicos/uso terapéutico , Aspergilosis Pulmonar Invasiva/diagnóstico , Aspergilosis Pulmonar Invasiva/tratamiento farmacológico , Aspergilosis Pulmonar Invasiva/prevención & control , Estudios Retrospectivos , Lavado Broncoalveolar , Líquido del Lavado Bronquioalveolar/microbiología , Sensibilidad y Especificidad , Neoplasias Hematológicas/complicaciones , Neutropenia Febril/tratamiento farmacológico , Neutropenia Febril/prevención & control , Mananos/análisis
2.
Hematol Oncol ; 41(4): 663-673, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37211991

RESUMEN

Glofitamab is a CD3xCD20 bi-specific antibody with two fragments directed to the CD20 antigen and a single CD3-binding fragment. Encouraging response and survival rates were recently reported in a pivotal phase II expansion trial conducted in patients with relapsed/refractory (R/R) B-cell lymphoma. However, the real-world data of patients of all ages with no strict selection criteria are still lacking. Herein, this retrospective study aimed to evaluate the outcomes of diffuse large B-cell lymphoma (DLBCL) patients who received glofitamab via compassionate use in Turkey. Forty-three patients from 20 centers who received at least one dose of the treatment were included in this study. The median age was 54 years. The median number of previous therapies was 4, and 23 patients were refractory to first-line treatment. Twenty patients had previously undergone autologous stem cell transplantation. The median follow-up time was 5.7 months. In efficacy-evaluable patients, 21% and 16% of them achieved complete response and partial response, respectively. The median response duration was 6.3 months. The median progression-free survival (PFS) and overall survival (OS) was 3.3 and 8.8 months, respectively. None of the treatment-responsive patients progressed during the study period, and their estimated 1-year PFS and OS rate was 83%. The most frequently reported toxicity was hematological toxicity. Sixteen patients survived, while 27 died at the time of the analysis. The most common cause of death was disease progression. One patient died of cytokine release syndrome during the first cycle after receiving the first dose of glofitamab. Meanwhile, two patients died due to glofitamab-related febrile neutropenia. This is the largest real-world study on the effectiveness and toxicity of glofitamab treatment in R/R DLBCL patients. The median OS of 9 months seems promising in this heavily pretreated group. The toxicity related mortality rates were the primary concerns in this study.

3.
Transfus Apher Sci ; 62(6): 103821, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37775358

RESUMEN

INTRODUCTION: The standard approach for allogeneic stem cell transplantation (allo-SCT) is to administer donor cells on the same day as a fresh product to a patient who has been given a preparative regimen. The difficulty in collecting and transporting donor cells, especially during the COVID-19 pandemic, has made it essential to collect and cryopreserve the grafts before the recipient begins the transplant preparation regimen. However, the short- and long-term impacts of cryopreservation on transplant outcomes remain controversial. MATERIALS AND METHODS: This retrospective study included 93 patients who underwent allo-SCT between January 2012 and August 2022 at the Stem Cell Transplant Unit of Bursa Uludag University Faculty of Medicine using frozen and fresh products of peripheral blood stem cells from a fully matched sibling donor. The effect of cryopreservation of donor grafts on engraftment kinetics was investigated. RESULTS: Frozen and fresh products were used in 37 and 56 patients, respectively. The majority of patients had acute myeloid leukemia and acute lymphoblastic leukemia. The median age at transplantation was 41 years. Neutrophil engraftment time was similar between the two groups (median: 14 vs. 16 days, p = 0.393). Platelet engraftment time was longer in the frozen product group (median: 12 vs. 15 days, p < 0.001). There was no statistically significant difference between freezing time and viability. The acute graft-versus-host disease (GVHD) rate was 37.8 % in the frozen product group and 28.6 % in the fresh product group (p = 0.349). There was no significant difference between the two groups in terms of primary and secondary graft failure, chronic GVHD, 30-day chimerism, relapse, overall survival, progression-free survival, and nonrelapse mortality. CONCLUSION: Having donor cells ready before transplantation significantly prevents donor-induced adverse events and provides confidence and practicality to both the clinician and the recipient. Allo-SCT with frozen products is a successful method that can be safely applied, especially when disruptions in donor-derived cell collection or transportation are foreseen.


Asunto(s)
Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Humanos , Adulto , Estudios Retrospectivos , Pandemias , Trasplante Homólogo , Trasplante de Células Madre Hematopoyéticas/métodos , Enfermedad Injerto contra Huésped/etiología , Trasplante de Células Madre , Criopreservación , Acondicionamiento Pretrasplante/métodos
4.
Ann Hematol ; 101(5): 1049-1057, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-35190843

RESUMEN

Acute promyelocytic leukemia (APL) differs from other forms of acute myeloid leukemia (AML), including coagulopathy, hemorrhage, disseminated intravascular coagulation (DIC), and treatment success with all-trans retinoic acid (ATRA). Despite ATRA, early deaths (ED) are still common in APL. Here, we evaluated factors associated with ED and applicability of scoring systems used to diagnose DIC. Ninety-one APL patients (55 females, 36 males, and median age 40 years) were included. ED was defined as deaths attributable to any cause between day of diagnosis and following 30th day. DIC was assessed based on DIC scoring system released by the International Society of Thrombosis and Hemostasis (ISTH) and Chinese Diagnostic Scoring System (CDSS). Patients' median follow-up time was 49.2 months, and ED developed in 14 (15.4% of) cases. Patients succumbing to ED had higher levels of the Eastern Cooperative Oncology Group Performance Status (ECOG PS), lactate dehydrogenase (LDH), and ISTH DIC, and lower fibrinogen levels (p <0.05). In multivariate Cox regression analysis, age >55 and ECOG PS ≥2 rates were revealed to be associated with ED. Based on ISTH and CDSS scores, DIC was reported in 47.3 and 58.2% of the patients, respectively. Despite advances in APL, ED is still a major obstacle. Besides the prompt recognition and correction of coagulopathy, those at high ED risk are recommended to be detected rapidly. Implementation of local treatment plans and creating awareness should be achieved in hematological centers. Common utilization of ATRA and arsenic trioxide (ATO) may be beneficial to overcome ED and coagulopathy in APL patients.


Asunto(s)
Coagulación Intravascular Diseminada , Leucemia Promielocítica Aguda , Trombosis , Adulto , Coagulación Intravascular Diseminada/terapia , Femenino , Humanos , Masculino , Estudios Retrospectivos , Factores de Riesgo , Trombosis/inducido químicamente , Tretinoina/uso terapéutico
5.
Transfus Apher Sci ; 61(1): 103373, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-35123893

RESUMEN

Graft versus host disease (GVHD) is still the most important cause of mortality and morbidity after allogeneic stem cell transplantation. Though perfect response rates are not achieved, steroids are still the first-line treatment. In the face of the presence of the drugs approved by FDA in recent years for acute and chronic GVHD as second-line therapy in the steroid-refractory group, there exists no standard approach. Extracorporeal photopheresis (ECP) with an immunomodulatory effect, is favored in the treatment of both acute and chronic steroid refractory GVHD as it does not increase the risk of relapses or infections. Having a low profile of side effects, ECP is also generally well-tolerated by patients. Being a time requiring procedure, the fact is that it is not able to be practiced in all health centers and requires central venous catheters in patients unfit for venous access may be enumerated among its shortcomings. No complete standard is available with respect to ECP application frequency-time; it varies from one center to another. The Turkish Society of Apheresis established the Turkish ECP (TECP) group and sought some answers to the questions regarding the use of ECP in the treatment of GVHD, and issued a position statement.


Asunto(s)
Eliminación de Componentes Sanguíneos/métodos , Enfermedad Injerto contra Huésped/terapia , Fotoféresis/métodos , Enfermedad Aguda , Enfermedad Crónica , Humanos , Turquía
6.
J Clin Apher ; 37(5): 430-437, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-35689647

RESUMEN

BACKGROUND: Turkish Stem Cell Coordination Center (TURKOK) carries out the procurement process of unrelated allogeneic hematopoietic stem cells in Turkey. This study aims to compare the efficacy of both once-daily and divided-dose G-CSF administration and the original and biosimilar G-CSF use and the frequency and severity of adverse events in TURKOK donors. METHOD: The study was conducted retrospectively with 142 healthy TURKOK donors. For PBSC mobilization, two different subcutaneous G-CSF programs were used as 10 µ/kg/day single-dose and 5 µ/kg/12 h. Neupogen (Amgen, Puerto Rico) and Tevagrastim (Teva, Kfar Saba, Israel) were used as G-CSF. All donors started apheresis on the fifth day, and all side effects were recorded during the procedure. RESULTS: Stem cell yield was similar between single-dose and divided-doses based on donor weight, favoring the split-dose based on recipient weight (P = .506 and P = .023, respectively). Both G-CSF posologies were comparable if the target CD34+ cell yield was ≥4 × 106 /kg. CD34+ cell yield was equivalent when evaluated against recipient weight, significantly favoring Tevagrastim vs Neupogen by donor weight (P = .740 and P = .021, respectively). Side effects, duration of pain, and need for analgesia favor Tevagratim over Neupogen. CONCLUSION: Split-dose may be recommended for cases where the need for large numbers of CD34+ cells to be harvested is anticipated due to significant cell yield relative to recipient weight. However, sufficient hematopoietic stem cells can be collected with both posology. Tevagrastim is non-inferiority effective to Neupogen. Side effects during administration are both low-grade and temporary.


Asunto(s)
Biosimilares Farmacéuticos , Factor Estimulante de Colonias de Granulocitos , Antígenos CD34/metabolismo , Filgrastim , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Movilización de Célula Madre Hematopoyética/métodos , Células Madre Hematopoyéticas , Humanos , Proteínas Recombinantes , Estudios Retrospectivos , Turquía
7.
Mycopathologia ; 179(1-2): 119-24, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25234793

RESUMEN

Members of the Fusarium solani species complex (FSSC) are causing the majority of the fusariosis in humans. Disseminated fusariosis has a high mortality and is predominantly observed in patients with leukemia. Here, we present the case of a fatal infection by a Fusarium strain with a degenerated phenotype, in a patient with acute lymphatic leukemia. Multiple nasal and skin biopsies as well as blood cultures yielded fungal growth, while in direct and histopathological examination of biopsy material septate hyphae were visible. Initial colonies were white with slimy masses with microconidia reminiscent of Fusarium/Acremonium, but with conidiospore production directly on the hyphae. Multi-locus sequence typing discerned a pionnotal-morphologically degenerated-colony of the recently recognized F. petroliphilum as etiological agent. The culture returned to a typical F. solani species complex morphology only after several weeks of growth in culture. Antifungal susceptibility tests indicate amphotericin B as best drug for this FSSC member rather than any of the azoles or echinocandins.


Asunto(s)
Antifúngicos/uso terapéutico , Fusariosis/tratamiento farmacológico , Fusariosis/mortalidad , Fusarium/efectos de los fármacos , Anfotericina B/uso terapéutico , Cefepima , Cefalosporinas/uso terapéutico , Claritromicina/uso terapéutico , Farmacorresistencia Fúngica , Femenino , Fusariosis/microbiología , Fusarium/clasificación , Humanos , Levofloxacino/uso terapéutico , Pruebas de Sensibilidad Microbiana , Persona de Mediana Edad , Tipificación de Secuencias Multilocus , Técnicas de Tipificación Micológica , Leucemia-Linfoma Linfoblástico de Células Precursoras , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico
8.
Mycoses ; 57(3): 169-75, 2014 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24010950

RESUMEN

Aspergillus infections are major causes of morbidity and mortality among immunocompromised patients. This study was designed to investigate the galactomannan assay optical density (OD) indices relative to the culture results in bronchoscopic samples obtained from neutropenic and non-neutropenic patients. Galactomannan OD indices from 1427 samples from 2005 to 2012, which were sent from 839 patients and were composed of bronchial lavage (BL = 727) and bronchoalveolar lavage fluids (BAL = 700), were retrospectively analysed. The recovery rates of Aspergillus species from these specimens were 9.4% from the combined patient group and 13.3% from the neutropenic group. Aspergillus fumigatus complex was the most frequently isolated species. The mean and median OD indices of the positive and negative culture samples are approximately 5 and 1, respectively, and 91% of all culture-positive samples have ≥1 OD index value. The receiver-operating characteristics curve analysis demonstrated that the feasibility of the Aspergillus galactomannan assay and Aspergillus galactomannan test has superior accuracy in BAL compared to BL fluids, and the test is not affected by the immune status of the patient. We suggest that the Aspergillus galactomannan test, which uses bronchoscopic material, leads to an earlier diagnosis and if the OD index is found ≥1, fungal growth can be expected.


Asunto(s)
Aspergillus fumigatus/aislamiento & purificación , Enfermedades Pulmonares Fúngicas/diagnóstico , Mananos/química , Neutropenia/metabolismo , Aspergilosis/diagnóstico , Líquido del Lavado Bronquioalveolar/microbiología , Galactosa/análogos & derivados , Neoplasias Hematológicas/microbiología , Humanos , Neutropenia/microbiología , Estudios Retrospectivos , Manejo de Especímenes
9.
Turk J Haematol ; 41(2): 97-104, 2024 05 30.
Artículo en Inglés | MEDLINE | ID: mdl-38545671

RESUMEN

Objective: Primary immune thrombocytopenia (pITP) is an acquired autoimmune disorder related to the increased destruction and/or impaired production of platelets. Its diagnosis and management are challenging and require expertise and the interpretation of international consensus reports and guidelines with national variations in availability. We aimed to assess the agreement of hematologists in Türkiye on certain aspects of both first-line and second-line management of patients with pITP. Materials and Methods: Applying a modified Delphi method, the Turkish National ITP Working Group (14 steering committee members), founded under the auspices of the Turkish Society of Hematology, developed a 21-item questionnaire consisting of statements regarding the first-line and second-line treatment of pITP. A total of 107 adult hematologists working in either university or state hospitals voted for their agreement or disagreement with the statements in two consecutive rounds. Results: The participants reached consensus on the use of corticosteroids as first-line treatment and with limited duration. Methylprednisolone was the corticosteroid of choice rather than dexamethasone. Use of intravenous immunoglobulin was not preferred for patients without bleeding. It was also agreed that thrombopoietin receptor antagonists (TPO-RAs) or rituximab should be recommended as second-line treatment and that splenectomy could be considered 12-24 months after diagnosis in patients with chronic pITP. Conclusion: The optimization of the dose and duration of TPO-RAs in addition to corticosteroids is necessary to improve the management of patients with pITP.


Asunto(s)
Consenso , Púrpura Trombocitopénica Idiopática , Humanos , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/terapia , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Adulto , Técnica Delphi , Manejo de la Enfermedad , Encuestas y Cuestionarios , Turquía/epidemiología , Esplenectomía , Corticoesteroides/uso terapéutico , Femenino , Guías de Práctica Clínica como Asunto
10.
Turk J Haematol ; 2024 May 27.
Artículo en Inglés | MEDLINE | ID: mdl-38801066

RESUMEN

Introduction: Primary immune thrombocytopenia (ITP) is an acquired disorder of platelets with complex and unclear mechanism of increased immune distruction or impaired production of platelets. While management of ITP is evolving, there is a need for guidance particularly in certain circumstances such as pregnancy, emergency and for patients requiring co-medications. We aimed to determine the tendencies of hematologists in Turkiye on such special conditions. Methods: As a modified Delphi method, Turkish National ITP Working Group founded under Turkish Society of Hematology developed a questionnaire consisting of statements regarding pregnancy, emergency and circumstances regarding co-treatment with antiaggregant or anticoagulants. 107 Hematologists working either in university or state hospitals voted for their agreement or disagreement of the statements for two consequential rounds. Results: Participant hematologists reached an agreement on the starting treatment in pregnant patients with platelets less than 30 x109/L and delivery of either normal or cesarian section to be safely performed above 50 x109/L. For emergency and rescue management of ITP, our panel have agreed against the use of high dose corticosteroids alone, preferred a combination with transfusion or IVIG. For patients who require interventions, platelet counts >50 x109/L were regarded as safe for low risk procedures as well as co-treatment with antiplatelets or anticoagulants. Conclusion: As National ITP study group, we have observed the need to increase the practice guidance in patients with primary ITP requiring additional treatments including invasive interventions, and co-treatments towards coagulation. Decisions on the management of ITP during pregnancy should be individualized. There is a certain lack of consensus on the thresholds of platelet counts as well as co-morbidities and co-medications. This lack of consensus may be due to the variations in the practices.

11.
Sci Rep ; 14(1): 13843, 2024 06 15.
Artículo en Inglés | MEDLINE | ID: mdl-38879594

RESUMEN

Myelodysplastic syndrome (MDS) is a heterogeneous spectrum of clonal hematopoietic disorders with varying degrees of cytopenia and morphologic dysplasia. The hemoglobin, albumin, lymphocyte, and platelet (HALP) score is a prognostic marker in several types of malignant tumors. Prognostic value of HALP score remains unclear for MDS. To determine the prognostic value of baseline HALP score in MDS. We retrospectively analyzed data from 130 newly diagnosed MDS patients evaluated and classified under HALP score. By the receiver operating characteristic (ROC) analysis, the optimal cut-off value of HALP was > 67.5 in predicting mortality. Patients were divided into two groups: with low and high HALP scores, and the characteristics were compared between both groups. Patients' median age was 68 (19-84) years, and 79 (60.8%) were male. Higher HALP score was detected in MDS patients with intermediate-risk under IPSS score, and at high and very high risks under IPSS-R score, and those receiving azacitidine (AZA) treatment. The survival rates of those with a HALP score > 67.5 were significantly lower than those with low HALP score at 17.77 ± 3.98 (median ± SE) (p < 0.001). The 3-, 5- and 10-years survival rates of individuals with HALP scores > 67.5 were found as 25, 18, and 11%, respectively. Median overall survival (OS) was also determined as 33.10 (95% CI 16.34-49.88) months by the Kaplan-Meier method. HALP score has shown an ability to be a useful prognostic biomarker in various cancers, including MDS. The meaningful cut-off value of HALP is disease-specific and largely study-specific. High HALP score is associated with unfavorable clinicopathological characteristics. Also, it may be useful in predicting OS and mortality of MDS.


Asunto(s)
Síndromes Mielodisplásicos , Humanos , Síndromes Mielodisplásicos/mortalidad , Síndromes Mielodisplásicos/diagnóstico , Síndromes Mielodisplásicos/tratamiento farmacológico , Síndromes Mielodisplásicos/patología , Masculino , Anciano , Femenino , Persona de Mediana Edad , Pronóstico , Anciano de 80 o más Años , Adulto , Estudios Retrospectivos , Hemoglobinas/análisis , Hemoglobinas/metabolismo , Adulto Joven , Curva ROC , Plaquetas/patología , Linfocitos/patología , Recuento de Plaquetas
12.
Transplant Proc ; 55(3): 715-717, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36931953

RESUMEN

Aplastic anemia is potentially fatal, particularly if the disease does not respond to immunotherapy and progresses to severe pancytopenia. Allogeneic hematopoietic stem cell transplant from an HLA-matched sibling donor, the first-line treatment in patients younger than 40 years, is used as a curative treatment option in severe aplastic anemia. The availability of an identical twin donor is infrequent, and there is limited experience in this context. Additionally, the choices for a conditioning regimen for a syngeneic transplant to prevent engraftment failure and the necessity of graft-vs-host disease prophylaxis are controversial. Although long-term survival gradually increases after an allogeneic hematopoietic stem cell transplant, hypogonadism and infertility are the main problems that significantly affect patients' quality of life. We present a patient diagnosed with severe aplastic anemia who has had a healthy pregnancy immediately after a syngeneic transplant.


Asunto(s)
Anemia Aplásica , Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Humanos , Embarazo , Femenino , Anemia Aplásica/cirugía , Anemia Aplásica/complicaciones , Trasplante Isogénico/efectos adversos , Trasplante Homólogo/efectos adversos , Calidad de Vida , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Enfermedad Injerto contra Huésped/etiología , Acondicionamiento Pretrasplante
13.
Sci Rep ; 13(1): 12539, 2023 08 02.
Artículo en Inglés | MEDLINE | ID: mdl-37532753

RESUMEN

The impact of inflammatory markers such as systemic immune-inflammation (SII) index and systemic inflammation response index (SIRI) on myelofibrosis (MF) prognosis was evaluated for the first time in this study. Data from 60 patients diagnosed with MF between March 2011 and September 2022 were retrospectively analyzed. In addition to disease-related markers, the impact of SII and SIRI on prognosis was evaluated. In our study, the overall median survival (OS) was 64 months. OS was significantly shorter in patients older than 65 years, with high ferritin and lymphocyte levels, transfusion dependence at diagnosis, platelet count below 100 × 109/L, Hb level below 8 g/dl, and high risk according to the dynamic international prognostic scoring system (DIPSS)-Plus score. When these variables were included in the multivariate Cox regression model, it was found that being older than 65 years, having a high ferritin value, being at high risk according to the DIPSS-plus score and Hb values below 8 increased the risk of death. Platelet-to-lymphocyte ratio (PLR) and SII index were lower in patients with a fatal outcome. No statistically significant relationship was found between SIRI and mortality. The findings of this study showed that low PLR and high ferritin were associated with poor prognosis in MF. Elevated SII and SIRI, evaluated for the first time in patients with myelofibrosis, did not predict prognosis. Since non-inflammatory variables play a role in the pathogenesis of MF, bone marrow indicators and systemic inflammation indicators derived from hematologic parameters may not be accurate.


Asunto(s)
Mielofibrosis Primaria , Humanos , Estudios Retrospectivos , Mielofibrosis Primaria/diagnóstico , Pronóstico , Inflamación/patología , Ferritinas
14.
Int J Lab Hematol ; 45(5): 751-757, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37309683

RESUMEN

INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening occlusive disease of the microcirculation characterized by systemic platelet plugs, organ ischemia, deep thrombocytopenia, and fragmentation of erythrocytes. One of the widely used scoring system to determine the clinical probability of TTP is the PLASMIC scoring system. This study aimed to evaluate the contribution of PLASMIC score modifications to sensitivity and specificity in patients with microangiopathic hemolytic anemia (MAHA) undergoing plasma exchange with a prediagnosis of TTP at our center. MATERIALS AND METHODS: The data of patients who were hospitalized with a previous diagnosis of MAHA and TTP and underwent plasma exchange at Bursa Uludag University, Faculty of Medicine, Department of Hematology between January 2000 and January 2022 were retrospectively analyzed. RESULTS: Overall, 33 patients (including 15 and 18 with and without TTP, respectively) were included in this study. Receiver operating characteristic (ROC) analysis revealed that the area under the curve (AUC) for the original PLASMIC score was 0.985 (95% confidence interval [95% CI]: 0.955-1.000), and AUC for the PLASMIC score without mean corpuscular volume (MCV) was 0.967 (95% CI: 0.910-1.000), which is close to the original AUC. With the removal of MCV from the scoring system, the sensitivity decreased from 100% to 93%, whereas the specificity increased from 33% to 78%. CONCLUSIONS: Based on the results of this validation study, removing MCV from the PLASMIC score led to the categorization of eight non-TTP cases in the low-risk category, and this could avoid unnecessary plasma exchange. However, in our study increasing the specificity was at the expense of the sensitivity by missing one patient with this new scoring system without MCV. Further multicenter studies with large sample sizes are required owing to the fact that different parameters may be effective in TTP prediction among different populations.


Asunto(s)
Anemia Hemolítica , Púrpura Trombocitopénica Trombótica , Humanos , Estudios Retrospectivos , Proteína ADAMTS13 , Intercambio Plasmático
15.
Ther Apher Dial ; 27(4): 802-812, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37039816

RESUMEN

INTRODUCTION: Our study investigated leukapheresis's effect on delayed induction therapy outcomes in patients with acute leukemia presenting with symptomatic hyperleukocytosis. METHODS: This retrospective cohort study included 30 adult patients diagnosed with acute leukemia who underwent leukapheresis for leukostasis. The patients were divided into the first 24 h and >24 h groups, according to the time from diagnosis to induction therapy (TDT). RESULTS: There was no significant difference between the TDT groups regarding complete remission (CR), 4-week mortality, and overall survival (OS) at a median follow-up of 409 days. Tumor lysis syndrome, disseminated intravascular coagulation, and hemoglobin levels were significant in early mortality. In univariate analysis, age, hemoglobin levels, patients' eligibility for intensive chemotherapy, and achieving CR were critical factors for OS. CONCLUSION: The study findings suggest that waiting for the clinical and laboratory results may be a safe and reasonable approach before assigning patients the best treatment option with leukapheresis.


Asunto(s)
Leucaféresis , Leucemia Mieloide Aguda , Adulto , Humanos , Leucaféresis/métodos , Estudios Retrospectivos , Quimioterapia de Inducción/métodos , Leucocitosis/terapia , Leucocitosis/patología , Leucemia Mieloide Aguda/terapia , Leucemia Mieloide Aguda/diagnóstico , Pronóstico , Enfermedad Aguda , Hemoglobinas
16.
Turk J Haematol ; 40(4): 251-257, 2023 12 05.
Artículo en Inglés | MEDLINE | ID: mdl-37791641

RESUMEN

Objective: In recent years, new developments have been incorporated into daily practice in the management of immune thrombotic thrombocytopenic purpura (iTTP). In particular, clinical scoring systems could help clinicians with clinical decision-making and early recognition. However, older patients frequently present with more organ involvement and in unusual ways. The ways in which age could affect these clinical prediction scoring systems remain unclear. We evaluated the use of PLASMIC and French scores in patients over 60 years of age. Materials and Methods: We performed a retrospective cross-sectional analysis of patients over 60 years of age with a presumptive diagnosis of iTTP between 2014 and 2022 at 10 centers. We calculated PLASMIC and French scores and compared our data with a single-center analysis of younger patients presenting with thrombotic microangiopathy. Results: Our study included 30 patients over 60 years of age and a control group of 28 patients younger than 60 years. The diagnostic sensitivity and specificity of a French score of ≥1 were lower in older patients compared to the control group (78.9% vs. 100% and 18.2% vs. 57.1%, respectively). The diagnostic sensitivity and specificity of a PLASMIC score of ≥5 were 100% vs. 95% and 27.3% vs. 100% for the study group and control group, respectively. Our study showed a higher mortality rate in older patients compared to the control group (30% vs. 7.1%, p=0.043). Conclusion: For a limited number of patients (n=6), our results showed that rituximab can reduce mortality. Given that the reliability of clinical prediction scores for iTTP in older patients may be lower, more caution must be undertaken in interpreting their results.


Asunto(s)
Púrpura Trombocitopénica Idiopática , Púrpura Trombocitopénica Trombótica , Trombosis , Microangiopatías Trombóticas , Humanos , Anciano , Persona de Mediana Edad , Púrpura Trombocitopénica Trombótica/diagnóstico , Púrpura Trombocitopénica Trombótica/terapia , Estudios Retrospectivos , Estudios Transversales , Reproducibilidad de los Resultados , Microangiopatías Trombóticas/diagnóstico , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/terapia , Proteína ADAMTS13
17.
Turk J Haematol ; 40(4): 242-250, 2023 12 05.
Artículo en Inglés | MEDLINE | ID: mdl-37961952

RESUMEN

Objective: This study aimed to evaluate patients with relapsed/refractory multiple myeloma (RRMM) who underwent daratumumab (DARA) therapy. Materials and Methods: This multicenter retrospective study included 134 patients who underwent at least two courses of DARA from February 1, 2018, to April 15, 2022. Epidemiological, disease, and treatment characteristics of patients and treatment-related side effects were evaluated. Survival analysis was performed. Results: The median age at the start of DARA was 60 (range: 35-88), with 56 patients (41.8%) being female and 48 (58.2%) being male. The median time to initiation of DARA and the median follow-up time were 41.2 (5.1-223) and 5.7 (2.1-24.1) months, respectively. The overall response rate after DARA therapy was 75 (55.9%), and very good partial response or better was observed in 48 (35.8%) patients. Overall survival (OS) and progression-free survival (PFS) for all patients were 11.6 (7.8-15.5) and 8.0 (5.1-10.9) months, respectively. OS was higher for patients undergoing treatment with DARA and bortezomib-dexamethasone (DARA-Vd) compared to those undergoing treatment with DARA and lenalidomide-dexamethasone (DARA-Rd) (16.9 vs. 8.3 months; p=0.014). Among patients undergoing DARA-Rd, PFS was higher in those without extramedullary disease compared to those with extramedullary disease (not achieved vs. 3.7 months; odds ratio: 3.4; p<0.001). The median number of prior therapies was 3 (1-8). Initiation of DARA therapy in the early period provided an advantage for OS and PFS, although it was statistically insignificant. Infusion-related reactions were observed in 18 (13.4%) patients. All reactions occurred during the first infusion and most reactions were of grade 1 or 2 (94.5%). The frequency of neutropenia and thrombocytopenia was higher in the DARA-Rd group (61.9% vs. 24.7%, p<0.001 and 42.9% vs. 15.7%, p<0.001). Conclusion: Our study provides real-life data in terms of DARA therapy for patients with RRMM and supports the early initiation of DARA therapy.


Asunto(s)
Mieloma Múltiple , Femenino , Humanos , Masculino , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Dexametasona/uso terapéutico , Lenalidomida/uso terapéutico , Mieloma Múltiple/tratamiento farmacológico , Neutropenia , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años
18.
Mol Biol Rep ; 39(9): 8663-7, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-22722988

RESUMEN

An activating mutation of Janus kinase 2 (JAK2-V617F) was previously described in chronic myeloproliferative disorders (MPD). In previously published studies, the frequency of the JAK2-V617F mutation was determined to be 80-90 % for patients with polycythemia vera (PV) and 40-70 % for essential thrombocythemia (ET). In this study, we analyzed the relationship between the JAK2-V617F mutation and clinical-hematological parameters in Turkish patients with MPD and compared these findings with published studies from other geographic regions. A total of 148 patients were studied; of which, 70 were diagnosed with PV and 78 with ET. The mutation status of JAK2 was determined using a tetra-primer polymerase chain reaction. We found that 80 % of the PV group and 42 % of the ET group were positive for the JAK2-V617F mutation. When all patients were analyzed, the levels of white blood cells, hemoglobin and splenomegaly were significantly different in patients with the JAK2-V617F mutation (p < 0.05). To our knowledge, this study is the first to evaluate the relationship between MPD and JAK2-V617F in Turkish patients. The JAK2-V617F mutation is frequently detected in the Turkish patients with MPD, and especially in patients with PV. Hence, it would be useful to include JAK2 mutation screening in the initial evaluation of patients suspected to have MPD.


Asunto(s)
Janus Quinasa 2/genética , Mutación , Policitemia Vera/genética , Trombocitemia Esencial/genética , Adulto , Anciano , Femenino , Genotipo , Humanos , Masculino , Persona de Mediana Edad , Tasa de Mutación , Trastornos Mieloproliferativos/genética , Turquía
19.
Exp Clin Transplant ; 2022 Oct 18.
Artículo en Inglés | MEDLINE | ID: mdl-36259607

RESUMEN

Although anemia is common after kidney transplant, pure erythroid aplasia due to parvovirus B19 infection is rare. Therefore, there are delays in diagnosis in transplant patients. Here, we aimed to raise awareness that pure red blood cell aplasia due to parvovirus B19 should be considered in the differential diagnosis of posttransplant anemia. Our report analyzes 2 kidney transplant recipients under immunosuppressive therapy who were diagnosed with pure red blood cell aplasia due to parvovirus B19 infection. Both patients were examined for anemia as a cause for transfusion dependence. Normochromic, normocytic anemia, and reticulocyte levels were low. Leukocyte and platelet counts and biochemical parameters were within reference ranges. Therefore, pure red blood cell aplasia associated with parvovirus B19 was included in the differential diagnosis. Bone marrow showed erythroid hypoplasia and megaloblastic changes with giant erythroblasts containing dark-stained inclusion structures. Results from the other series (neutrophils, lymphocytes, platelets) were within reference ranges. Parvovirus B19 immunoglobulin M and G levels were negative in both patients, yet serum parvovirus B19 DNA polymerase chain reaction test results were positive. Therefore, diagnosis was parvovirus B19-associated pure red blood cell aplasia. Anemia resolved completely by 4 weeks after reduction of immunosuppression and intravenous immunoglobulin. Both patients relapsed in month 5 of treatment. One patient relapsed 3 times during follow-up, with complete response to intravenous immunoglobulin for all 3 events. The second patient showed partial response to intravenous immunoglobulin after relapse. We suggest that pure red blood cell aplasia associated with parvovirus B19 should be considered in transplant patients who present with anemia and reticulocytopenia. Negative serology does not exclude the diagnosis, and it is important to perform a parvovirus B19 DNA polymerase chain reaction test. Intravenous immunoglobulin therapy is effective to cure anemia within weeks. Follow-up of patients is important because relapse may occur after treatment.

20.
Mediterr J Hematol Infect Dis ; 14(1): e2022039, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35615332

RESUMEN

Background: This study aimed to evaluate the effects of the appropriate use of empiric glycopeptide therapy in hematologic malignancy patients with febrile neutropenia (FN). Materials and Methods: Patients with FN who were hospitalized in our clinic and started empiric glycopeptide therapy were retrospectively analyzed. Empiric glycopeptide treatment initial indications were determined according to 7 specific criteria in the IDSA guidelines. In addition, the duration of glycopeptide use according to initial indications, causative pathogens in culture positivity, frequency of VRE infection, and the mortality rate was identified. Results: 87 patients were included. Of these, 102 episodes of FN were analyzed. Appropriate use of glycopeptides was observed in 98% of patients. The most common initial indication for glycopeptide was skin or soft-tissue infection, with 52% (n = 53). The mean duration of glycopeptide use was 11 (2-22) days. The time of glycopeptide use was longer in patients with catheter-related infections than in those with severe mucositis and hemodynamic instability (p = 0,041/p = 0,016). The duration of glycopeptide use was shorter in patients with consolidation therapy than in those without consolidation therapy. The mortality rate in culture-positive patients was significantly higher than in culture-negative patients (p = 0.041). At 72 h, glycopeptide therapy was discontinued in 8 of 79 FN episodes within culture-negative patients. Conclusion: This study showed that the mortality rate was higher in culture-positive patients. Additionally, glycopeptides should be discontinued early with no evidence of gram-positive infection.

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