Primary Synovial Sarcoma of Kidney: A Rare Differential Diagnosis of Renomegaly

Synovial sarcomas (SS) are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC) due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria

Primary Vaginal Myeloid Sarcoma: A Rare Case Report and Review of the Literature

Myeloid sarcoma (chloroma, granulocytic sarcoma, or extramedullary myeloid tumour) is an extramedullary mass forming neoplasm composed of myeloid precursor cells. It is usually associated with myeloproliferative disorders but very rarely may precede the onset of leukemia. Here, we are presenting a rare case of primary vaginal myeloid sarcoma in a geriatric female patient without initial presentation of acute myeloid leukemia (AML). A 68-year-old female patient with ECOG Performance Score of 1 presented with pervaginal bleeding for 20 days. On colposcopic examination, she was found to have mass in the anterior fornix of vagina. A punch biopsy specimen revealed chloromatous infiltration of the vagina. LCA (leukocyte common antigen), MPO (myeloperoxidase), and c-kit were strongly positive on IHC (immunohistochemistry). The patient’s routine blood investigations were normal including peripheral smear, lactose dehydrogenase, uric acid, 2D echocardiography, conventional cytogenetics, bone marrow aspiration, and biopsy. The patient was given 4 cycles of decitabine (Decitex, manufactured by Sun Pharmaceutical Industries Limited, India), 20 mg/m2 for 5 days at an interval of 28 days. There was a partial response to decitabine according to RECIST criteria. As decitabine therapy was well tolerated, we are continuing in the same way until disease progression without any complications. The patient is undergoing regular follow-up at our centre

Successful Allogeneic Hematopoietic Stem Cell Transplantation of a Patient Suffering from Type II Congenital Dyserythropoietic Anemia A Rare Case Report from Western India

The most frequent form of congenital dyserythropoiesis (CDA) is congenital dyserythropoietic anemia II (CDA II). CDA II is a rare genetic anemia in humans, inherited in an autosomally recessive mode, characterized by hepatosplenomegaly normocytic anemia and hemolytic jaundice. Patients are usually transfusion-independent except in severe type. We are here reporting a case of severe transfusion-dependent type II congenital dyserythropoietic anemia in a 5-year-old patient who has undergone allogeneic hematopoietic stem cell transplantation (HSCT) at our bone marrow transplantation centre. Patient has had up until now more than 14 mL/kg/month of packed cell volume (PCV), which he required every 15 to 20 days to maintain his hemoglobin of 10 gm/dL and hematocrit of 30%. His pre-HSCT serum ferritin was 1500 ng/mL and he was on iron chelating therapy. Donor was HLA identical sibling (younger brother). The preparative regimen used was busulfan, cyclophosphamide, and antithymocyte globulin (Thymoglobulin). Cyclosporine and short-term methotrexate were used for graft versus host disease (GVHD) prophylaxis. Engraftment of donor cells was quick and the posttransplant course was uneventful. The patient is presently alive and doing well and he has been transfusion-independent for the past 33 months after HSCT

Obeticholic acid for the treatment of non-alcoholic steatohepatitis: interim analysis from a multicentre, randomised, placebo-controlled phase 3 trial

Background Non-alcoholic steatohepatitis (NASH) is a common type of chronic liver disease that can lead to cirrhosis. Obeticholic acid, a farnesoid X receptor agonist, has been shown to improve the histological features of NASH. Here we report results from a planned interim analysis of an ongoing, phase 3 study of obeticholic acid for NASH. Methods In this multicentre, randomised, double-blind, placebo-controlled study, adult patients with definite NASH,non-alcoholic fatty liver disease (NAFLD) activity score of at least 4, and fibrosis stages F2–F3, or F1 with at least oneaccompanying comorbidity, were randomly assigned using an interactive web response system in a 1:1:1 ratio to receive oral placebo, obeticholic acid 10 mg, or obeticholic acid 25 mg daily. Patients were excluded if cirrhosis, other chronic liver disease, elevated alcohol consumption, or confounding conditions were present. The primary endpointsfor the month-18 interim analysis were fibrosis improvement (≥1 stage) with no worsening of NASH, or NASH resolution with no worsening of fibrosis, with the study considered successful if either primary endpoint was met. Primary analyses were done by intention to treat, in patients with fibrosis stage F2–F3 who received at least one dose of treatment and reached, or would have reached, the month 18 visit by the prespecified interim analysis cutoff date. The study also evaluated other histological and biochemical markers of NASH and fibrosis, and safety. This study is ongoing, and registered with ClinicalTrials.gov, NCT02548351, and EudraCT, 20150-025601-6. Findings Between Dec 9, 2015, and Oct 26, 2018, 1968 patients with stage F1–F3 fibrosis were enrolled and received at least one dose of study treatment; 931 patients with stage F2–F3 fibrosis were included in the primary analysis (311 in the placebo group, 312 in the obeticholic acid 10 mg group, and 308 in the obeticholic acid 25 mg group). The fibrosis improvement endpoint was achieved by 37 (12%) patients in the placebo group, 55 (18%) in the obeticholic acid 10 mg group (p=0·045), and 71 (23%) in the obeticholic acid 25 mg group (p=0·0002). The NASH resolution endpoint was not met (25 [8%] patients in the placebo group, 35 [11%] in the obeticholic acid 10 mg group [p=0·18], and 36 [12%] in the obeticholic acid 25 mg group [p=0·13]). In the safety population (1968 patients with fibrosis stages F1–F3), the most common adverse event was pruritus (123 [19%] in the placebo group, 183 [28%] in the obeticholic acid 10 mg group, and 336 [51%] in the obeticholic acid 25 mg group); incidence was generally mild to moderate in severity. The overall safety profile was similar to that in previous studies, and incidence of serious adverse events was similar across treatment groups (75 [11%] patients in the placebo group, 72 [11%] in the obeticholic acid 10 mg group, and 93 [14%] in the obeticholic acid 25 mg group). Interpretation Obeticholic acid 25 mg significantly improved fibrosis and key components of NASH disease activity among patients with NASH. The results from this planned interim analysis show clinically significant histological improvement that is reasonably likely to predict clinical benefit. This study is ongoing to assess clinical outcomes

Safety and Efficacy of Ledipasvir plus Sofosbuvir with or without ribavirin in hepatitis C genotype 1 patients who failed previous treatment with Simeprevir plus Sofosbuvir

Combination therapy with Simeprevir (SIM), NS3/4 protease inhibitor, with Sofosbuvir (SOF), NS5b polymerase inhibitor is an FDA approved treatment option for chronic hepatitis C genotype 1 patients with an over all SVR 12 rate of 85-95%. Single tablet fixed dose combination of Ledipasvir (LDV), NS5a inhibitor, with SOF is also FDA approved for treatment of hepatitis C genotype 1 with SVR 12 rates of ≥ 95%. However, there is no data on the efficacy of retreatment with LDV+SOF in patients who failed initial treatment with SIM+SOF. Methods: Data was collected from treatment cohorts at 2 large hepatology referral centers in Dallas-Fort Worth area. Patients included in the analysis were previously treated with SIM+SOF with or without RBV for 12 weeks but failed to achieve SVR 12 and then undergone re-treatment with LDV+SOF with or without RBV for 12-24 weeks. Patients with cirrhosis, including decompensated Child’s class B or C were included. Decompensation was defined by the presence of fluid overload, hepatic encephalopathy or variceal bleeding. Patients with HCC as the only event that defined decompensation were excluded.. Patients received singlet tablet fixed-dose combination of Ledipasvir 90 mg with Sofosbuvir 400 mg PO +/- wt based ribavirin (RBV) daily for 12-24 weeks at the discretion of the treating hepatologist. Baseline and end of treatment (EOT) laboratory tests & viral load were obtained on all patients. SVR 12 defined as undetectable viral load 12 weeks after EOT was collected on all patients who had reached that time point by Nov 10, 2015. Adverse effects during treatment were obtained on all patients. Data was analyzed using 2 sided t test for continuous variables and chi-quare test for categorical variables. Results: SVR 12 was achieved for 11/13 of all patients and 10/11 for patients who were cirrhotic. 100% (29/29) had achieved EOT response. 10/29 had no side effects on treatments. Of those who had side effects, none were considered severe enough to warrant discontinuation. Conclusions: Ledipasvir + Sofosbuvir is a viable treatment option with high SVR 12 rate in patients who have failed 12 weeks of treatment with SIM + SOF. High SVR 12 rates of 100% (4/4) & 86% (6/7) were achieved in patients with compensated and decompensated cirrhosis respectively •Treatment was generally well tolerated requiring no discontinuations including in those with cirrhosi

Assessment and Management of Eyelid Injury

Background and aim: Among all sites of injury, eyelid laceration seems to be neglected in terms of sufficient epidemiological investigations. With a thorough understanding of the causes of eye lid lacerations, it is possible to develop a better preventive strategy and hence improve the public health policy in this respect. Hence the aim of the study was to understand the type of eyelid injury and study management of the injuries.&#x0D; Materials and methods: A total of 100 cases that were reported to the hospital department opd with the chief complain of blunt as well as penetrating eye lid injuries were included in the study. Evaluation by a physician was done for all cases to note the presence of any systemic diseases and for opinion regarding fitness for surgery. In cases where General Anaesthesia (GA) was used, Anaesthetist examined the patient and opined regarding status of the patient towards                           GA.&#x0D; Results: The results of the present study showed that 3rd and 4th decade were more prone to the eyelid injuries. Majority of the 58 cases showed involvement of left eye whereas the involvement of right eye was seen in 42 cases. In the present study, Minimonoka stent was used for 8 cases of canalicular lacerations and 8 cases of canalicular tear that were not affordable were repaired with 24G Venflon tube.&#x0D; Conclusion: As the injuries occur more commonly due to road traffic accidents showing 54 cases in our study, preventive measures are to be taken while riding such as controlling speed. Domestic injuries are more common in females. This study showed that Minimonoka stent is an effective and easy tool in reconstructing canalicular tear, with successful anatomical and functional integrity.</jats:p