Extracorporeal photopheresis did not prevent the development of an autoimmune disease: myasthenia gravis

WOS: 000390578300026PubMed ID: 27612294BACKGROUNDMyasthenia gravis (MG) is a neuromuscular disorder characterized by an autoimmune defect in the neuromuscular junction. In most patients, the autoimmune attack is mediated by antibodies against the acetylcholine receptor (AChR) on the postsynaptic membrane. Deficient immunoregulation, including regulatory T cells, is consistently observed. Extracorporeal photopheresis (ECP) leads to the induction of regulatory T cells that mediate immunologic tolerance in autoimmune diseases; however, the data regarding MG are very limited. CASE REPORTHere, we report a patient who, during ongoing ECP therapy for his severe, refractory, chronic graft-versus-host disease (cGVHD), developed MG, although he responded very well to ECP, as indicated by the lowering of his chronic cGVHD severity grade to moderate. RESULTSDespite receiving ECP, our patient developed MG, which was resistant to treatment and required intensive care unit support. CONCLUSIONSClose surveillance is required when ECP is planned as one of the treatment alternatives in myasthenia gravis that develop in cGVHD

Neuobičajeni klinički slučajevi koji oponašaju akutni diseminirani encefalomijelitis

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated monophasic inflammatory demyelinating disorder of the central nervous system which poses a diagnostic challenge. We report on six cases of different etiologies that mimicked the clinical and radiologic findings of ADEM. The cases were collected from four different reference hospitals in Turkey. The same radiologist from the Akdeniz University Faculty of Medicine examined the magnetic resonance images of all patients. Three (50%) patients had antecedent infections. Initial symptoms of the patients were as follows: fever in 50%, altered consciousness in 33.3% and convulsions in 16.7% of patients. Neurologic examination showed long tract signs in 83.3%, ataxia in 50% and altered consciousness in 50% of patients. Cerebrospinal fluid examination revealed lymphocytic pleocytosis only in case 6. Four patients received steroid pulse therapy and one of these initially underwent intravenous immunoglobulin therapy. The patients’ definitive diagnoses were as follows: paraspinal neuroblastoma-associated paraneoplastic syndrome; histiocytic sarcoma; mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes; and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy in one patient each, while two patients had hemophagocytic syndrome. The present case series demonstrated difficulties in diagnosing ADEM while revealing extremely rare disorders that mimic ADEM radiologically and clinically.Akutni diseminirani encefalomijelitis (ADEM) je immuno posredovana monofazna upalna demijelinizacijska bolest središnjega živčanog sustava koja je dijagnostički vrlo zahtjevna. Prikazuje se šest slučajeva različite etiologije gdje su klinički i radiološki nalazi oponašali ADEM. Slučajevi su prikupljeni iz četiri različite referentne bolnice u Turskoj. Slikovne prikaze dobivene magnetskom rezonancom u svih bolesnika pregledao je isti radiolog s Medicinskog fakulteta Sveučilišta u Akdenizu. Troje (50%) bolesnika imalo je prethodnu infekciju. Početni simptomi bili su groznica u 50%, poremećaj svijesti u 33,3% te konvulzije u 16,7% bolesnika. Neurološki pregled pokazao je znakove oštećenja srednjeg ili gornjeg dijela leđne moždine (long tract signs) u 83,3%, ataksiju u 50% te poremećaj svijesti u 50% bolesnika. Pregled likvora otkrio je limfocitnu pleocitozu samo u slučaju br. 6. Četiri bolesnika primilo je pulsnu steroidnu terapiju, a jedan od njih je prvotno bio na terapiji intravenskim globulinom. U bolesnika su postavljene sljedeće konačne dijagnoze: paraneoplastični sindrom udružen s paraspinalnim neuroblastomom; histiocitni sarkom; mitohondrijska miopatija, encefalopatija, laktična acidoza i epizode slične moždanom udaru (MELAS); i cerebralna autosomna dominantna arteriopatija sa subkortikalnim infarktima i leukoencefalopatijom (CADASIL) u po jednog bolesnika, te hemofagocitni sindrom u dvoje bolesnika. Ovaj niz slučajeva ukazuje na teškoće u dijagnosticiranju ADEM-a i istodobno pokazuje iznimno rijetke bolesti koje radiološki i klinički oponašaju ADEM

Hematopoietic stem cell transplantation and high dose chemotherapy in recurrent and/or chemotherapy resistant hodgkin lymphoma cases: A single center experience

Giriş: Standart tedavi alan Hodgkin Lymphoma (HL) hastalarının yaklaşık %20’sinde hastalık dirençli seyredebilir veya tekrar edebilir. Tekrar eden/ dirençli HL’da standart tedavi yüksek doz kemoterapi ve takip eden otolog kök hücre naklidir (OKHN). Otolog KHN sonrası tekrar eden hastalarda ise allojeneik kök hücre nakli (AKHN) önemli bir kurtarma tedavisi olarak görülmektedir. Amaç: Medipol Üniversitesi Tıp Fakültesi çocuk kemik iliği nakil ünitesinde OKHN ve AKHN yapılan hastalarda sonuçları değerlendirmek. Yöntem: Tekrar eden/dirençli HL nedeniyle 2014 Kasım ile Temmuz 2019 tarihleri arasında merkezimizde OKHN yapılan 18 olgu retrospektif olarak değerlendirilmiştir. Otolog KHN sonrası hastalığı tekrar eden ve AKHN yapılan hastalarda ayrıca değerlendirilmiştir. Bulgular: Onaltı hasta halen hayattadır. Onbir hastada OKHN sonrası has talık tekrar etmiştir. Relaps eden hastalardan 10’una AKHN yapılmıştır. Bu hastalardan üçünde tekrar görülmüş olup, sekizi nakil sonrası hayattadır lar.Background: Nearly 20% of patients with Hodgkin Lymphoma (HL) who receive standard treatment will relapse or have a refractory disease. Standard treatment for the Relapsed/Refractory (RR) HL is salvage high dose chemotherapy followed by autologous stem cell transplantation (AuSCT). Management of RR HL after AuSCT with allogeneic stem cell transplantation (ASCT) is also considered as an important salvage therapy. Objective: To describe the outcome in pediatric patients with RR HL who underwent AuHSCT and ASCT in Medipol University hematopoietic stem cell transplantation center. Method: We retrospectively evaluated 18 pediatric patients with RR HL who underwent AHSCT between November 2014 and July 2019. The evaluation of ASCT after RR HL AuSCT is also done. Results: Sixteen patients are still alive. Eleven of them relapsed after AuHSCT. AllogeneicHSCT was performed on 10 patients who relapsed. Relapse was seen in three patients after AHSCT. Eight of them are still alive

A 5.9mW/Gb/s 7Gb/s/pin 8-Lane Single-Ended RX with Crosstalk Cancellation Scheme using a XCTLE and 56-tap XDFE in 32nm SOI CMOS

This work reports an 8-lane single-ended RX featuring compact and low power far-end crosstalk (FEXT) cancellation circuits. The RX data-path consists of a cross continuous-time linear equalizer (XCTLE) to remove FEXT by nearest aggressors within the channel bundle. Residual post-cursor FEXT is suppressed by a direct feedback 7x8-tap cross decision feedback equalizer (XDFE). A CTLE and 8-tap DFE equalize single-ended channels with 28dB insertion loss at Nyquist frequency without TX FFE. The circuit, fabricated in 32nm SOI CMOS, was measured to receive 7Gb/s/pin PRBS11 data at BER< 10^-12 with 12.5%UI margin. It occupies 300x350um2 with an energy efficiency of 5.9mW/Gb/s

Treatment and Outcome Analysis of 639 Relapsed Non-Hodgkin Lymphomas in Children and Adolescents and Resulting Treatment Recommendations.

Despite poor survival, controversies remain in the treatment for refractory or relapsed pediatric non-Hodgkin lymphoma (r/r NHL). The current project aimed to collect international experience on the re-induction treatment of r/r NHL, hematopoietic stem cell transplantation (HSCT), risk factors associated with outcome, and to suggest treatment recommendations. Inclusion criteria were (i) refractory disease, disease progression or relapse of any NHL subtype except anaplastic large cell lymphoma, (ii) age < 18 years at initial diagnosis, (iii) diagnosis in/after January 2000. Data from 639 eligible patients were evaluable. The eight-year probability of overall survival was 34 ± 2% with highly significant differences according to NHL subtypes: 28 ± 3% for 254 Burkitt lymphoma/leukemia, 50 ± 6% for 98 diffuse large B-cell lymphomas, 57 ± 8% for 41 primary mediastinal large B-cell lymphomas, 27 ± 3% for 177 T-lymphoblastic lymphomas, 52 ± 10% for 34 precursor-B-cell lymphoblastic lymphomas and 30 ± 9% for 35 patients with rare NHL subtypes. Subtype-specific factors associated with survival and treatment recommendations are suggested. There were no survivors without HSCT, except in few very small subgroups. Conclusions: There is an urgent need to further improve survival in r/r NHL. The current study provides the largest real-world series, which underlines the role of HSCT and suggests treatment recommendations

Green Polymers and Applications

Polimerler; istenilen özellikte sentezlenebilme, kolay işlenebilme, korozyona uğramama, hafiflik ve düşük maliyete sahip olma gibi özelliklere sahip gelişmiş materyallerdir. Sahip oldukları bu özellikler sayesinde polimerler, endüstride birçok kullanım alanı bulmuştur ve bu alanlar gün geçtikçe artmaktadır. Endüstriyel polimerler göstermiş oldukları yüksek potansiyel ve özelliklerin aksine, doğal bir süreç ile yok edilememesi sonucu kirlilik oluşturmakta ve ancak maliyetli parçalanma işlemleri sonucunda bertaraf edilebilmektedir. Bununla birlikte, petrol kaynakları gibi tükenir kaynaklardan elde edilmeleri, polimer sektöründe süreklilik ve çevre uyumu açısından yeni arayışlara yol açmıştır. Biyopolimerler, endüstriyel polimerlerin tükenir kaynaklardan elde edilmeleri ve bertarafının zor olması gibi dezavantajları sonucu, başta ambalaj sektörü olmak üzere birçok alanda alternatif materyal olarak ön plana çıkmıştır. Doğal ve sürdürülebilir kaynaklardan elde edilen biyopolimerler, mekanik ve termal özellikleri sayesinde endüstriyel polimerlerin taşıdığı özelliklere eşdeğer özelliklerde olup; biyopolimerlerin ilerleyen yıllarda polimer endüstrisinde önemli bir yere sahip olacağı öngörülmektedir. Bu derleme çalışmasında biyopolimerin çeşitleri hakkında bilgiler verilmiş ve sektörlere göre kullanım alanları incelenmiştir.Polymers are highly sophisticated materials having properties such as being able to be synthesized, easily processed, not to be corroded, light weight and low cost. Polymers are used in many areas of the industry through these properties and these areas are increasing day by day. Contrary to the high potentials and properties of industrial polymers, they lead to pollution due to the fact that they cannot be destroyed by a natural process, and they can only be disposed of as a result of costly fragmentation processes. In addition, their availability from exhaustive sources such as petroleum has led to new quests for continuity and environmental compatibility in the polymer sector. Since industrial polymers have these disadvantages (availability from exhaustive sources and difficult disposal), biopolymers have come to the forefront as alternative materials in many fields, especially in the packaging sector. Biopolymers, which are obtained from natural and sustainable sources, are equivalent to those possessed by industrial polymers through their mechanical and thermal properties. Furthermore, it is predicted that the biopolymers will have an important place in the polymer industry in the following years. In this review study, information about the types of biopolymers has been given and their application areas have been examined according to the sectors

Cross-sectional analysis of health-related quality of life in survivors of childhood cancer: A study from Turkey

WOS: 000343932101134

High ferritin levels in fever of unknown origin: Possible first sign of hemophagocytosis in bmt patients?

...European Hematology Associatio

Outcomes of high-grade gastrointestinal graft-versus-host disease posthematopoietic stem cell transplantation in children

WOS: 000388566200044PubMed ID: 27858879We explored the clinical course of acute high-grade gastrointestinal graft-versus-host disease in children in a single center. This was a retrospective analysis of 28 pediatric patients who presented with a clinical diagnosis of stage III and IV acute graft-versus-host disease (aGVHD) of the gastrointestinal system (GIS). Generally, skin involvement was the initial manifestation of aGVHD that began in the first 3 weeks of hematopoietic stem cell transplantation (HSCT); on the other hand, GIS involvement predominated after the second week of HSCT. Reported adult data show a survival rate of only 25%; however, our study showed more favorable outcomes in children with a survival rate of 55%. We monitored levels of albumin and immunoglobulin G and observed low levels overall during treatment of unresponsive patients, although only albumin levels were shown to be significantly different. We observed a significant increase in mortality with the use of antithymocyte globulin in GIS aGVHD, although antithymocyte globulin used for graft-versus-host disease prophylaxis had no demonstrable effect on GIS aGVHD mortality. Whether the significantly lower GIS aGVHD mortality among the children recruited in our study than among their historical adult counterparts is a primary result of the specific attributes of the pediatric GIS, or whether it originated from HSCT kinetics remains to be determined by future studies