Blastic Plasmacytoid Dendritic Cell Neoplasm: Single Center Experience on a Rare Hematological Malignancy

PurposeBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and poor prognostic hematological malignancy. There is still no standard treatment established for BPDCN patients. We aim to summarize the main clinical, biological features and treatment of 9 BPDCN patients.MethodsNine patients with BPDCN who had been diagnosed between July 2008 and December 2018 in Ankara University School of Medicine, were retrospectively evaluated.ResultsAll patients (n = 9) were male, median age was 64 (21–80). Five patients (55.6%) had bone marrow infiltration, 5 patients (55.6%) cutaneous lesions, 6 patients (66.7%) lymph node involvement, 2 patients (22.2%) central nervous system involvement and 2 patients (22.2%) spleen involvement at time of diagnosis. Complex karyotype was observed in 2 patients. CHOP was given to 5 patients (55.6%), hyper-CVAD to 2 patients (22.2%), fludarabine, cyclophosphamide and mitoxantrone to 1 patient (11.1%) and cyclophosphamide, etoposide, methylprednisolone to 1 patient (11.1%) as first line chemotherapy. Four patients (44.4%) underwent allogeneic hematopoietic stem cell transplantation (AHSCT) in complete remission (CR) 1. Venetoclax was given to a transplant ineligible patient who had skin and lymph node involvement, with the off-label use. The median follow-up time was 15.9 months (3–48.6 months). Estimated median overall survival was 15.9 + 1.6 (95% CI 12.7–19.1) months.ConclusionIntensive induction therapies followed by AHSCT in CR seems to be best approaches for patients with BPDCN. Thus, more effective treatment strategies particularly targeted therapies should be warranted to improve the survival of patients with this rare disease.     

A Mysterious Crescent in the Heart: Ruptured Sinus of Valsalva Dissecting Interatrial Septum,Multimodality Imaging,and Full Recovery

A 59‐year‐old man who was admitted to the emergency department with new and spontaneous onset of fatigue, dyspnea, and palpitations. There was neither a history of trauma, chest pain, nor infection. Transthoracic two‐ and three‐dimensional echocardiography and computer tomography demonstrated sinus of Valsalva aneurysm rupture dissecting interatrial septum and leading to a huge thrombus formation in it. The diagnosis with multimodality imaging performed within hours, and urgent surgery saved the patient's life.     

Mind-Body Interventions for Individuals With Heart Failure: A Systematic Review of Randomized Trials

Background

The effects of mind-body interventions (MBIs) (eg, Tai Chi, yoga, meditation) for individuals with heart failure (HF) have not been systematically evaluated.

Left ventricular remodeling and aortic distensibility in elite power athletes

The aim of this study was to determine left ventricular (LV) morphology and aortic function in power athletes and to compare them with normal subjects. Thirty-two elite male wrestlers and 15 age-matched healthy male controls were included. All subjects underwent echocardiographic examination. Measurements included LV cavity dimension at systole and diastole, wall thickness, diastolic parameters, and aortic diameter, 3cm above aortic valve, at systole and diastole. Left ventricular mass and mass index were found to be higher in the athletes than in control subjects. The aortic distensibility index was found to be reduced in the athletes compared with controls (2.53 ± 0.91 vs 3.94 ± 1.77cm²dyne^–1 10^–6, P = 0.003), while the aortic stiffness index was significantly higher in the athletes than in controls (9.12 ± 3.23 vs 6.65 ± 2.35, P = 0.02). However, LV end-systolic wall stress was lower in the athletes than in controls. Furthermore, transmitral early (E) and late (A) peak velocity, peak velocity of the myocardial systolic wave (S _m), and early (E _m) and atrial (A _m) diastolic waves at the inferior wall were higher in the athletes than in controls. Reduced aortic distensibility in elite power athletes may be one of the cardiovascular adaptation factors which affect LV hypertrophy.     

Coronary artery-left ventricular fistulae associated with apical hypertrophic cardiomyopathy.

We describe an unusual case of coronary artery-left ventricular fistulae associated with apical hypertrophic cardiomyopathy in a 63-year-old man who had a 2-year history of angina pectoris without significant coronary atherosclerosis. It is important to recognize this anomaly as it may be the source of angina in patients without angiographic evidence of major atherosclerotic coronary artery disease.     

Trace element status (Se, Zn, Cu) in heart failure.

OBJECTIVE: It has been speculated that trace elements may play a role in the pathogenesis of heart failure. In the present study, we aimed to assess serum concentrations of selenium (Se), zinc (Zn) and copper (Cu) in patients with heart failure (HF) and to compare idiopathic dilated cardiomyopathy (IDCM) and ischemic cardiomyopathy (ICM) patients with healthy controls. METHODS: This study population included 54 HF patients (26 IDCM patients and 28 ICM patients) and 30 healthy subjects. Serum levels of selenium, zinc, and copper were assessed by atomic absorption spectrophotometry method. RESULTS: Serum concentrations of Se and Zn in HF patients were significantly lower than in healthy controls (p=0.000 and p<0.01, respectively). However, serum Cu concentrations in these patients were significantly higher than in controls (p=0.000). There were no significant difference in the trace elements status between IDCM and ICM patients (p>0.05 for all parameters). Relationships of the serum trace element concentrations studied with echocardiographic and hemodynamic parameters were not statistically significant. CONCLUSION: Our study showed that heart failure is associated with lower Se and Zn concentrations, and higher Cu concentration, and serum Se, Zn and Cu element profiles were similar in IDCM and ICM.     

Effect of N-acetylcysteine on oxidative stress and ventricular function in patients with myocardial infarction

Recent evidence suggests that postischemic myocardial dysfunction (“stunning”) may be mediated by oxygen free radicals. Various studies have reported the beneficial effects of antioxidants in ischemia–reperfusion injury. The aim of this study was to assess the effect of N-acetylcysteine (NAC) treatment on oxidative stress, infarct size, and left ventricular (LV) function, as adjunct therapy in myocardial infarction (MI). Patients with acute MI received either 15 g NAC infused over 24 h (n = 15) or no NAC (n = 15), combined with streptokinase. Peripheral venous blood was serially sampled to measure creatine kinase (CK)-MB levels. Plasma malondialdehyde (MDA) level was measured at admission and after 4 and 24 h. Echocardiography was performed within 3 days of MI and after 3 months. At admission, plasma MDA levels were not different between the groups. In the NAC-treated patients plasma MDA levels decreased, whereas in the nontreated NAC patients MDA levels increased at 4 and 24 h (P < 0.01 and P < 0.001, respectively). Left ventricular ejection fraction was higher (P < 0.05) and LV end-systolic and end-diastolic diameters were lower (P < 0.001 and P < 0.001) in patients receiving NAC on day 3. Left ventricular wall motion score index was significantly lower in patients treated with NAC on day 3 (P < 0.05). Left ventricular diastolic parameters were not different whether patients were treated with NAC or not. No difference in reduction of infarct size was detected between the groups according to CK-MB levels. It was thus demonstrated that administration of NAC in combination with streptokinase significantly diminished oxidative stress and improved LV function in patients with acute MI. These encouraging results would justify the performance of a larger controlled study.     

Hemostatic changes in gestational diabetes mellitus

The purpose was to characterize the hemostatic changes in women with gestational diabetes mellitus (GDM). In this case–control study, 50 women with newly diagnosed GDM at 24–28 weeks of pregnancy and 41 normal pregnant women, matched for age, body mass index, and gestational age, were enrolled. Anthropometric, metabolic patterns, coagulation parameters, and plasminogen were measured in each subject. Plasma fibrinogen levels, plasminogen, and von Willebrand factor (vWF) activities were significantly higher in patients with GDM as compared to normal pregnant women (p < 0.001, p < 0.001, and p < 0.05, respectively). Although protein S was significantly elevated in diabetic group (p < 0.05), free protein S was similar in both groups. Coagulation factors VIII and IXa were significantly higher in patients with GDM (p < 0.001 and p < 0.01, respectively). In the group with GDM, factor VIII was positively correlated with HbA1c (r = 0.192, p < 0.001). A weak but significant negative correlation was observed between protein S and fasting glucose (r =−0.006, p < 0.05). GDM potentiates the alteration in coagulation and fibrinolysis during normal pregnancy. The question of whether the hemostatic balance is unchanged or shifts toward a hypercoagulable status remains unanswered.