Introduction: Gilteritinib is a FLT3 kinase inhibitor approved for FLT3-mutated acute myeloid leukemia (AML). We present a case of febrile neutropenia and neutrophilic dermatosis consistent with Sweet's syndrome (SS). Case history: A 55-year-old woman presented with fever and skin lesions after 4 weeks of initiation of Gilteritinib for AML. She was febrile, pancytopenic and neutropenic with absolute neutrophil count (ANC) of 0.1x10E3/UI. Examination revealed reddish and violaceous rashes on her extremities. Pathology showed superficial dermal edema, widespread epidermal spongiosis and multiple neutrophils in the dermal infiltrate. Rash improved with prednisone 60 mg daily and started to flare with taper. She was still on Gilteritinib all this time. Gilteritinib was finally stopped due to non-response and possible contribution in flaring her SS. Shortly after, the patient succumbed to progressive disease and complications of sepsis. Discussion: There have been reports of SS in neutropenic patients although SS is typically a neutrophilic dermatosis. The pathogenesis of SS in neutropenia remains uncertain. Our study represents an additional medication-associated cutaneous complication of AML therapy. Clinicians need to be aware of potential neutrophilic dermatoses with FLT-3 inhibition, even with peripheral neutropenia.
BackgroundNon-alcoholic fatty liver disease (NAFLD), one of the leading causes of end-stage liver disease, is known to be associated with obesity. However, only a few studies in the United States (US) have described non-obese NAFLD, most of which were on the outpatient population. AimWe aimed to investigate the proportion of hospitalizations in the US with a diagnosis code that included NAFLD in the non-obese population. MethodsWe analyzed adult discharges from the Nationwide Inpatient Sample with a diagnosis of NAFLD from January 2010 to December 2014. We created two groups: obese (overweight or obese) and non-obese (normal or underweight) groups. Basic demographic and clinical characteristics were compared using the chi-square test and Student's t-test. ResultsA total of 194,787 hospitalizations with NAFLD were identified over the five-year period. It was observed that the prevalence of non-obese NAFLD hospitalizations increased yearly. Non-obese NAFLD hospitalizations had a higher mean age (57.5 vs 51.5 years, p < 0.0001) and a higher proportion of males (43.3% vs 36.1%, p < 0.0001) than obese NAFLD hospitalizations. With univariate analysis, non-obese NAFLD hospitalizations had lower odds of hypertension (OR 0.74, p < 0.0001), diabetes mellitus (OR 0.65, p < 0.0001). Non-obese hospitalizations had higher odds of cirrhosis (OR 1.30, p < 0.001) and decompensated cirrhosis (OR 1.30, p < 0.001) after adjusting for age, sex, race, diabetes mellitus, and dyslipidemia. Hospitalizations with non-obese NAFLD had higher odds of death (OR 1.49, p < 0.001) after adjusting for age, gender, race, co-morbidities, cirrhosis, and liver decompensation. ConclusionThere is a continued rise in the proportion of non-obese NAFLD among hospitalizations in the US. Nonobese NAFLD hospitalizations were less likely to have hypertension and diabetes, but more likely to have decompensated liver disease. Further studies are needed to better characterize these patients to enable early detection, treatment, and reduction in complications of liver disease.
Unusual clinical course Background:Paravalvular leaks (PVL) are becoming more commonly experienced in clinical practice due to the increasing number of mitral valve replacements performed. There are about 182 000 valve replacements performed annually, with a 5-15% prevalence rate of paravalvular leaks. Due to increased mortality associated with surgical repair, percutaneous transcatheter closure procedures are increasingly being performed as an alternative to repeat surgery. Case Report:We present the case of a 52-year-old woman with past medical history of mitral valve endocarditis who developed worsening acute heart failure 1 month after surgical bioprosthetic mitral valve replacement. Transesophageal echocardiography at the time revealed dehiscence of the bioprosthetic mitral valve and severe mitral regurgitation. She subsequently had emergent surgical bioprosthetic mitral valve replacement and annular reconstruction. The post-operative course was complicated by increasing dyspnea and lower-extremity edema, with recurrent pericardial tamponade requiring placement of a pericardial window. Based on her multiple comorbidities, most notably the concomitant right ventricular failure with severe pulmonary hypertension and prior pericardial patch repair with compromise of her mitral valve annulus, she was deemed inoperable for re-do surgery and eventually underwent a successful percutaneous closure of the mitral paravalvular leak with a ventricular septal defect (VSD) Amplatzer occluder device. The patient made good recovery and was discharged home a few days after the procedure. Conclusions:Although use of the Amplatzer VSD occluder device for this indication currently remains off-label, our report supports the use of the VSD occluder device in this subset of patients considering the high mortality rates associated with repeat surgical procedure.
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