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Key Points High-dose daunorubicin benefits AML patients with favorable and intermediate cytogenetics and with FLT3-ITD, NPM1, and DNMT3A mutations. High-dose daunorubicin is required for the favorable impact of the NPM1 mutation in AML.

Isolated left ventricular noncompaction (LVNC) is a genetic cardiomyopathy characterized by prominent ventricular trabeculations and deep intertrabecular recesses, or sinusoids, in communication with the left ventricular cavity. The low prevalence of patients with this cardiomyopathy presents a unique challenge for large, prospective trials to assess its pathogenesis, management, and outcomes. In this paper we review the embryology and genetics of LVNC, the diagnostic approach, and propose a management approach based on the current literature available.

SummaryThe Wood's lamp aids in the diagnosis of multiple infectious, inflammatory and neoplastic dermatologic conditions. Although the Wood's lamp has many applications, which have improved both the diagnosis and management of disease, the man credited for its invention is relatively unknown in medicine. Robert Williams Wood, a prominent physicist of the early 20th century, is credited for the invention of the Wood's lamp. Wood was the father of infrared and ultraviolet photography and made significant contributions to other areas in optics and spectroscopy. Wood's work encompassed the formative years of American Physics; he published over 200 original papers over his lifetime. A few years after the invention of the Wood's lamp for ultraviolet photography, physicians in Europe adopted the Wood's lamp for dermatologic applications. Wood's lamp remains popular in clinics globally, given its ease of use and ability to improve diagnostic precision.

An all-in-one Circulating Tumor Cell (CTC) isolation platform with streamlined multiplex integration of proteomic and genomic clinical testing.

BackgroundThe objective of this work was to describe the association between baseline clinical characteristics and attendance at follow‐up visits after endoscopic sinus surgery (ESS).MethodsA retrospective review of chronic rhinosinusitis (CRS) patients electing ESS at a tertiary‐care center (2011‐2013) was conducted. Baseline characteristics were studied for association with clinic attendance at 3, 6, 12, and 24 months post‐ESS.ResultsWe identified 268 patients. Significant decline occurred in the number of patients following up after ESS (3 months: 246 patients [92%]; 6 months: 145 [54%]; 12 months: 94 [35%]; and 24 months: 90 [34%]). Older patients were more likely to follow up at 3 months (mean age 55.7 vs 48.4 years, p = 0.04) and 6 months (57.5 vs 52.3 years; p = 0.01) post‐ESS. Those with higher preoperative sinus computed tomography (CT) (Lund‐Mackay) scores were more likely to follow at 6 months (11.8 vs 10.3, p = 0.01), 12 months (12.6 vs 10.3, p < 0.001), and 24 months (12.2 vs 10.5, p = 0.01). At 12 months having asthma (p = 0.03), previous ESS (p = 0.04), nasal polyps (p < 0.0001), allergic fungal sinusitis (AFS) (p = 0.002), and granulomatosis with polyangiitis (GPA) (p = 0.01) were associated with clinic attendance. At 24 months asthma status (p = 0.003), previous ESS (p = 0.002), AFS (p = 0.04), GPA (p = 0.001), and aspirin‐exacerbated respiratory disease (AERD) (p = 0.006) were associated with higher attendance. Geographical proximity did not impact attendance.ConclusionThe sharpest decline in patient follow‐up occurred between 3–6 months after ESS. Higher preoperative sinus CT scores, previous ESS, presence of asthma, and diagnosis of recalcitrant CRS subtype (nasal polyps, AERD, AFS, GPA) were factors associated with increased long‐term attendance.

Dysrhythmias and Death in BiMVPIntroductionBileaflet mitral valve prolapse (BiMVP) is common among survivors of otherwise unexplained sudden cardiac death, but prognostic implications of BiMVP are unknown. This study evaluated whether patients with BiMVP are at higher risk for ventricular dysrhythmias, ICD placement, or death compared to controls with either single‐leaflet mitral valve prolapse (SiMVP) or no mitral valve prolapse (MVP).Methods and ResultsThis retrospective, matched cohort study included 18,786 patients who underwent echocardiography at Mayo Clinic between June 1990 and September 2014. The study included three cohorts: BiMVP, SiMVP, and controls without MVP. We assessed rates of ventricular dysrhythmias, ICD placement, and all‐cause mortality between groups. BiMVP was associated with higher rates of ventricular tachycardia compared to SiMVP and controls (adjusted HR 1.48 [1.14–1.92], P = 0.003 and 1.40 [1.04–1.88], P = 0.026, respectively); however, there were no statistically significant differences in rates of ventricular fibrillation/cardiac arrest or ICD placement between groups. BiMVP was associated with a lower rate of all‐cause mortality compared to SiMVP and controls (adjusted HR 0.86 [0.79–0.94], P = 0.0008, and 0.55 [0.50–0.60], P < 0.0001, respectively).ConclusionAlthough BiMVP is associated with ventricular tachycardia, it is not associated with an increased risk of cardiac arrest/ventricular fibrillation or ICD implantation and is, paradoxically, associated with a better survival compared to SiMVP or matched controls. The findings suggest that, despite its association with ventricular tachycardia, BiMVP in the absence of other risk factors does not seem to portend a poor prognosis at the population level.

ObjectiveTo study the relationship between serum hydroxychloroquine (HCQ) concentrations and flares of systemic lupus erythematosus (SLE) in a longitudinal cohort of patients.MethodsPatients who fulfilled ≥4 American College of Rheumatology classification criteria for SLE and had been treated with HCQ for >6 months were studied. Blood was assayed for HCQ levels by tandem mass spectrometry. Patients were serially assessed for disease activity, using the Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA) version of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and flares (SELENA flares instrument). Comparison of the mean summated SLEDAI scores over time and rates of flares in groups with different HCQ levels was performed by the Kruskal‐Wallis test.ResultsA total of 276 SLE patients were studied (93% women, mean ± SD age 41.0 ± 13.8 years). The proportion of patients with HCQ levels <10 (total noncompliance), 10–500 (subtherapeutic), and >500 ng/ml (therapeutic) was 11%, 77%, and 12%, respectively. HCQ levels correlated significantly with the prescribed dose but not with body weight or renal function. The prescribed HCQ dose also correlated significantly with baseline SLEDAI scores, indicating that higher doses were used for more active manifestations. After a mean ± SD observation period of 32.5 ± 5.5 months, the mean summated SLEDAI score and the incidence of SLE flares was not statistically different among patients with different baseline HCQ levels. In a subgroup of 73 patients with serologic and clinical remission and having therapeutic HCQ levels, a trend of lower disease activity and fewer incidences of flares was observed.ConclusionNoncompliance and subtherapeutic serum HCQ levels were seen frequently in these SLE patients, which was partly due to the low prescribed dose. In patients in remission, higher HCQ concentrations were associated with a trend showing fewer flares over time.