ABSTRACT
The fungal pathogen Scedosporium apiospermum is a ubiquitous opportunistic pathogen found in soil and water that can cause severe infection in hosts with impaired immunity. Patients with systemic autoimmune diseases such as systemic lupus erythematosus (SLE) are already at risk for infections given their altered immunity. This can be exacerbated further in patients taking immune-suppressing medications to control their disease status. Here, we present a case of a rare and challenging clinical scenario of a woman with refractory multi-organ SLE on steroids who developed neurologic deficits found to have a brain abscess caused by a unique fungal etiology.
ABSTRACT
Few data exist regarding the disease and clinical characteristics of patients with essential thrombocythemia (ET) in the United States. The ongoing, multicenter, noninterventional, prospective, Myelofibrosis and Essential Thrombocythemia Observational STudy (MOST) was designed to collect data pertaining to the demographics, clinical management, and patient-reported outcomes in patients with myelofibrosis or ET in the United States (NCT02953704). This analysis examines the clinical characteristics of patients with clinical diagnoses of high-risk or low-risk ET receiving ET-directed therapy at enrollment. At data cutoff (June 17, 2019), 1207 of 1234 enrolled patients were eligible for this analysis (median age, 70 years; 65% female; 88% white); 917 patients (76%) had mutation testing results available. The median time from ET diagnosis to study enrollment was 4.2 years. The majority of patients (87%) had high-risk ET. Of 333 patients with a history of thrombotic events, 247 had at least 1 event classified as arterial and/or venous. Platelet count was above normal range in 54% of patients. Hypertension (56%) was the most common comorbidity. At enrollment, the majority of patients (low-risk ET, 94%; high-risk ET, 79%) were receiving ET-directed monotherapy. Additional prospective analyses from MOST will help to identify areas of unmet need.
