Optical coherence tomography angiography features in Waldenström macroglobulinemia patients without Hyperviscosity syndrome: A pilot prospective study.

PURPOSE: To evaluate the retinal vessel density (VD) with optical coherence tomography angiography (OCTA) in asymptomatic patients affected by Waldenström macroglobulinemia (WM) without hyperviscosity syndrome (HVS) and to highlight the presence of microvascular damage in theese clinically asymptomatic WD patients. DESIGN: Prospective study. METHODS: A total of 43 eyes from 43 WM patients (24 females, 19 males, mean age 55.1 ± 13.6 years) were enrolled from January 2023 to December 2023 in the Eye Clinic of the University of Naples Federico II. Along with WM patients, 40 healthy subjects (HS) (20 females, 20 males, mean age 52.3 ± 15.6 years) with a normal ophthalmic examination and no history of intraocular surgery or retinal pathologic features were included as control group All patients and controls underwent OCTA RESULTS: The two groups were not significantly different for age and sex Visual acuity examination showed no statistically significant difference in BCVA between controls and patients Compared to HS, WD patients showed lower VD values in the SCP in the whole image (47.95 ± 5.17% vs. 52.99 ± 2.52 %; p < 0.001), as well as in the parafovea (53.01 ± 6.69% vs. 55.30 ± 2.61 %; p = 0.002), and fovea (21.38 ± 9.01% vs. 30.31 ± 5.84 %; p < 0.0001). On the other hand, in the DCP VD values were significantly higher in patients compared to controls in the whole image (55.82 ± 8.07% vs. 50.83 ± 5.46 %; p = 0.005), as well as in the parafovea (56.76 ± 6.26% vs. 52.59 ± 5.46 %; p = 0.0001), and fovea (38.75 ± 8.59% vs. 33.43 ± 8.68 %; p < 0.0001). CONCLUSION: The finding that OCTA confirmed the presence of widespread microvascular damage in WD patients clinically silent. Thus, OCTA is a safe rapid imaging technique that could represent a valid biomarker of systemic vascular dysfunction.

Primary cutaneous B cell lymphoma, leg type presenting as a diabetic ulcer: A challenging diagnosis.

INTRODUCTION: A crescent number of reports describe malignant dermal malignancies presenting as diabetic ulcers, such as melanoma, Kaposi's sarcoma, squamous cell carcinoma and cutaneous lymphoma. METHODS: The authors reported the clinical and histopathological features of this challenging case of a PCBCL, leg type presenting as a foot ulcer to exemplify the diagnostic difficulties, mainly when, at the onset, this tumour exhibits uncharacteristic features. CASE REPORT: A 43 years-old male with a 10-year history of compensated type I diabetes developed an ulcerated 3 cm of diameter tumour on the lateral region of the right foot. This lesion had previously been biopsied and treated as a diabetic neuropathic ulcer elsewhere. Due to the appearance of intralesional necrosis associated with stable inflammation and diabetes laboratory parameters, the clinicians made a provisional clinical diagnosis of pyoderma gangrenosum and performed further two incisional biopsies. Histology showed a clear-cut PCBCL, leg type. CONCLUSIONS: Diabetic skin lesions, especially in older patients with persistent non-healing characteristics of pain and tenderness, must be carefully managed through the close correlation of clinical, imaging, and histological features. A correct diagnosis allows avoiding inadequate treatment, which would lead to severe consequences for these patients.

Prognostic value of Bcl2 and Bcl6 in primary cutaneous large B-cell lymphoma: A systematic review and meta-analysis.

AIMS: To assess the prognostic value of Bcl2 and Bcl6 in primary cutaneous diffuse large B-cell lymphoma (pcDLBCL), through a systematic review and meta-analysis. METHODS: Electronic databases were searched from their inception to April 2021 for studies reporting Bcl2 and Bcl6 expression and survival outcomes in pcDLBCL series. Kaplan-Meier and Cox regression survival analyses with hazard ratio calculation were performed for overall survival (OS), with a significant p-value< 0.05. RESULTS: Eight studies with 148 patients were included. OS was significantly decreased in Bcl2-pos itive pcDLBCLs (5-year OS= 52.9 ± 5.2%) compared to Bcl2 negative pcDLBCLs (5-year OS= 86.6 ± 7.2%), with a HR of 4.615 (95% CI, 1.827-11.657; p = 0.001); no significant difference in OS was found between Bcl6-positive pcDLBCLs (5-year OS= 61.3 ± 6.5%) and Bcl6-negative pcDLBCLs (5-year OS= 56.8 ± 7.2%), with a HR of 0.789 (95% CI, 0.462-1.350; p = 0.388). CONCLUSIONS: In pcDLBCL, Bcl2 expression is a strong unfavourable prognostic marker; Bcl6 does not seem to be associated with survival instead. Further studies are necessary in this field.

A Novel Acceleration Signal Processing Procedure for Cycling Safety Assessment.

With the growing rate of urban population and transport congestion, it is important for a city to have bike riding as an attractive travel choice but one of its biggest barriers for people is the perceived lack of safety. To improve the safety of urban cycling, identification of high-risk location and routes are major obstacles for safety countermeasures. Risk assessment is performed by crash data analysis, but the lack of data makes that approach less effective when applied to cyclist safety. Furthermore, the availability of data collected with the modern technologies opens the way to different approaches. This research aim is to analyse data needs and capability to identify critical cycling safety events for urban context where bicyclist behaviour can be recorded with different equipment and bicycle used as a probe vehicle to collect data. More specifically, three different sampling frequencies have been investigated to define the minimum one able to detect and recognize hard breaking. In details, a novel signal processing procedure has been proposed to correctly deal with speed and acceleration signals. Besides common signal filtering approaches, wavelet transformation and Dynamic Time Warping (DTW) techniques have been applied to remove more efficiently the instrument noise and align the signals with respect to the reference. The Euclidean distance of the DTW has been introduced as index to get the best filter parameters configuration. Obtained results, both during the calibration and the investigated real scenario, confirm that at least a GPS signal with a sampling frequency of 1Hz is needed to track the rider's behaviour to detect events. In conclusion, with a very cheap hardware setup is possible to monitor riders' speed and acceleration.

Involvement of Helicobacter Pylori in Ocular Adnexa Lymphoma.

Helicobacter pylori has been proposed as a possible etiologic factor of ocular adnexa lymphoma (OAL), although with conflicting results. To assess the involvement of H. pylori in OAL, as (1) H. pylori DNA positivity on OAL specimens, and (2) prevalence of H. pylori gastric infection in patients with OAL. A systematic review of studies assessing H. pylori in patients with OAL was conducted by searching electronic databases from their inception to May 2019. Pooled positivity for H. pylori in OAL specimens detected by polymerase chain reaction, and pooled prevalence of H. pylori gastric infection, were calculated with 95% confidence interval (CI). Eleven studies with 308 patients were included. Pooled positivity for H. pylori was 16.8% in all OALs and 22.7% in MALT OAL, with high heterogeneity among studies. Pooled prevalence of H. pylori gastric infection in patients with OAL was 34.7%, with low statistical heterogeneity. In conclusion, H. pylori seems to be involved in a subset of OAL, but the heterogeneity found needs to be investigated in further studies. The prevalence of H. pylori gastric infection in patients with OAL does not seem to differ from that of the general population.

Clinical features associated with high pathological grade in primary thyroid lymphoma.

This study aimed to identify clinical features associated with high pathological grade (HG) in primary thyroid lymphoma (PTL), through a systematic review and meta-analysis. Electronic databases were searched for studies assessing PTL. Extracted data were age, sex, lymph node involvement, symptoms, lymphocytic thyroiditis, serum tumor markers; the association with HG was assessed by using odds ratio (OR), with a significant p-value<0.05. Twenty-four studies with 1916 PTLs were included. HG was significantly associated with age≤55 (OR = 0.54; p < 0.0001); female sex (OR = 1.31; p = 0.02), lymph node involvement (OR = 2.23; p < 0.00001), compressive symptoms (OR = 2.61; p < 0.0001), absence of lymphocytic thyroiditis (OR = 0.45; p = 0.0002), and increased LDH levels (OR = 4.90; p < 0.00001), but not with age>60 (OR = 0.74; p = 0.24), age>70 (OR = 1.16; p = 0.66), and B symptoms (OR = 1.30; p = 0.54). In conclusion, Age≤55, female sex, lymph node involvement, compressive symptoms and absence of lymphocytic thyroiditis may be predictive factors for HG in PTL. On the other hand, B symptoms does not seem to be associated with pathological grade.

Dysplastic bone marrow changes during maintenance therapy for acute leukemia.

We describe the case of an 8-year-old girl with common precursor B-cell acute lymphoblastic leukemia who presented with severe pancytopenia during maintenance therapy with methotrexate and 6-mercaptopurine. The bone marrow smear showed moderate hypocellularity and trilinear dysplastic changes consistent with a diagnosis of drug toxicity, with no evidence of lymphoblasts. Flow cytometric immunophenotyping was negative for leukemic cells. Blood cell counts normalized after treatment with folinic acid. Maintenance therapy was gradually restarted and she remained well at follow-up visits. Myelotoxicity from methotrexate and 6-mercaptopurine may represent an unpredictable incident during an otherwise uneventful maintenance therapy, and may occur independently of other organ toxicities.

Allogeneic transplantation in multiple myeloma / Transplante alogênico no mieloma múltiplo

In this review the authors present a state of art tretment of multiple myeloma.High dose chemo-radiotherapy followed by autologous hematopoietic stem cell transplantation has been show to be superior a conventional chemotherapy and a double transplantation. The authors discuss too, the allogeneic transplantation with reduced intensity conditioning, allogeneic versus tandem autologous, results the patients long term outcome and a approach about the use of donor lymphocytes, anti thimocyte globulin and a overview of post transplant therapies.

Neste relato os autores apresentam uma revisão sobre o estado atual do tratamento mieloma múltiplo. São enfatizados aspectos sobre a vantagem do transplante autólogo em seguimento à quimioterapia convencional e o duplo transplante. São discutidos o transplante alogênico e o condicionamento com intensidade reduzida, além do uso de linfócitos do doador, da globulina antitimocítica e uma visão geral do futuro da terapia da moléstia.

Efficacy of imatinib mesylate in a patient with idiopathic hypereosinophilic syndrome and severe heart involvement.

Idiopathic hypereosinophilic syndrome (HES) is a rare, chronic hematological disease mainly characterized by unexplained prolonged eosinophilia, with frequent evidence of secondary organ damage. Treatment with steroids, chemotherapy, interferon-alpha (IFN-alpha), or imatinib-mesylate may improve the prognosis. Here we describe the case of a young male patient with a six-year history of HES and severe heart involvement who, after unsuccessful treatment attempts with steroids, hydroxyurea and IFN-alpha, had a prompt, clinical and hematological complete remission following administration of imatinib. As his cardiac function also markedly improved, he was considered for heart transplant. However, seven years after the onset of the disease and four months after the termination of imatinib treatment the patient died of a cerebral hemorrhage that occurred during an episode of acute respiratory sepsis. Imatinib has been previously reported to be effective in some hematological conditions with no evidence of the BCR/ABL transcript. The mechanisms that are probably involved in the response to imatinib in HES are also discussed.