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This prospective study was conducted on 108 consecutive implantations straight, cementless, taper-designed stem with variable lateral flare (Pegasus; DePuy, Warsaw, Indiana) at an 8- to 10-year follow-up; DEXA scanning was performed at 3 and 12 months on a second cohort of 40 patients. The mean Harris hip score was 94.2, and no revision was performed. Measurable subsidence of the stem was found in three (2.8%) patients; the subsidence was always measured within the first year of follow-up, and did not progress at the subsequent controls. Bone mineral density decreased at 1 year, 15.1% in zone 1 and 3.4% in zone 7. Uncemented stems with a taper design, which provide a high metaphyseal fit and a low diaphyseal fit, lead to comparable and reproducible long-term results independently from other design characteristics. [ABSTRACT FROM AUTHOR]

Left ventricular noncompaction (LVNC), involving mainly the right ventricle, is a rare form of congenital heart disorder characterized by a developmental arrest in myocardial compaction, resulting in a spongy appearance of the myocardium, mainly of the right ventricle, rarely detected in fetuses. We report the case of a female fetus with a gestational age of 41 +4 weeks who came to our attention for intrapartum sudden unexpected death, resulting in stillbirth. The ventricular walls, particularly the right ventricular wall, appeared thick, hypertrabeculated and spongy, leading to the diagnosis of LVNC involving mainly the right ventricle. The atrioventricular node and His bundle presented areas of fetal dispersion and resorptive degeneration; islands of conduction tissue were detected in the central fibrous body. Arcuate nucleus of the brainstem showed bilateral severe hypoplasia. The right bundle branch was hypoplastic. The final cause of death was an electrical conduction disfunction in an LVNC involving mainly the right ventricle. To the best of our knowledge, the herein described case is the first reported observation of sudden intrapartum death from LVNC involving mainly the right ventricle well documented post-mortem with cardiac conduction and brainstem studies. Our findings confirm the need of an accurate post-mortem examination including the study of the cardiac conduction system on serial section in every case of sudden unexpected fetal death, although there are no universally recognized guidelines., Competing Interests: Declaration of competing interest All authors do not have any financial or personal relationships with people or organizations that could inappropriately influence their work., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background:  Low von Willebrand factor (VWF) refers to subjects with plasma levels of 30 to 50 IU/dL. The mechanism of low VWF is poorly understood. We chose to determine the clinical presentation, laboratory phenotype, and underlying mechanisms of low VWF., Material and Methods:  We included 250 patients characterized with low VWF. The International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT) was used to assess clinical symptoms. To determine the underlying mechanisms of low VWF, we used as markers the VWF propeptide (VWFpp) assay and FVIII:C/VWF:Ag ratio for VWF synthesis and the VWFpp/VWF:Ag ratio for VWF clearance. Results were compared with those of 120 healthy controls. Cases with abnormal screening tests were further evaluated for coagulation factor levels and platelet disorders., Results:  The median age of the cohort was 35 years (range 3-85), 21% were children ( n  = 53), 34% were adult males ( n  = 85), and 45% ( n  = 112) were adult females. According to the ISTH-BAT, abnormal bleeding was found in 35% of children, 47% of males, and 49% of females. No association was found between VWF activity levels and ISTH-BAT. Patients showed an overall decreased VWF synthesis/secretion and an enhanced VWF clearance was identified in 33% of them. In 89 patients (36%), there were other hemostasis-related defects, but there was no difference in the ISTH-BAT between the two groups., Conclusion:  Our findings indicate that reduced VWF synthesis/secretion and enhanced VWF clearance are major mechanisms of low VWF levels. Patients with low VWF have significant bleeding manifestations. While other hemostasis defects occurred together with low VWF, this combination did not exacerbate clinical symptoms., Competing Interests: F.P. reports participation at educational meetings and the advisory board of Sanofi, Sobi, Takeda, Roche, and Biomarin. The other authors state that they have no conflict of interest., (Thieme. All rights reserved.)

Introduction: The increased life expectancy of patients with haemophilia A (HA) has led to a growing prevalence of cardiovascular risk factors and events. There is still scarce evidence on the safety and appropriate duration of dual antiplatelet therapy (DAPT) after acute coronary syndrome (ACS) in HA patients., Aim: We describe our experience on the clinical management of Italian HA patients after ACS., Methods: Nine patients with congenital HA treated with DAPT after a revascularization procedure performed for ACS have been enrolled and followed at the Angelo Bianchi Bonomi Haemophilia and Thrombosis Center in Milan between 2005 and September 2022. The safety and efficacy of DAPT with or without FVIII prophylaxis were assessed., Results: Ten ACS events occurred in the nine HA patients (four mild and five severe). All events were treated with percutaneous transluminal coronary angioplasty with deployment of 1 to 3 drug-eluting stents followed by DAPT for 1-12 months. All patients except one were treated with FVIII prophylaxis during DAPT aimed at achieving FVIII trough levels ≥20-30 IU/dL. DAPT was effective in all cases in preventing early ACS recurrence, with only a late recurrence. We observed two clinically relevant non-major bleeds (one in a patient without FVIII prophylaxis) and three minor bleeds. No venous thrombosis occurred., Conclusion: The long-term secondary antithrombotic prevention consisting of DAPT and FVIII prophylaxis achieving a trough level of 20-30 IU/dL can be effective and safe in HA patients., (© 2024 John Wiley & Sons Ltd.)

Basosquamous carcinoma (BSC), an uncommon and aggressive nonmelanoma skin cancer exhibiting characteristics ranging from basal cell carcinoma (BCC) to squamous cell carcinoma (SCC), is a subject of controversy in terms of its classification, pathogenesis, histologic morphology, biologic behavior, prognosis, and management. This narrative review is based on an electronic search of English-language articles in PubMed that included the terms "basosquamous carcinoma" and/or "metatypical carcinoma of the skin" in their titles. The review aims to succinctly present and assess current data on the epidemiology, clinical presentation, dermoscopic, LC-OCT, and histopathologic characteristics, as well as the genetics and management of BSC, providing insight into this intriguing entity. As a conclusion, dermoscopy, deep incisional biopsies, and immunohistologic techniques should be applied in clinically suspicious lesions to achieve an early diagnosis and better prognosis of this tumor. Surgical treatments, including wide excision and Mohs' micrographic surgery, remain the treatment of choice. Finally, Hedgehog pathway inhibitors and checkpoint inhibitors, must be thoroughly investigated with large controlled trials, since they may offer an alternative solution to irresectable or difficult-to-treat locally advanced cases of basosquamous carcinoma.

Objective: The rationale for the use of autologous fat grafting in the treatment of vulvar lichen sclerosus (VLS) consists in reduction of inflammation, regeneration of tissues, volume increase, and pain fiber control. The main outcome of this study was the evaluation of patients' satisfaction after treatment. Secondary outcomes included modifications in symptoms, psychosexual wellbeing, vulvar hydration, and histology after surgery., Methods: Eligible for this study were women aged 18-85 years with a histological diagnosis of VLS who underwent at least one autologous vulvar fat grafting at the authors' center, between 2010 and 2019. In 2021, all women underwent a clinical reevaluation, comprehensive of vulvoscopy, vulvar biopsy, and handing out of validated questionnaires., Results: Overall, 88.7% of patients declared themselves very satisfied/satisfied with the procedure. All symptoms were improved postsurgery; in particular, the difference was statistically significant for pruritus, burning, and dyspareunia ( p < .05). Sexual function was also improved at time of reevaluation, as were depressive and anxiety symptoms ( p < .05). No cases of vulvar intraepithelial neoplasia or cancer occurred during follow-up and vulvar architecture remained stable, although patients reported a significantly reduced need for topical steroids ( p < .0001). Lastly, in postoperative biopsies, inflammatory infiltrate was stable or reduced, and the distribution of elastic fibers was comparable or restored in most patients., Conclusions: Patient satisfaction with fat grafting is detectable up to 11 years after surgery, and as such, it may represent a valid therapeutic option in selected cases of VLS., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2023, ASCCP.)

Background: To evaluate the effect of different experimental staining procedures on color stability and translucency of a nano-hybrid resin-based composite (RBC)., Methods: Forty-eight cylindrical-shaped specimens (10 × 2 mm) were prepared with a nano-hybrid RBC (Clearfil Majesty ES-2) and randomly divided in four groups according to the experimental staining procedure: G1) static immersion in a staining solution (coffee) (44 ± 1 °C); G2) staining cycling between coffee (44 ± 1°C) and distilled water (37 ± 1°C) with an experimental staining machine based on Arduino, an Open Source hardware development platform; G3) staining cycles as in G2 + brushing with a low abrasive toothpaste (Relative Dentin Abrasion RDA = 30) (Elmex Sensitive Professional); G4) staining cycles as in G3, with brushing performed with a very strong abrasive toothpaste (RDA = 90) (Lacult Active). Color parameters were recorded at the baseline (T0) after staining procedures (T1) and repolishing (T2) using a spectrophotometer. Color change (∆E00) and translucency (TP, CR) were evaluated. Data were statistically analyzed ( p < 0.05)., Results: For ∆E00 after staining, Group 1 showed the highest color change and Group 3 the lowest. All groups were significantly different ( p < 0.001) except for Group 2 vs. Group 4; after repolishing, Group 1 was significantly higher than Group 3 ( p < 0.001), Group 2 ( p < 0.001), and Group 4 ( p = 0.003); Group 2 was higher than Group 3 ( p < 0.001). For TP variable, after staining procedures, Group 2 was significantly higher than all other groups ( p < 0.001), and Group 1 was significantly higher than Group 3 ( p < 0.001) and Group 4 ( p = 0.007). After repolishing, Group 4 was significantly lower than Group 3 ( p = 0.008) and Group 2 ( p = 0.027). Repolishing procedure significantly reduced color parameters., Conclusions: The investigated staining procedure induced significant differences in color stability and translucency. The use of a very strong abrasive toothpaste (RDA = 90) induced higher color change than a low abrasive one (RDA = 30). Repolishing procedures are able to partially reduce color change induced by artificial staining procedures.

Competing Interests: Conflict of interest: The authors declare that they have no conflicts of interest.

Background: Breast cancer with osteoclast-like stromal giant cells (OSGC) is an exceedingly rare morphological pattern of invasive breast carcinoma. The tumor immune microenvironment (TIME) of these tumors is populated by OSGC, which resemble osteoclasts and show a histiocytic-like immunophenotype. Their role in breast cancer is unknown. The osteoclast maturation in the bone is regulated by the expression of cytokines that are also present in the TIME of tumors and in breast cancer tumor-associated macrophages (TAMs). TAMs-mediated anti-tumor immune pathways are regulated by miRNAs akin to osteoclast homeostasis. Here, we sought to characterize the different cellular compartments of breast cancers with OSGC and investigate the similarities of OSGC with tumor and TIME in terms of morphology, protein, and miRNA expression, specifically emphasizing on monocytic signatures. Methods and Results: Six breast cancers with OSGC were included. Tumor-infiltrating lymphocytes (TILs) and TAMs were separately quantified. The different cellular populations (i.e., normal epithelium, cancer cells, and OSGC) were isolated from tissue sections by laser-assisted microdissection. After RNA purification, 752 miRNAs were analyzed using a TaqMan Advanced miRNA Low-Density Array for all samples. Differentially expressed miRNAs were identified by computing the fold change (log2Ratio) using the Kolmogorov-Smirnov test and p values were corrected for multiple comparisons using the false discovery rate (FDR) approach. As a similarity analysis among samples, we used the Pearson test. The association between pairs of variables was investigated using Fisher exact test. Classical and non-classical monocyte miRNA signatures were finally applied. All OSGC displayed CD68 expression, TILs (range, 45-85%) and high TAMs (range, 35-75%). Regarding the global miRNAs profile, OSGC was more similar to cancer cells than to non-neoplastic ones. Shared deregulation of miR-143-3p, miR-195-5p, miR-181a-5p, and miR-181b-5p was observed between OSGC and cancer cells. The monocyte-associated miR-29a-3p and miR-21-3p were dysregulated in OSGCs compared with non-neoplastic or breast cancer tissues. Conclusion: Breast cancers with OSGC have an activated TIME. Shared epigenetic events occur during the ontogenesis of breast cancer cells and OSGC but the innumophenotype and miRNA profiles of the different cellular compartmens suggest that OSGC likely belong to the spectrum of M2 TAMs., Competing Interests: The reviewer FF declared a shared affiliation with the author(s) AT to the handling editor at the time of review. The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Sajjadi, Gaudioso, Terrasi, Boggio, Venetis, Ivanova, Bertolasi, Lopez, Runza, Premoli, Lorenzini, Guerini-Rocco, Ferrero, Vaira and Fusco.)

Thrombocytopenia is a common feature of antiphospholipid syndrome (APS) and rarely requires treatment. Here we present the case of a 71-year-old man hospitalized for severe immune thrombocytopenia (ITP) secondary to APS and concomitant SARS-CoV-2 infection. The patient was successfully treated with systemic corticosteroids, intravenous immunoglobulins, and plasma exchange (PEX). Few data are published on the use of plasma exchange in the treatment of thrombocytopenia in non-catastrophic APS. In the setting of acute infection when immunosuppressive therapies might be contraindicated, plasma exchange may be considered an effective therapeutic option. SARS-CoV-2 infection may be a trigger for a relapse of immune thrombocytopenia., (© 2022. The Author(s).)

Competing Interests: None disclosed.

High-risk Myelodysplastic syndromes (MDS) represent therapeutical challenges and are usually managed with hypomethylating agents such as azacitidine. Given the lack of data in the literature concerning azacitidine effects on bone marrow, we retrospectively analyzed 57 high-risk MDS cases in order to identify any changes induced by azacitidine therapy or relevant correlations between therapy response and pre- or post-treatment features. Azacitidine treatment had no significant impact on bone marrow cellularity or morphological dysplastic features. On the contrary, although not statistically significant, we observed a slight decrease in CD34+ and CD117+ blasts and p53+ precursors after treatment. Moreover, pre-treatment IPSS-R cytogenetic score ( p = 0.004), lymphocytic infiltrate ( p = 0.017) and p53+ elements ( p = 0.001) correlated with AML progression; pre-treatment lymphocytic infiltrate was also linked to better response to therapy ( p = 0.004), suggesting an anti-tumoral role of bone marrow microenvironment. Post-treatment blast count impacted negatively on overall survival ( p = 0.035) and risk of leukemic progression ( p = 0.04), while both post-treatment lymphocytic infiltrate and p53+ elements showed significant correlation with treatment response ( p = 0.004 and p = 0.003 respectively). Higher post-treatment p53+ elements correlated also with risk of leukemic progression ( p = 0.013). Our results suggest the possible role of lymphocytic infiltrate and p53+ elements as predictive markers in MDS treated with azacitidine, disclosing new chapters in the understanding of MDS evolution and treatment.

We observed ten children with a papular eruption with purpuric features during the SARS-CoV-2 pandemic in Northern Italy (May-December 2020). Histological examination showed signs of SARS-CoV-2-related dermatosis. Evidence of nucleocapsid viral proteins using SARS-CoV-2 (2019-nCoV) nucleocapsid antibody revealed cuticular staining of the deep portion of the eccrine glands in all cases., (© 2021 Wiley Periodicals LLC.)

Background: Formaldehyde and xylene are two hazardous chemicals widely used in pathology laboratories all over the world. The aim of this work was to survey a large volume pathology lab, measuring exposure of workers and residents to formaldehyde and xylene, and verify the efficacy of the undertaken preventive actions and the accomplishment with occupational limit values., Methods: Environmental, personal, and biological monitoring of exposure to formaldehyde and xylene in different lab rooms and in 29 lab attendants was repeated yearly from 2017 to 2020. Continuous monitoring of airborne formaldehyde was performed to evaluate the pattern of airborne concentrations while specific tasks were performed. Several risk management and mitigation measures, including setting a new grossing room, reducing the number of samples to be soaked in formaldehyde, and improving the lab practices and equipment, such as the use of chemical hoods, were undertaken after each monitoring campaign, based on the results obtained from the exposure monitoring., Results: Significant exposures to formaldehyde in pathologists and residents, especially during the grossing of samples, were observed in the first 2 years, with exposure exceeding the occupational exposure limit value; the following surveys showed that the risk management and mitigation measures were effective in reducing airborne concentrations and personal exposure. Xylene, assessed with both environmental and biological monitoring, was always well below the occupational exposure limit value and biological limit values, respectively., Conclusion: Critical exposure to air formaldehyde in attendants of a pathology laboratory could be reduced with the re-organization of lab spaces, new and improved work procedures, and awareness and training initiatives., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Occupational Hygiene Society.)

Introduction: The first-line therapy for patients with low-risk myelodysplastic syndromes (MDSs) commonly consists of erythropoietin stimulating agents (ESAs), with a response rate ranging from 34 to 62%. For nonresponder patients, outside clinical trials, blood transfusions are the most frequent therapeutic option, with detrimental effect on the quality of life and with risks of iron-overload. Since no studies have been yet conducted on this topic, we investigated the potential predictive role of bone marrow (BM) histological evaluation in patients treated with ESAs., Materials and Methods: We performed a morphological and immunohistochemical retrospective analysis of BM biopsies of 96 patients with low-risk MDSs subsequently treated with ESAs., Results: In our series, substantial morphological overlap was found between responder and nonresponder patients. On the contrary, patients with a percentage of CD34-positive blasts >3% or with p53 protein expression <1% responded with a significantly higher frequency to ESAs., Conclusions: Our study reinforces the role of BM biopsy as diagnostic tool in MDSs, being also able to supply information related to response to ESAs and to its loss over time., (© 2021 S. Karger AG, Basel.)

Skin manifestations of COVID-19 infections are diverse and are new to the dermatology community. We had the opportunity to examine the clinical and histopathological features of several patients who were divided into 3 groups. The first group included 8 COVID-19-positive patients who were hospitalized and quarantined at home. The second group included children and young adults who presented with chilblain erythema, erythema multiforme, and urticaria-like lesions. This group of patients was negative for the COVID-19 gene sequences by polymerase chain reaction but had a high risk of COVID-19 infection. The third group included clinically heterogeneous and challenging lesions. These patients were not subject to either polymerase chain reaction tests or serological analyses because they sought dermatological attention only for a dermatosis. The histopathological analysis of these cases showed a wide spectrum of histopathological patterns. What appears to be constant in all skin biopsies was the presence of prominent dilated blood vessels with a swollen endothelial layer, vessels engulfed with red blood cells, and perivascular infiltrates, consisting mainly of cytotoxic CD8+ lymphocytes and eosinophils. In 2 cases, there was diffuse coagulopathy in the cutaneous vascular plexus. In the early phases of the disease, there were numerous collections of Langerhans cells in the epidermis after being activated by the virus. The presence of urticarial lesions, chilblains, targetoid lesions (erythema multiforme-like lesions), exanthema, maculohemorrhagic rash, or chickenpox-like lesions associated with the histopathological features mentioned previously should cause clinical dermatologists to suspect the possibility of COVID-19 infection, especially in patients with fever and cough.

During the COVID-19 pandemic, chilblain-like lesions have been reported in mildly symptomatic children and adolescents. We present four children investigated for suspected COVID-19 infection who presented with acral skin findings and mild systemic symptoms. Histology from one case showed signs of vasculitis with evident fibrin thrombus., (© 2020 Wiley Periodicals LLC.)