Primary cutaneous lymphomas (CLs) are a heterogeneous group of lymphoproliferative neoplasms, with lymphatic proliferation limited to the skin with no involvement of lymph nodes, bone marrow or viscera at the diagnosis. Cutaneous lymphomas originate from mature T-lymphocytes (65% of all cases), mature B-lymphocytes (25%) or NK cells. Histopathological evaluation including immunophenotyping of the skin biopsy specimen is the basis of the diagnosis, which must be complemented with a precise staging of the disease and identification of prognostic factors, to allow for the choice of the best treatment method as well as for the evaluation of the treatment results.
Sézary syndrome (SS) is an aggressive, leukemic cutaneous T-cell lymphoma variant. Molecular pathogenesis of SS is still unclear despite many studies on genetic alterations, gene expression and epigenetic regulations. Through whole genome and transcriptome next generation sequencing nine Sézary syndrome patients were analyzed in terms of copy number variations and rearrangements affecting gene expression. Recurrent copy number variations were detected within 8q (MYC, TOX), 17p (TP53, NCOR1), 10q (PTEN, FAS), 2p (DNMT3A), 11q (USP28), 9p (CAAP1), but no recurrent rearrangements were identified. However, expression of five genes involved in rearrangements (TMEM244, EHD1, MTMR2, RNF123 and TOX) was altered in all patients. Fifteen rearrangements detected in Sézary syndrome patients and SeAx resulted in an expression of new fusion transcripts, nine of them were in frame (EHD1-CAPN12, TMEM66-BAIAP2, MBD4-PTPRC, PTPRC-CPN2, MYB-MBNL1, TFG-GPR128, MAP4K3-FIGLA, DCP1A-CCL27, MBNL1-KIAA2018) and five resulted in ectopic expression of fragments of genes not expressed in normal T-cells (BAIAP2, CPN2, GPR128, CAPN12, FIGLA). Our results not only underscored the genomic complexity of the Sézary cancer cell genome but also showed an unpreceded large variety of novel gene rearrangements resulting in fusions transcripts and ectopically expressed genes.
IntroductionAtopic dermatitis (AD) is a chronic and relapsing skin disorder, which is characterized by abnormal skin barrier function within the entire skin surface. Several noninvasive bioengineering methods have been commonly used to quantify disease severity. High-frequency ultrasonography (HF-USG) is an important contribution to this field.AimTo evaluate noninvolved skin during the external treatment in relation to involved regions in patients with AD skin using noninvasive methods.Material and methodsTransepidermal water loss (TEWL), capacitance and erythema assessment and HF-USG were performed in 55 AD patients within 2 regions (involved and uninvolved skin) before and after therapy. The clinical severity of the disease process was based on the eczema area and severity index (EASI) score. A control group consisting of 15 subjects was also included.ResultsOn the basis of 4 bioengineering methods our study revealed that uninvolved skin in AD presents subclinical disturbances and significantly changes during therapy. The HF-USG detects inflammation in the upper dermis in AD patients in the form of a hypoechoic band, which may also be observed to a lesser extent within normal-appearing skin.ConclusionsNonlesional skin differs significantly from lesional skin in AD and from skin of healthy subjects. Noninvasive methods are able to measure subclinical skin disturbances within normal-appearing skin, which are not evaluated using standard clinical scores. They are objective and may facilitate communication between different research groups.
IntroductionRadiotherapy (RT) in combination with chemotherapy is a standard of care for patients with head and neck squamous cell carcinoma (HNSCC). The RT is associated with side effects, which impact on quality of life (QoL). Thus, the aim of this prospective longitudinal study was to investigate the impact of RT on the QoL of patients with HNSCC during RT.Material and methodsFrom September 2008 to February 2010, 205 patients with locally advanced HNSCC were enrolled. The data pertaining to their QoL were collected using the EORTC QLQ-C30 and the EORTC Head and Neck Module (QLQ-H&N35) and then all items were transformed to a 0-100 scale according to the guidelines of the EORTC. The following clinical factors were chosen to study their potential influence on the QoL; site of primary, clinical stage, and methods of therapy: RT vs. chemoradiotherapy (CRT). Additionally, the sociodemographic factors (age, gender, education, habit of smoking) were studied.ResultsDeterioration of almost all scales and items in the QLQ-C30 and QLQ-H&N35 questionnaire were noted at the end of RT. The following factors negatively influenced the QoL: age < 60 years (p < 0.05), female gender (p < 0.05), habit of smoking (p < 0.01), advanced clinical stage (III and IV) (p < 0.05), site of primary (larynx, hypopharynx) (p < 0.01), and CRT (p < 0.01).ConclusionsOur study showed that RT significantly negatively influenced QoL at the end of the RT course. Additionally, this study demonstrated that age, gender, smoking habit, tumor site, and clinical stage of disease showed a significant effect on the QoL of HNSCC patients during RT.
A b s t r a c t I In nt tr ro od du uc ct ti io on n: : Atopic dermatitis (AD) is an inflammatory pruritic dermatosis, which is characterized by an impaired skin barrier function manifested as an increased transepidermal water loss (TEWL). A Ai im m: : Presentation results of instrumental evaluation of the skin barrier function in AD patients in relation to the disease severity as well as pruritus. M Ma at te er ri ia al l a an nd d m me et th ho od ds s: : Fifty-five AD patients aged from 8 to 60 years were enrolled to the study. Epidermal barrier function (TEWL measurement and corneometry) as well as erythema measurement were determined in each patient within the same affected skin region, antecubital fossa. Clinical evaluation was performed using the W-AZS scoring system. R Re es su ul lt ts s: : There was a statistically significant difference in the mean TEWL values between groups of patients with different degrees of skin inflammation expressed by the W-AZS index, while no significant differences were observed in relation to the skin hydration and ertyhema measurement. There was a statistically significant correlation between the W-AZS index and TEWL as well as between the W-AZS I index and TEWL. C Co on nc cl lu us si io on ns s: : Selected parameters of the skin barrier function (TEWL, skin hydration) and the level of erythema are useful in evaluation of AD. Transepidermal water loss measurement presents a good correlation with W-AZS and with intensity of patients itching. Instrumental assessment provides us with a fast and objective evaluation of the eczema status, what seems to be very important in the era of evidence-based medicine.K Ke ey y w wo or rd ds s: : atopic dermatitis, skin barrier, transepidermal water loss.
This preliminary report indicates that UVA1 phototherapy might be an efficient treatment for early-stage MF.
The aim of the present study was to assess the effectiveness of UVA1 and PUVA therapy in treating patients with mycosis fungoides (MF) and to evaluate high-frequency ultrasonography (HF-USG) to monitor the clinical response of these patients. A total of 18 patients diagnosed with MF (stages I-IIA) underwent phototherapy, either UVA1 (6 cases) or PUVA (12 cases). Clinical response was evaluated according to modified Severity Weighted Assessment Tool (mSWAT) criteria and HF-USG (20 MHz). In the PUVA group, 50% of patients (6/12) achieved complete remission (CR) versus 33% (2/6) of patients in the UVA1 group. Before treatment, all subjects (100%) presented a subepidermal low echogenic band (SLEB) on HF-USG in the lesional skin. After phototherapy, the SLEB decreased significantly in all cases, with complete disappearance in 66% of cases. SLEB thickness was associated with disease severity and was wider in stage IIA patients than in stage IA and IB. These findings demonstrate that skin ultrasonography can be used to monitor treatment response in these patients. Moreover, HF-USG can quantify response, thus providing an objective measure of response that closely corresponds to scoring systems such as mSWAT used in routine clinical practice.
Introduction High‐frequency ultrasonography (HF‐USG) is a noninvasive method used in evaluation of depth and width of skin neoplasms. Recent data suggest that this method may also supplement objective clinical assessment in skin lymphomas, especially in mycosis fungoides, where subepidermal low echogenic band (SLEB) can be observed. The aim of the study was to present characteristic ultrasonic picture of MF in relation to histopathologic findings. Materials and methods Ten patients diagnosed as MF were included in the study. The USG examination was performed with the use of 20 MHz transducer within representative plaque. From the scanning lesion, the skin biopsy was taken. The relationship between histopathologic infiltrate with clonal T cells and USG image was investigated. Results In all analyzed sonograms obtained from lesional skin of early‐stage MF, we could detect the presence of subepidermal low echogenic band (SLEB). We detected strong correlations between SLEB thickness and the thickness of subepidermal infiltration (0.994, P < 0.05). Conclusions Subepidermal low echogenic band is a typical sign of infiltrative stage of MF, and its thickness may depend on the type of skin lesion. HF‐USG may be a reliable noninvasive method of quantitive assessments in MF, which corresponds to the thickness on T‐cell infiltration in histopathology.
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