Gilteritinib (XOSPATA (R)) in Turkey: Early access program results

Background And Objectives: Gilteritinib (XOSPATA (R), Astellas) is a type I oral FLT3 inhibitor, a tyrosine kinase AXL inhibitor, involved in both c-Kit and FMS-like tyrosine kinase 3 (FLT3) resistance. In the phase 3 ADMIRAL trial, gilteritinib was compared with the standard of care in (R/R) acute myeloid leukemia (AML) patients who harbored any FLT3 mutation and showed superior efficacy with regard to response and survival. Objectives: This research aimed to investigate the real-life efficacy and safety of gilteritinib in FLT3-positive R/R AML patients who were treated as a part of an early access program held in Turkey in April 2020 (NCT03409081). Results: The research included 17 R/R AML patients who had received gilteritinib from seven centers. The overall response rate was 100%. The most common adverse events were anemia and hypokalemia (7 patients, 41.2%). Grade 4 thrombocytopenia was observed in one patient only (5.9%), leading to permanent treatment discontinuation. Patients with peripheral edema had a 10.47 (95% CI: 1.64-66.82) times higher risk of death than those without peripheral edema (p<0.05). Conclusion: This research showed that patients with febrile neutropenia and peripheral edema were at a high risk of death when compared to patients without febrile neutropenia and peripheral edema

(Trans)cribing History: The Oral Histories of Transgender Clergy in America

Popular American rhetoric around religion and LGBTQ issues puts the two at odds. The Bible has been the center of many debates over sexuality, gender identity, and faith. However, very few conversations have included or revolved around the authentic experiences of LGBTQ people of faith. Nine participants from various Protestant backgrounds shared their narratives with me and spoke on issues of gender and faith. This report is an in-depth summary of a short oral history project of transgender clergy (as well as candidates for ordination or exemplary church members)

Refrakter myelom seyrinde asit oluşumu ve intraperitoneal deksametazon tedavisine yanıt

Multiple myeloma (MM) is a plasma cell dyscrasia characterized by the malignant proliferation of a plasma cell clone. Ascites is a rare complication of MM and generally occurs because of portal hypertension due to the infiltration of liver by plasma cells. Infrequently, ascites is detected as a result of peritoneal infiltration by plasma cells. Ascites in the course of myeloma has a progressive course and is refractory to high dose chemotherapy. A refractory myeloma case with massive ascites due to peritoneal infiltration and responding to intra-peritoneal dexametasone treatment is presented.Multipl Myelom (MM) plazma hücre klonunun malign proliferasyonu ile karakterize bir plazma hücre diskrazisidir. Asit oluşumu MM'un nadir bir komplikasyonudur ve genellikle karaciğerin plazma hücreleri ile infiltrasyonu sonucu meydana gelen portal hipertansiyona bağlıdır. Asit nadiren peritonun plazma hücre infiltrasyonu sonucu da görülebilir. Myelom seyrinde asit oluşumu progresif seyreder ve yüksek doz kemoterapiye refrakterdir. Peritoneal infiltrasyona bağlı masif asit ile başvuran ve intraperitoneal deksametazon tedavisine yanıt veren bir refrakter myelom olgusu sunulmaktadı

Therapeutic Plasma Exchange for Neurologic Disorders: Single Center Experiences

Giriş: Terapötik plasma değişimi(TPD) kan elemanlarının ayrılması, plazmanın değişimi ve ardından özellikle kırmızı kan hücreleri olmak üzere diğer kan komponentlerinin hastaya geri verilmesine dayalı bir tedavi yöntemidir. İmmun patogenezin rol oynadığı nörolojik hastalıklarda TPD etkili bir tedavidir. Bu çalışmada farklı nörolojik hastalık gruplarında merkezimizde uygulanan TPD tedavilerine alınan yanıtların literatür bilgileri eşliğinde tartışılması amaçlanmıştır. Materiyal-Metod: 2010-2014 tarihleri arasında hastanemiz nöroloji yoğunbakım ünitesi ve aferez merkezinde TPD tedavisi uygulanan hastaların verileri retrospektif olarak değerlendirilmiştir. Sonuçlar: Hasta grubunu 17 Nöromyelitis Optika, 4 Guillain-Barre sendromu, 3 Myastenia Gravis, 2 Multipl Skleroz, 5 paraneoplastik ensefolapati, 1 akut dissemine ensefalomyelit, 1 akut hemorajik lökoensefalopati, 2 transvers myelit, 1 kronik inflamatuar demyelinizan polinöropati ve 1 Multifokal edinsel demiyelinizan sensori ve motor nöropati tanılı hasta oluşturmakta idi. Ortalama TPD seans sayısı 6 idi(1-25 aralığında). Toplam TPD seans sayısı 236 saptandı. Tartışma: İmmunpatogenezin rol oynadığı nörolojik hastalıklarda TPD tedavi seçenekleri arasında yer almaktadır. Guillain-Barre sendromu ve Myastenia Gravis hastalıklarında TPD birinci basamak tedavi seçeneklerinden biri olarak kabul edilmektedir. almaktadır. Nöromyelitis Optika hastalarında merkezimizde TPD uygulaması ile elde ettiğimiz yanıtlar ışığında TPD'nin bu hasta grubunda da birinci basamak tedavi seçenekleri arasında yer alabileceğini düşünmekteyiz.Introduction: Therapeutic plasma exchange (TPE), is a procedure that involves separating the blood, exchanging the plasma and returning the other components, primarily red blood cells, to the patient. TPE is an effective treatment in neurologic diseases with immune pathogenesis. We report our single center experience about TPE in patients with different kind of neurologic diseases. Material- Method: We reviewed the medical records of 37 patients who had been consecutively treated by TPE between 2010 and 2014 at Neurologic Intensive Care Unit and The Apheresis Center. Results: Neurologic indications included neuromyelitis optica (NMO) (17 patients), GuillainBarre syndrome (GBS) (4 patients), myastenia gravis (MG) (3 patients), multiple sclerosis (MS) (2 patients), paraneoplastic encephalopathy (5 patients), acute disseminated encephalomyelitis (ADEM) 1 patient, acute hemorrhagic leucoencephalopathy 1 patient, transverse myelitis (2 patients), chronic inflammatory demyelinating polyneuropathy (CIDP) (1) and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) 1 patient. The median TPE session number was 6 . Total number of TPE procedures in all cases was 236. Discussion: In neurological diseases with immune pathogenesis TPE must always be kept in mind as a treatment option. TPE is widely accepted as a first line treatment for GBS and MG. According to our experience NMO is the third disease in which TPE must be admitted as the first therapeutic option

Seasonal Association of Immune Thrombocytopenia in Adults

Saydam, Guray/0000-0001-8646-1673; GURKAN, EMEL/0000-0002-3060-4054WOS: 000362846400002PubMed: 26740892Background: Immune thrombocytopenia (ITP) is an autoimmune disorder. It is characterized by thrombocytopenia due to thrombocyte destruction mediated by auto-antibodies; however, cytotoxic and defective regulatory T-lymphocytes play an important role in its pathogenesis. While childhood ITP is usually acute, self-limiting and generally seasonal in nature, ITP in adults is usually chronic; its relation with seasons has not been studied. Aims: We investigated whether months and/or seasons have triggering roles in adults with ITP. Study Design: Descriptive study. Methods: A retrospective case review of adult patients with primary ITP diagnosed at various University Hospitals in cities where Mediterranean climate is seen was performed. Demographic data, date of referral and treatments were recorded. Corticosteroid-resistant, chronic and refractory cases were determined. Relation between sex, corticosteroid-resistant, chronic and refractory ITP with the seasons was also investigated. Results: The study included 165 patients (124 female, mean age=42.8 +/- 16.6). Most cases of primary ITP were diagnosed in the spring (p=0.015). Rates of patients diagnosed according to the seasons were as follows: 35.8% in spring, 23% in summer, 20.6% in fall, and 20.6% in winter. With respect to months, the majority of cases occurred in May (18.2%). Time of diagnosis according to the seasons did not differ between genders (p=0.699). First-line treatment was corticosteroids in 97.3%, but 35% of the cases were corticosteroid-resistant. Steroid-resistant patients were mostly diagnosed in the spring (52.1%) (p=0.001). ITP was chronic in 52.7% of the patients and they were also diagnosed mostly in the spring (62.7%) (p=0.149). Conclusion: This is the first study showing seasonal association of ITP in adults and we have observed that ITP in adults is mostly diagnosed in the spring. The reason why more patients are diagnosed in the spring may be due to the existence of atmospheric pollens reaching maximum levels in the spring in places where a Mediterranean climate is seen

Kronik Miyeloproliferatif Neoplazi Tanılı Türk Hastalarda TET2, ASXL1, IDH1 ve IDH2 Tek Nükleotid Polimorfizmleri

We aimed to determine the genotype distribution, allele frequency, and prognostic impact of IDH1/2, TET2, and ASXL1 single nucleotide polymorphisms (SNPs) in myeloproliferative neoplasms (MPNs). TET2 (rs763480), ASXL1 (rs2208131), and IDH1 (rs11554137) variant homozygous genotype frequencies were found at rates of 1.5%, 9.2%, and 2.3%, respectively. No IDH2 SNP was identified. IDH1 and TET2 frequencies were 5% in essential thrombocythemia (ET) and 1.7% in ET and 5% in primary myelofibrosis (PMF), respectively. ASXL1 frequencies were 8.3%-10% in MPN subgroups. The TET2 mutant allele T and ASXL1 mutant allele G had the highest frequencies with 0.272 in the PMF and 0.322 in the polycythemia vera (PV) group, respectively. There was no impact of the SNPs on prognosis. IDH1 frequency in MPNs was found similar to the literature. ASXL1 frequencies were similar between ET, PV, and PMF patients. The ASXL1 and TET2 allele frequencies of the Turkish population are similar to those of the European population. The role of SNPs in MPNs might be further evaluated in larger multicenter studies.Bu çalışmada biz ASXL1, TET2, IDH1/2 genlerindeki tek nükleotid polimorifizmlerin (SNP) alel sıklığını, genotipik dağılımını ve prognostik etkisini saptamayı amaçladık. TET2 (rs763480), ASXL1 (rs2208131) ve IDH1 (rs11554137) varyant homozigot genotip sıklığı sırasıyla %1,5, %9,2 ve %2,3 saptandı. IDH2 SNP saptanmadı. IDH1 sıklığı ET'de %5 ve TET2 sıklığı ET'de %1,7 ve PMF'te %5 idi. ASXL1 sıklığı ise MPN alt gruplarında %8,3-10'du. En yüksek TET2 mutant allel T ve ASXL mutant allel G sıklığı sırasıyla PMF'te 0,272 ve PV'de 0,322 olarak saptandı. SNP'lerin prognoz üzerine etkisi yoktu. MPN'de IDH1 sıklığı literatür ile uyumlu bulundu. ASXL1 sıklığı PV, PMF ve ET alt gruplarında benzerdi. Türklerde ASXL1 ve TET2 allel sıklığı Avrupalılar ile benzer saptandı. MPN'lerde SNP'lerin rolü, büyük ve çok merkezli çalışmalarda değerlendirilmelidir

Seasonal Association of Immune Thrombocytopenia in Adults

Background: Immune thrombocytopenia (ITP) is an au-toimmune disorder. It is characterized by thrombocyto-penia due to thrombocyte destruction mediated by auto-antibodies; however, cytotoxic and defective regulatory T-lymphocytes play an important role in its pathogen-esis. While childhood ITP is usually acute, self-limiting and generally seasonal in nature, ITP in adults is usually chronic; its relation with seasons has not been studied.Aims: We investigated whether months and/or seasons have triggering roles in adults with ITP.Study Design: Descriptive study.Methods: A retrospective case review of adult patients with primary ITP diagnosed at various University Hos-pitals in cities where Mediterranean climate is seen was performed. Demographic data, date of referral and treatments were recorded. Corticosteroid-resistant, chronic and refractory cases were determined. Rela-tion between sex, corticosteroid-resistant, chronic and refractory ITP with the seasons was also investigated.Results: The study included 165 patients (124 female, mean age=42.8&plusmn;16.6). Most cases of primary ITP were diagnosed in the spring (p=0.015). Rates of patients diagnosed according to the seasons were as follows: 35.8% in spring, 23% in summer, 20.6% in fall, and 20.6% in winter. With respect to months, the majority of cases occurred in May (18.2%). Time of diagnosis according to the seasons did not differ between genders (p=0.699). First-line treatment was corticosteroids in 97.3%, but 35% of the cases were corticosteroid-resis-tant. Steroid-resistant patients were mostly diagnosed in the spring (52.1%) (p=0.001). ITP was chronic in 52.7% of the patients and they were also diagnosed mostly in the spring (62.7%) (p=0.149).Conclusion: This is the first study showing seasonal as-sociation of ITP in adults and we have observed that ITP in adults is mostly diagnosed in the spring. The reason why more patients are diagnosed in the spring may be due to the existence of atmospheric pollens reaching maximum levels in the spring in places where a Medi-terranean climate is seenBackground: Immune thrombocytopenia (ITP) is an au-toimmune disorder. It is characterized by thrombocyto-penia due to thrombocyte destruction mediated by auto-antibodies; however, cytotoxic and defective regulatory T-lymphocytes play an important role in its pathogen-esis. While childhood ITP is usually acute, self-limiting and generally seasonal in nature, ITP in adults is usually chronic; its relation with seasons has not been studied.Aims: We investigated whether months and/or seasons have triggering roles in adults with ITP.Study Design: Descriptive study.Methods: A retrospective case review of adult patients with primary ITP diagnosed at various University Hos-pitals in cities where Mediterranean climate is seen was performed. Demographic data, date of referral and treatments were recorded. Corticosteroid-resistant, chronic and refractory cases were determined. Rela-tion between sex, corticosteroid-resistant, chronic and refractory ITP with the seasons was also investigated.Results: The study included 165 patients (124 female, mean age=42.8&plusmn;16.6). Most cases of primary ITP were diagnosed in the spring (p=0.015). Rates of patients diagnosed according to the seasons were as follows: 35.8% in spring, 23% in summer, 20.6% in fall, and 20.6% in winter. With respect to months, the majority of cases occurred in May (18.2%). Time of diagnosis according to the seasons did not differ between genders (p=0.699). First-line treatment was corticosteroids in 97.3%, but 35% of the cases were corticosteroid-resis-tant. Steroid-resistant patients were mostly diagnosed in the spring (52.1%) (p=0.001). ITP was chronic in 52.7% of the patients and they were also diagnosed mostly in the spring (62.7%) (p=0.149).Conclusion: This is the first study showing seasonal as-sociation of ITP in adults and we have observed that ITP in adults is mostly diagnosed in the spring. The reason why more patients are diagnosed in the spring may be due to the existence of atmospheric pollens reaching maximum levels in the spring in places where a Medi-terranean climate is see

Seasonal Association of Immune Thrombocytopenia in Adults

Background: Immune thrombocytopenia (ITP) is an au-toimmune disorder. It is characterized by thrombocyto-penia due to thrombocyte destruction mediated by auto-antibodies; however, cytotoxic and defective regulatory T-lymphocytes play an important role in its pathogen-esis. While childhood ITP is usually acute, self-limiting and generally seasonal in nature, ITP in adults is usually chronic; its relation with seasons has not been studied.Aims: We investigated whether months and/or seasons have triggering roles in adults with ITP.Study Design: Descriptive study.Methods: A retrospective case review of adult patients with primary ITP diagnosed at various University Hos-pitals in cities where Mediterranean climate is seen was performed. Demographic data, date of referral and treatments were recorded. Corticosteroid-resistant, chronic and refractory cases were determined. Rela-tion between sex, corticosteroid-resistant, chronic and refractory ITP with the seasons was also investigated.Results: The study included 165 patients (124 female, mean age=42.8±16.6). Most cases of primary ITP were diagnosed in the spring (p=0.015). Rates of patients diagnosed according to the seasons were as follows: 35.8% in spring, 23% in summer, 20.6% in fall, and 20.6% in winter. With respect to months, the majority of cases occurred in May (18.2%). Time of diagnosis according to the seasons did not differ between genders (p=0.699). First-line treatment was corticosteroids in 97.3%, but 35% of the cases were corticosteroid-resis-tant. Steroid-resistant patients were mostly diagnosed in the spring (52.1%) (p=0.001). ITP was chronic in 52.7% of the patients and they were also diagnosed mostly in the spring (62.7%) (p=0.149).Conclusion: This is the first study showing seasonal as-sociation of ITP in adults and we have observed that ITP in adults is mostly diagnosed in the spring. The reason why more patients are diagnosed in the spring may be due to the existence of atmospheric pollens reaching maximum levels in the spring in places where a Medi-terranean climate is seenBackground: Immune thrombocytopenia (ITP) is an au-toimmune disorder. It is characterized by thrombocyto-penia due to thrombocyte destruction mediated by auto-antibodies; however, cytotoxic and defective regulatory T-lymphocytes play an important role in its pathogen-esis. While childhood ITP is usually acute, self-limiting and generally seasonal in nature, ITP in adults is usually chronic; its relation with seasons has not been studied.Aims: We investigated whether months and/or seasons have triggering roles in adults with ITP.Study Design: Descriptive study.Methods: A retrospective case review of adult patients with primary ITP diagnosed at various University Hos-pitals in cities where Mediterranean climate is seen was performed. Demographic data, date of referral and treatments were recorded. Corticosteroid-resistant, chronic and refractory cases were determined. Rela-tion between sex, corticosteroid-resistant, chronic and refractory ITP with the seasons was also investigated.Results: The study included 165 patients (124 female, mean age=42.8±16.6). Most cases of primary ITP were diagnosed in the spring (p=0.015). Rates of patients diagnosed according to the seasons were as follows: 35.8% in spring, 23% in summer, 20.6% in fall, and 20.6% in winter. With respect to months, the majority of cases occurred in May (18.2%). Time of diagnosis according to the seasons did not differ between genders (p=0.699). First-line treatment was corticosteroids in 97.3%, but 35% of the cases were corticosteroid-resis-tant. Steroid-resistant patients were mostly diagnosed in the spring (52.1%) (p=0.001). ITP was chronic in 52.7% of the patients and they were also diagnosed mostly in the spring (62.7%) (p=0.149).Conclusion: This is the first study showing seasonal as-sociation of ITP in adults and we have observed that ITP in adults is mostly diagnosed in the spring. The reason why more patients are diagnosed in the spring may be due to the existence of atmospheric pollens reaching maximum levels in the spring in places where a Medi-terranean climate is see