Safety and Efficacy of Ankaferd Hemostat (Abs) in the Chemotherapy-Induced Oral Mucositis

Oral mucositis, characterized by ulcerative lesions in the oral mucosa of patients undergoing chemotherapy, is currently considered to be the most severe complication of anti-cancer therapy which is affecting 40-80% of those patients. Ankaferd hemostat, (ABS) is the first topical hemostatic agent regarding the red blood cell (RBC)-fibrinogen interactions tested in the clinical trials. ABS also has pleiotropic effects particularly in the tissue healing and has anti-infective properties. The aim of this study is to assess the safety and efficacy of ABS in the management of chemotherapy-induced oral mucositis in adult patients with hematological malignancies. ABS was topically applied to the patients with grade 3-4 mucositis according to the WHO classification. Patients were said to mouthwash and swallow the five milliliters of ABS. Twenty patients with oral mucositis were evaluated. Eleven patients with acute myeloid leukemia, four acute lymphoblastic leukemia, three non-hodgkin lymphoma, one hemophagocytosis with acute myleoid leukemia and one hemophagocytosis were included in study. Median extract amount was calculated as 74.50 ml (30-100 ml) and median healing time was 6.6 days (3-10). Consequently, ABS is an effective agent in the treatment of chemotherapy related severe oral mucositis in patients with hematological malignancies. Further experimental and clinical trials about ABS shall focus on the interrelationships between proteomic content, fibrinogen gamma, and vital erythroid aggregation due to ABS.WoSScopu

The Impact Of Jak1/Jak2 Inhibitor Ruxolitinib On The Spleen Size And Symptom Burden In Myeloproliferative Diseases

Ruxolitinib as a JAK1 and JAK2 inhibitor drug has recently been approved for the treatment of patients with high-or intermediate-risk myelofibrosis with symptomatic splenomegaly. Clinical development of ruxolitinib has currently focused on the Ph* negative myeloproliferative neoplastic disorders (MPN). The aim of this study is to assess the impact of ruxolitinib treatment on the clinical course of Ph* negative myeloproliferative disorders. Forty-three patients who were under ruxolitinib treatment and followed-up between years 1987-2015 in Hacettepe University Medical School Hematology Clinic and Ondokuz Mayis University Hematology Clinic with myeloproliferative disease without Philadephia chromosome translocation were retrospectively analyzed. The constitutional symptoms were decreased in 97% of patients after ruxolitinib treatment. The mean spleen sizes before and after ruxolitinib treatment were 229 +/- 35 versus 202 +/- 31 mm, respectively (p<0.001). In this study, we observed a reduction in spleen size after ruxolitinib treatment in Turkish patients with MPN and this reduction was statistically significant. Moreover, nearly all of the MPN patients' constitutional symptoms were improved. Those observations are concordant with other geographical MPN data obtained from different countries. Further experimental and clinical studies into the efficacy and safety of ruxolitinib in patients with MPN are necessary to elucidate its role in special subgroups of MPN patients, such as patients undergoing hematopoietic stem cell transplantation and the patients with vascular disorders such as hepatoportal thrombosis.WoSScopu

De novo and dominantly inherited SPTAN1 mutations cause spastic paraplegia and cerebellar ataxia

ABSTRACT Background Pathogenic variants in SPTAN1 have been linked to a remarkably broad phenotypical spectrum. Clinical presentations include epileptic syndromes, intellectual disability, and hereditary motor neuropathy. Objectives We investigated the role of SPTAN1 variants in rare neurological disorders such as ataxia and spastic paraplegia. Methods We screened 10,000 NGS datasets across two international consortia and one local database, indicative of the level of international collaboration currently required to identify genes causative for rare disease. We performed in silico modeling of the identified SPTAN1 variants. Results We describe 22 patients from 14 families with five novel SPTAN1 variants. Of six patients with cerebellar ataxia, four carry a de novo SPTAN1 variant and two show a sporadic inheritance. In this group, one variant (p.Lys2083del) is recurrent in four patients. Two patients have novel de novo missense mutations (p.Arg1098Cys, p.Arg1624Cys) associated with cerebellar ataxia, in one patient accompanied by intellectual disability and epilepsy. We furthermore report a recurrent missense mutation (p.Arg19Trp) in 15 patients with spastic paraplegia from seven families with a dominant inheritance pattern in four and a de novo origin in one case. One further patient carrying a de novo missense mutation (p.Gln2205Pro) has a complex spastic ataxic phenotype. Through protein modeling we show that mutated amino acids are located at crucial interlinking positions, interconnecting the three‐helix bundle of a spectrin repeat. Conclusions We show that SPTAN1 is a relevant candidate gene for ataxia and spastic paraplegia. We suggest that for the mutations identified in this study, disruption of the interlinking of spectrin helices could be a key feature of the pathomechanism. © 2022 International Parkinson and Movement Disorder Societ