Browsing by Author "Akcay, Arzu"

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Extremity Necrosis Due to Intrauterine Arterial Ischemia
(GALENOS YAYINCILIK, 2021-01-01) Beken, Serdar; Sariyilmaz, Kerim; Albayrak, Eda; Akcay, Arzu; Korkmaz, Ayse
Hematopoietic Stem Cell Transplantation in Patients with Heterozygous STAT1 Gain-of-Function Mutation
(SPRINGER/PLENUM PUBLISHERS, 2019-01-01) Kiykim, Ayca; Charbonnier, Louis Marie; Akcay, Arzu; Karakoc-Aydiner, Elif; Ozen, Ahmet; Ozturk, Gulyuz; Chatila, Talal A.; Baris, Safa
PurposeHuman signal transducer and activator of transcription 1 (STAT1) gain-of-function (GOF) mutations present with a broad range of manifestations ranging from chronic mucocutaneous candidiasis and autoimmunity to combined immunodeficiency (CID). So far, there is very limited experience with hematopoietic stem cell transplantation (HSCT) as a therapeutic modality in this disorder. Here, we describe two patients with heterozygous STAT1 GOF mutations mimicking CID who were treated with HSCT.MethodsData on the HSC sources, conditioning regimen, graft-versus-host disease (GvHD) and antimicrobial prophylaxis, and the post-transplant course including engraftment, GvHD, transplant-related complications, infections, chimerism, and survival were evaluated. Pre- and post-transplant immunological studies included enumeration of circulating interferon gamma (IFN-)- and interleukin 17 (IL-17)-expressing CD4(+) T cells and analysis of IFN--induced STAT1 phosphorylation in patient 1 (P1)'s T cells.ResultsP1 was transplanted with cord blood from an HLA-identical sibling, and P2 with bone marrow from a fully matched unrelated donor using a reduced toxicity conditioning regimen. While P1 completely recovered from her disease, P2 suffered from systemic CMV disease and secondary graft failure and died due to severe pulmonary involvement and hemorrhage. The dysregulated IFN- production, suppressed IL-17 response, and enhanced STAT1 phosphorylation previously found in the CD4(+) T cells of P1 were normalized following transplantation.ConclusionHSCT could be an alternative and curative therapeutic option for selected STAT1 GOF mutant patients with progressive life-threatening disease unresponsive to conventional therapy. Morbidity and mortality-causing complications included secondary graft failure, infections, and bleeding.
Mesenchymal Stem Cell Treatment for Steroid Refractory Graft-versus-Host Disease in Children: A Pilot and First Study from Turkey
(HINDAWI LTD, 2016-01-01) Erbey, Fatih; Atay, Didem; Akcay, Arzu; Ovali, Ercument; Ozturk, Gulyuz
This study evaluated the efficacy of mesenchymal stem cells (MSCs) from bone marrow of a third-party donor for refractory aGVHD. We report the first experience using MSCs to treat refractory aGVHD in 33 pediatric patients undergoing allogeneic HSCT from Turkey. Totally, 68 doses of bone marrow derived MSCs were infused. The median dose of MSC was 1.18 x 10(6) cells per kg body weight. Overall, complete response (CR) was documented in 18 patients, partial response (PR) was documented in 7 patients, and no response (NR) was documented in 8 patients. The 2-year estimated probability of overall survival (OS) for patients achieving CR and PR/NR was 63.8\% and 29.4\%, respectively (p = 0.0002). While the cumulative incidence of transplant related mortality (TRM) at day 100 after first MSC infusion was 46.6\% in PR/NR patients, there was no any TRM at day 100 after first MSC infusion in CR patients (p = 0.001). Twelve patients developed chronic GVHD (cGVHD)
Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging
(HINDAWI LTD, 2015-01-01) Keskindemirci, Gonca; Tugcu, Deniz; Aydogan, Gonul; Akcay, Arzu; Ayaz, Nuray Aktay; Er, Ali; Yekeler, Ensar; Bilgic, Bilge
Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old boy with local aggressive vascular lesions associated with KMP. Magnetic resonance imaging revealed an extensive lesion at paravertebral and retroperitoneal regions that was infiltrating vertebrae. Although we did not get any response to steroid or propranolol treatment, partial response was observed radiologically with interferon-alpha treatment. Unfortunately, the patient died because of the uncontrolled consumptive coagulopathy that led to intracranial hemorrhage which was caused by huge knee hematoma after minor trauma.
Sustained hyperferritinemia in a child with macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis - perforinopathy: case based review
(TURKISH J PEDIATRICS, 2018-01-01) Cakan, Mustafa; Aktay-Ayaz, Nuray; Gemici, Hakan; Annayev, Agageldi; Citak, Agop; Akcay, Arzu; Ozturk, Gulyuz
Systemic juvenile idiopathic arthritis is a subtype of juvenile idiopathic arthritis and characterized by arthritis and many systemic features like fever, rash, hepatosplenomegaly, lymphadenopathy and serositis. Macrophage activation syndrome is the most dreadful complication of systemic juvenile idiopathic arthritis and can cause mortality and morbidity if not recognized and treated early and aggressively. Hemophagocytic lymphohistiocytosis (HLH) is characterized by diminished or absent activities of natural killer cells and cytotoxic T lymphocytes leading to cytokine storm and uncontrolled activation of T cells and macrophages. Primary (familial) HLH is a group of autosomal recessive disorders caused by mutations in the perforin and other related genes and distinctive for onset during early infancy and high rate of mortality. Secondary HLH may be caused by infectious, oncologic and rheumatologic disorders. The term Perforinopathy is used to describe cases with classical familial HLH and also for cases with familial HLH gene mutations but not following a classical familial HLH course. Herein we report a case of chronic perforinopathy in which clinical symptoms started with systemic juvenile idiopathic arthritis and severe macrophage activation syndrome that needed plasma exchange and extracorporeal membrane oxygenation during acute period and ongoing interleukin-1 blockage for sustained hyperferritinemia.
The Approaches of Physicians Working in the Field of Pathology Regarding Forensic Pathology Practice and the Training Process
(DE GRUYTER OPEN LTD, 2013-01-01) Ersoy, Gokhan; Ozoran, Yavuz; Akcay, Arzu; Kolusayin, Melek Ozlem; Pakis, Isil; Urer, Halide Nur; Gulmen, Mete Korkut; Oz, Buge
Objective: Forensic autopsies are performed by the forensic medicine department and the microscopic examination processes by pathology specialists within the forensic medicine practice in Turkey. This disconnection in the process raises problems in the training of both branches. The aim of this study was to determine the awareness of pathology staff on forensic medicine practices and responsibilities and their opinion on the pathology training model in the forensic medicine specialty and to discuss the matter within the framework of the present situation and global applications. Material and Method: A 15-item questionnaire form distributed to the participant physicians during registration at the 21st National Pathology Congress held in 2011 was evaluated. Results: 94 participants responded. A negative opinion was expressed by 72\% about the interest in the general post-mortem process. The view that pathology specialists should undergo a separate training to perform autopsies was predominant and there was a general lack of interest in all kinds of autopsy processes. The percentage who said they knew the legal responsibility of a pathology specialist regarding forensic autopsies correctly was 37\%. The questions ``what are the necessary factors to contribute to the pathology training in forensic medicine{''} and ``if anything is required, which of them would take priority{''} were respectively answered as ``for me to be interested (46\%){''} and ``a system guaranteeing that training will always be given by pathology specialists (67\%){''}. Despite the possibility of becoming a forensic medicine specialist in two years, the mean answer score of the participants to the phrase `` I do not consider becoming a forensic medicine specialist{''} was 4.1 (out of 5). Conclusion: A reluctance among the pathologists in our country was seen regarding forensic medicine specialists being able to perform post-mortem microscopic examination. However, despite their legal responsibilities, their interest in forensic pathology practice was low. There seems to be rational factor that would increase this interest in the near future. Cooperation is necessary to enable forensic medicine specialists to perform post-mortem pathology procedures. This cooperation should be based on improving the training of pathology research assistants.
Thrombolysis with Systemic Recombinant Tissue Plasminogen Activator in Children: A Multicenter Retrospective Study
(GALENOS YAYINCILIK, 2021-01-01) Zengin, Emine; Sarper, Nazan; Erdem, Arzu Yazal; Al, Isik Odaman; Evim, Melike Sezgin; Yarali, Nese; Belen, Burcu; Akcay, Arzu; Yildirim, Aysen Turedi; Karapinar, Tuba Hilkay; Gunes, Adalet Meral; Gelen, Sema Aylan; Oren, Hale; Olcay, Lale; Baytan, Birol; Gulen, Huseyin; Ozturk, Gulyuz; Orhan, Mehmet Fatih; Oymak, Yesim; Akpinar, Sibel; Tufekci, Ozlem; Albayrak, Meryem; Gunen, Burcak Tatli; Canpolat, Aylin; Ozbek, Namik
Objective: This study aimed to evaluate systemic thrombolysis experiences with recombinant tissue plasminogen activator (rtPA). Materials and Methods: Retrospective data were collected from 13 Turkish pediatric hematology centers. The dose and duration of rtPA treatment, concomitant anticoagulant treatment, complete clot resolution (CCR), partial clot resolution (PCR), and bleeding complications were evaluated. Low-dose (LD) rtPA treatment was defined as 0.01-0.06 mg/kg/h and high-dose (HD) rtPA as 0.1-0.5 mg/kg/h. Results: Between 2005 and 2019, 55 thrombotic episodes of 54 pediatric patients with a median age of 5 years (range: 1 day to 17.75 years) were evaluated. These patients had intracardiac thrombosis (n=16), deep vein thrombosis (DVT) (n=15), non-stroke arterial thrombosis (n=14), pulmonary thromboembolism (PE) (n=6), and stroke (n=4). The duration from thrombus detection to rtPA initiation was a median of 12 h (range: 2-504 h) and it was significantly longer in cases of DVT and PE compared to stroke, non-stroke arterial thrombosis, and intracardiac thrombosis (p=0.024). In 63.6\% of the episodes, heparin was initiated before rtPA treatment. LD and HD rtPA were administered in 22 and 33 of the episodes, respectively. Concomitant anticoagulation was used in 90\% and 36\% of the episodes with LD and HD rtPA, respectively (p=0.0001). Median total duration of LD and HD rtPA infusions was 30 h (range: 2-120 h) and 18 h (2-120 h), respectively (p=0.044). Non-fatal major and minor bleeding rates were 12.5\% and 16.7\% for LD and 3.2\% and 25.8\% for HD rtPA, respectively. At the end of the rtPA infusions, CCR and PCR were achieved in 32.7\% and 49.0\% of the episodes, respectively. The most successful site for thrombolysis was intracardiac thrombosis. HD versus LD rtPA administration was not correlated with CCR/PCR or bleeding (p>0.05). Conclusion: Systemic thrombolytic therapy may save lives and organs effectively if it is used at the right indications and the right times in children with high-risk thrombosis by experienced hematologists with close monitoring of recanalization and bleeding.