A Case Series of Blastic Plasmacytoid Dendritic Cell Neoplasia

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), an extremely rare and aggressive tumor, derives from plasmacytoid dendritic cell precursors and is characterized by CD4 and CD56 positivity accompanied by the expression of isolated myeloid, B- or T-cell lineage markers. Despite the recent introduction of specific targeted therapies, prognosis is still poor with a median overall survival of one year, and allogeneic bone marrow transplantation remains the only curative treatment in eligible patients. In this series, we described two cases of adult BPDCN treated with high dose cytarabine and methotrexate and autologous hematopoietic stem cell transplantation, or fludarabine, cytarabine, and idarubicin achieving the first a complete lasting remission, while the second only a transient improvement in skin lesion

Lung Ultrasound, Echocardiography, and Fluid Challenge for the Differential Diagnosis of Pulmonary Hypertension

Objectives: The differential diagnosis between pulmonary arterial hypertension (PAH) and postcapillary pulmonary hypertension (PH) in heart failure with preserved ejection fraction (HFpEF) is sometimes difficult despite guidelines-derived standardized step-by-step diagnostic algorithms. We therefore explored the added value of lung ultrasound to a previously validated echocardiographic score of right heart catheterization measurements. Methods: Patients referred for PH underwent a right heart catheterization, echocardiography, and lung ultrasound before and after rapid infusion of 7 mL/kg of saline. A 7-point echocardiographic score based on cardiac chamber dimensions and estimates of filling pressures was implemented for the prediction of precapillary PH. Pulmonary congestion was identified by lung ultrasound B lines. Results: The study enrolled 70 patients with PAH and 77 patients with HFpEF. The PAH patients had a higher echocardiographic score (3.5 ± 1.8 vs 1.6 ± 1.5; P < .001). The HFpEF patients had more B lines both before (8.1 ± 4.2 vs 5.1 ± 3.0; P < .001) and after fluid challenge (14.6 ± 5.4 vs 7.6 ± 3.5; P < .001) and a more important increase (Δ) of B lines after fluid challenge (6.5 ± 2.9 vs 2.5 ± 1.6; P < .001). The sensitivity and specificity of the echocardiographic score (cutoff ≥2) alone for PAH were 0.91 and 0.49, respectively (area under the curve of 0.78). The best diagnostic improvement was observed with addition of ΔB lines + E/e' post-fluid challenge to the echocardiographic score, with a significant increase of the area under the curve (0.98) and (with a cutoff given by the presence of echo score ≥2, ΔB lines <4 and E/e' post < 11) a sensitivity of 0.90 (95% CI, 0.83; 0.97) and specificity of 0.84 (95% CI, 0.76; 0.93). Conclusions: Lung ultrasound combined with echocardiography at baseline and after fluid challenge has an incremental value for the differential diagnosis between PAH and PH-HFpEF

Right heart failure in left heart disease: imaging, functional, and biochemical aspects of right ventricular dysfunction

: For decades, cardiologists have largely underestimated the role of the right heart in heart failure due to left heart disease. Nowadays, the importance of evaluating right ventricular (RV) structure and function in left heart failure is well documented and this concept has been emphasized in the most recent heart failure guidelines. However, several relevant questions remain unanswered such as the following: (a) which imaging technique (standard or 3D echocardiography or strain imaging or cardiac magnetic resonance) and, more, which parameters should be used to grade the severity of RV dysfunction? (b) do less widespread and less applied diagnostic tools such as cardiopulmonary stress testing and bioelectrical impedance analysis play a role in this field? (c) are there specific biochemical aspects of RV failure? (d) why notion of pathophysiology of heart and lung interaction are so well appreciated at an academic level but are not applied in the clinical setting? The present review has been prepared by the Heart Failure (HF) working group of the Italian Society of Cardiology and its main objective is to improve our understanding on RV dysfunction in heart failure