Amyloidosis: What does pathology offer? The evolving field of tissue biopsy

Since the mid-nineteenth century pathology has followed the convoluted story of amyloidosis, recognized its morphology in tissues and made identification possible using specific staining. Since then, pathology studies have made a significant contribution and advanced knowledge of the disease, so providing valuable information on the pathophysiology of amyloid aggregation and opening the way to clinical studies and non-invasive diagnostic techniques. As amyloidosis is a heterogeneous disease with various organ and tissue deposition patterns, histology evaluation, far from offering a simple yes/no indication of amyloid presence, can provide a wide spectrum of qualitative and quantitative information related to and changing with the etiology of the disease, the comorbidities and the clinical characteristics of patients. With the exception of cardiac transthyretin related amyloidosis cases, which today can be diagnosed using non-biopsy algorithms when stringent clinical criteria are met, tissue biopsy is still an essential tool for a definitive diagnosis in doubtful cases and also to define etiology by typing amyloid fibrils. This review describes the histologic approach to amyloidosis today and the current role of tissue screening biopsy or targeted organ biopsy protocols in the light of present diagnostic algorithms and various clinical situations, with particular focus on endomyocardial and renal biopsies. Special attention is given to techniques for typing amyloid fibril proteins, necessary for the new therapies available today for cardiac transthyretin related amyloidosis and to avoid patients receiving inappropriate chemotherapy in presence of plasma cell dyscrasia unrelated to amyloidosis. As the disease is still burdened with high mortality, the role of tissue biopsy in early diagnosis to assure prompt treatment is also mentioned

Phenotype and immune function of lymph node and peripheral blood CLL cells are linked to transendothelial migration

everal lines of evidence suggest that homing of tumor cells to lymphoid tissue contributes to disease progression in chronic lymphocytic leukemia (CLL). Here, we demonstrate that lymph node (LN)-derived CLL cells possess a distinct phenotype, and exhibit enhanced capacity for T-cell activation and superior immune synapse formation when compared with paired peripheral blood (PB) samples. LN-derived CLL cells manifest a proliferative, CXCR4(dim)CD5(bright) phenotype compared with those in the PB and higher expression of T-cell activation molecules including CD80, CD86, and HLA-D-related (DR). In addition, LN-CLL cells have higher expression of α4β1 (CD49d) which, as well as being a co-stimulatory molecule, is required for CLL cells to undergo transendothelial migration (TEM) and enter the proliferation centers of the LNs. Using an in vitro system that models circulation and TEM, we showed that the small population of CLL cells that migrate are CXCR4(dim)CD5(bright) with higher CD49d, CD80, CD86, and HLA-DR compared with those that remain circulating; a phenotype strikingly similar to LN-derived CLL cells. Furthermore, sorted CD49d(hi) CLL cells showed an enhanced capacity to activate T cells compared with CD49d(lo) subpopulations from the same patient. Thus, although PB-CLL cells have a reduced capacity to form immune synapses and activate CD4(+) T cells, this was not the case for LN-CLL cells or those with the propensity to undergo TEM. Taken together, our study suggests that CLL cell immunologic function is not only modulated by microenvironmental interactions but is also a feature of a subpopulation of PB-CLL cells that are primed for lymphoid tissue homing and interaction with T cells

Obstructive Sleep Apnea Syndrome Analysis Using Stereophotogrammetry: A Sistematic Review

Aim: Obstructive sleep apnea syndrome (OSAS) is a disorder characterized by nocturnal breathing interruptions due to upper airway obstruction. The aim of the review is to verify the existence of any current literature concerning the craniofacial stereophotogrammetric evaluation in order to predict the presence and severity of OSAS. This technology uses a three-dimensional model obtained from multiple frames of the face taken from different angles. Methods: The research was performed on MEDLINE-PUBMED without applying temporal or linguistic restrictions. From 19 studies, 4 were selected. Results: The study of Banabilh et al. 2009 shows how the severity of OSAS depends on the deposition of submandibular fat, neck circumference and body mass index. According to the study of Lin et al. 2018 numerous measurements concerning areas, volumes and anthropometric lines of the head and neck district indicate the presence and severity of the pathology. In the study of Ohmura et al. 2022 it has been demonstrated that mandibular width, length, depth and width-lenght angle are correlated with OSAS severity. In the study of Tyler et al. 2022 emerged that an increasing obtuse angle of facial convexity is found in severe OSAS subject. Conclusions: In this review emerged a correlation between OSAS severity and submandibular fat deposition, neck circumference, body mass index, facial convexity as well as numerous anthropometric parameters from the literature. It should be note the limited amount of scientific material currently available in this regard

Association between radiological findings and severity of community-acquired pneumonia in children

Background: There are few published data concerning radiological findings and their relationship with community-acquired pneumonia (CAP) severity. The aim if this study was to assess radiographic findings in children with CAP of different severity in order to evaluate whether some parameters are associated with severe CAP. Methods. We analysed the characteristics of parenchymal densities in 335 chest radiographs of otherwise healthy children (173 males; mean age \ub1 standard deviation, 7.5 \ub1 4.5 years) admitted to our Emergency Room for CAP. Upon admission, chest radiographs were obtained in the two standard projections, and the children with severe or mild/moderate CAP were compared in order to identify any correlations between CAP severity and the radiological findings. Results: Seventy-six of the 335 enrolled children (22.7%) fulfilled the criteria for severe CAP. In comparison with the children with mild/moderate CAP, in severe CAP there was a significantly greater frequency of a bilateral multifocal distribution (p = 0.01), the simultaneous involvement of 653 sites (p = 0.007), and the involvement of the right hilum (p = 0.02). The same results were confirmed in the multiple logistic regression model. Conclusions: This study shows that radiological findings such as a multifocal bilateral distribution, the simultaneous involvement of at least three sites, and right hilar consolidation are associated with severe CAP in otherwise healthy children, and could be considered markers of disease severity in children with CAP