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American Journal of Ophthalmology Case... Mar 2024To report a case of bilateral peripheral ulcerative keratitis (PUK) in a patient with underlying Sézary syndrome.
PURPOSE
To report a case of bilateral peripheral ulcerative keratitis (PUK) in a patient with underlying Sézary syndrome.
OBSERVATIONS
A 58-year-old male presented with bilateral corneal ulceration with stromal thinning and was diagnosed with PUK. He was actively being treated for Sézary syndrome, a cutaneous T-cell lymphoma. He had no lagophthalmos or other adnexal abnormalities that would lead to ocular surface breakdown. A systemic autoimmune and infectious workup for PUK was unremarkable. His keratitis resolved after treatment with oral prednisone.
CONCLUSIONS AND IMPORTANCE
We describe a previously undocumented association of PUK with Sézary syndrome in a patient without adnexal disease.
PubMed: 38235438
DOI: 10.1016/j.ajoc.2023.101990 -
Journal of Cutaneous Medicine and... 2024Skin diseases have been shown to worsen psychological distress, which, in turn, may be detrimental to treatment outcomes. Both the impact of psychological distress on...
BACKGROUND
Skin diseases have been shown to worsen psychological distress, which, in turn, may be detrimental to treatment outcomes. Both the impact of psychological distress on response to treatment in mycosis fungoides (MF) and the effect of treatments on psychological well-being are unclear.
OBJECTIVES
To evaluate (1) the association between pretreatment psychological morbidity and treatment outcome in early-stage MF and (2) the impact of response to treatment on psychological well-being.
METHODS
This was a prospective cohort study of patients with early-stage MF who started a new stage-directed treatment for their disease. The response was determined using the modified severity-weighted assessment tool, and psychological distress was assessed using the 12-item General Health Questionnaire (GHQ-12) and Penn State Worry Questionnaire (PSWQ). Participants were followed for 1 year.
RESULTS
In all, 24 consecutive patients were recruited. Objective response rate was 71% (17/24), consistent with existing literature. Prior to treatment, 9 patients (38%) had clinically significant psychological distress on the GHQ-12, while 8 (33%) demonstrated high-level worry on the PSWQ. Of these patients, 6 had pathologic scores on both instruments. Patients with significantly less baseline anxiety/depression on the GHQ-12 responded better to treatment than patients with higher levels ( = .004). In addition, responders' mean GHQ-12 scores decreased by 39% and their PSWQ scores by 17%, whereas nonresponders' GHQ-12 scores increased by 93% ( = .042) and their PSWQ scores by 11% ( = .019).
CONCLUSIONS
These findings suggest that (1) baseline psychological distress is associated with worse outcomes in patients with early-stage MF and that (2) effective treatment improves psychological morbidity.
Topics: Humans; Prospective Studies; Treatment Outcome; Mycosis Fungoides; Psychological Distress; Skin Neoplasms
PubMed: 38229268
DOI: 10.1177/12034754231220913 -
Cureus Dec 2023Juvenile dermatomyositis (JDM) is a chronic autoimmune inflammatory disorder and is considered the most common form of idiopathic inflammatory myopathies. JDM primarily...
Juvenile dermatomyositis (JDM) is a chronic autoimmune inflammatory disorder and is considered the most common form of idiopathic inflammatory myopathies. JDM primarily affects the skin and the skeletal muscles. Characteristic signs and symptoms include Gottron papules, heliotrope rash, calcinosis cutis, and symmetrical proximal muscle weakness. However, JDM presenting with generalized scaly poikeloderma is an unfamiliar presentation. Herein we report a 14-month-old female toddler presented with generalized progressive asymptomatic scaly mottled violaceous patches (poikilodermatous) that started when she was seven months old. Her lab results were unremarkable. She was diagnosed with poikilodermatous skin rash with a differential diagnosis of Amyopathic dermatomyositis, poikilodermatous genodermatosis, and patch-stage mycosis fungoides. She was prescribed moisturizer creams only. A year later, during a follow-up, she presented with a full picture of JDM, with a history of scaly poikilodermatous skin patches that became more widespread, frequent choking during oral intake, and not being able to stand and sit unsupported. Laboratory workup was significant for low WBC and hemoglobin counts, along with elevated CPK, LDH, ferritin, CRP, and ESR levels. MRI revealed the right anterior thigh and vastus lateralis subcutaneous edema. Therefore, the child was diagnosed and treated as a case of JDM.
PubMed: 38222200
DOI: 10.7759/cureus.50573 -
Frontiers in Immunology 2023Immunosequencing has emerged as a newer clinical test for assessment of T-cell clonality in the blood and skin of cutaneous T-cell lymphoma (CTCL) patients. Utilization... (Review)
Review
Immunosequencing has emerged as a newer clinical test for assessment of T-cell clonality in the blood and skin of cutaneous T-cell lymphoma (CTCL) patients. Utilization of immunosequencing, also known as high-throughput sequencing of the T-cell receptor (HTS-TCR), enables identification and quantification of the precise genetic signature of dominant T-cell clones. Although immunosequencing is more sensitive than commonly used methods such as polymerase chain reaction (PCR) paired with capillary electrophoresis or flow cytometry, it remains underutilized for CTCL management. Nonetheless, incorporation of HTS-TCR in clinical practice offers distinct advantages compared to other molecular analyses that may improve diagnostic evaluation, prognostication, and disease monitoring in CTCL. The objective of this comprehensive review is to provide a thorough explanation of the application of immunosequencing in the context of CTCL. We describe the significance of T-cell clonality and the methods used to detect it, including a detailed comparison between PCR paired with capillary electrophoresis and HTS-TCR. The utilization of immunosequencing in the blood and skin of CTCL patients is discussed in depth, specifically outlining how HTS-TCR can assist in diagnosing CTCL, predicting outcomes, and tracking disease progression. Finally, we address the potential applications of immunosequencing in clinical management and research as well as the novel challenges it presents.
Topics: Humans; Skin Neoplasms; Polymerase Chain Reaction; Lymphoma, T-Cell, Cutaneous; Skin; Receptors, Antigen, T-Cell
PubMed: 38213330
DOI: 10.3389/fimmu.2023.1300061 -
Cancers Jan 2024This study pioneers the application of artificial intelligence (AI) and hyperspectral imaging (HSI) in the diagnosis of skin cancer lesions, particularly focusing on...
This study pioneers the application of artificial intelligence (AI) and hyperspectral imaging (HSI) in the diagnosis of skin cancer lesions, particularly focusing on Mycosis fungoides (MF) and its differentiation from psoriasis (PsO) and atopic dermatitis (AD). By utilizing a comprehensive dataset of 1659 skin images, including cases of MF, PsO, AD, and normal skin, a novel multi-frame AI algorithm was used for computer-aided diagnosis. The automatic segmentation and classification of skin lesions were further explored using advanced techniques, such as U-Net Attention models and XGBoost algorithms, transforming images from the color space to the spectral domain. The potential of AI and HSI in dermatological diagnostics was underscored, offering a noninvasive, efficient, and accurate alternative to traditional methods. The findings are particularly crucial for early-stage invasive lesion detection in MF, showcasing the model's robust performance in segmenting and classifying lesions and its superior predictive accuracy validated through k-fold cross-validation. The model attained its optimal performance with a k-fold cross-validation value of 7, achieving a sensitivity of 90.72%, a specificity of 96.76%, an F1-score of 90.08%, and an ROC-AUC of 0.9351. This study marks a substantial advancement in dermatological diagnostics, thereby contributing significantly to the early and precise identification of skin malignancies and inflammatory conditions.
PubMed: 38201644
DOI: 10.3390/cancers16010217 -
Cancers Dec 2023Primary cutaneous T-cell lymphomas (CTCLs) are rare lymphoproliferative malignancies characterized by significant morbidity and mortality in advanced disease stages. As...
BACKGROUND
Primary cutaneous T-cell lymphomas (CTCLs) are rare lymphoproliferative malignancies characterized by significant morbidity and mortality in advanced disease stages. As curative approaches apart from allogeneic stem cell transplantation are lacking, establishing new treatment options, especially combination therapies, is crucial.
METHODS
This retrospective study included 11 patients with SS or MF receiving therapy with mogamulizumab in combination with ECP from four European expert centers. The response rates in the skin and blood as well as treatment use and adverse events (AE) were described.
RESULTS
8/11 patients (73%) showed an overall response (OR) in the skin. The mean mSWAT decreased from 98.2 ± 40.8 to 34.6 ± 23.8. The overall response rate (ORR) in the blood was 64% with two complete responses. During combination therapy, the mean number of Sézary cells decreased from 3365.3 × 10/L before treatment to 1268.6 × 10/L. The mean minimum known period without progress was 7.2 months in the skin and 7.6 months in the blood. The most common AEs were mogamulizumab-associated rash (MAR) (45.5%), anemia (27.3%), lymphocytopenia (27.8%), and infusion related reaction (16.7%). No AE led to treatment discontinuation.
CONCLUSIONS
Our study presents the combination of mogamulizumab and ECP as an effective therapy in the blood and skin in CTCL with good tolerability, similar to mogamulizumab monotherapy.
PubMed: 38201568
DOI: 10.3390/cancers16010141 -
Leukemia Research Reports 2024T-cell lymphomas are aggressive neoplasms characterized by poor responses to current chemotherapeutic agents. Expression of the l-type amino acid transporter 1 (LAT 1,...
T-cell lymphomas are aggressive neoplasms characterized by poor responses to current chemotherapeutic agents. Expression of the l-type amino acid transporter 1 (LAT 1, SLC7A5) allows for the expansion of healthy T-cell counterparts, and upregulation of LAT1 has been reported in precursor T-cell acute leukemia. Therefore, the expression of LAT1 was evaluated in a cohort of cutaneous and peripheral T-cell lymphomas. The findings demonstrated that LAT1 is upregulated in aggressive variants and absent in low-grade or indolent disease such as mycosis fungoides. In addition, upregulated LAT1 expression was seen in a large proportion of aggressive peripheral T-cell lymphomas, including peripheral T-cell lymphoma not otherwise specific (PTCL-NOS) and angioimmunoblastic T-cell lymphoma (AITL). The anti-tumor effects of two novel non-cleavable and bifunctional compounds, QBS10072S and QBS10096S, that combine a potent cytotoxic chemotherapeutic domain (tertiary N-bis(2-chloroethyl)amine) with the structural features of a selective LAT1 substrate (aromatic β-amino acid) were tested in vitro and in vivo in T-cell lymphoma cell lines. The findings demonstrated decreased survival of T-cell lymphoma lines with both compounds. Overall, the results demonstrate that LAT1 is a valuable biomarker for aggressive T-cell lymphoma counterparts and QBS10072S and QBS10096S are successful therapeutic options for these aggressive diseases.
PubMed: 38192502
DOI: 10.1016/j.lrr.2023.100398 -
Medicine Jan 2024Acute promyelocytic leukaemia (APL) is a rare subtype of acute myelogenous leukaemia. With advances in treatment regimens, namely, introduction of...
RATIONALE
Acute promyelocytic leukaemia (APL) is a rare subtype of acute myelogenous leukaemia. With advances in treatment regimens, namely, introduction of all-trans-retinoicacid, outcomes have drastically improved, its side effects should not be ignored. Mycosis fungoides is one of the side effects of all-trans-retinoicacid treatment, but it may also be a clinical manifestation before disease progression. However, it rarely appears and is easily overlooked. This leads to being easily misled during the treatment process, affecting the treatment plan, and resulting in adverse consequences. Therefore, early identification and judgment can not only provide appropriate treatment options, but also prevent and treat further disease progression.
PATIENT CONCERNS
The patient was hospitalized for pancytopaenia. After completing the examination, the patient was finally diagnosed with acute promyelocytic leukaemia (acute myelogenous leukaemia-M3). We administered tretinoin and arsenous acid. Evaluation of the treatment effect on the 7th day after chemotherapy showed that the bone marrow morphology showed complete remission. After the second course of chemotherapy, the patient developed red miliary macular papules, which gradually worsened. After completing relevant inspections, Considering that the cases was complicated with skin mycosis fungoides, the patient was treated with budesonide ointment and methylprednisolone as chemotherapy.
DIAGNOSES
Upon examination, the patient was initially diagnosed with acute promyelocytic leukaemia. Evaluation of the treatment effect on the 7th day after chemotherapy showed that the bone marrow morphology showed complete remission. After the second course of chemotherapy, we discovered the patient was diagnosed with skin mycosis fungoides.
INTERVENTIONS
Systemic chemotherapy is first given when a patient was diagnosed with acute promyelocytic leukaemia. After the patient happened skin mycosis fungoides, We have adjusted the treatment plan and supplemented it with other treatment plans based on the original chemotherapy, After 2 months of treatment, his condition gradually improved.
OUTCOMES
All-trans-retinoicacid in the treatment of APL must be given attention because mycosis fungoides should not only be distinguished from infectious diseases but also be further assessed with regard to disease progression and metastasis.
LESSONS
Acute promyelocytic leukemia needs to be treated with arsenic trioxide. All-trans-retinoicacid in the treatment of APL must be given attention mycosis fungoides. Early diagnosis can guide accurate treatment, which is of great help in alleviating the pain of patients and improving the cure rate.
Topics: Humans; Leukemia, Promyelocytic, Acute; Mycosis Fungoides; Skin; Dermatomycoses; Disease Progression; Skin Neoplasms
PubMed: 38181249
DOI: 10.1097/MD.0000000000036619 -
Frontiers in Medicine 2023Cutaneous adverse events of both topical and systemic drugs in patients with mycosis fungoides (MF) present a diagnostic challenge as it is often difficult to...
Cutaneous adverse events of both topical and systemic drugs in patients with mycosis fungoides (MF) present a diagnostic challenge as it is often difficult to distinguish drug associated rash from disease progression in the skin. Mogamulizumab and mechlorethamine gel are approved treatments for MF, both of which can cause treatment related cutaneous adverse events. It can often be challenging to distinguish mogamulizumab associated rash (MAR) and mechlorethamine gel associated hypersensitivity dermatitis from MF progression both clinically and histologically. High-throughput sequencing (HTS) of the T-cell receptor (TCR), also known as immunosequencing, can be used to assess T-cell clonality to support a diagnosis of MF. After identification of the malignant TCR clone at baseline, immunosequencing can track the established malignant TCR sequence and its frequency over time with high sensitivity. As a result, immunosequencing clone tracking can aid in distinguishing disease progression from treatment side effects. Here, we present a case series to demonstrate how monitoring of the malignant T-cell frequency by immunosequencing can aid in diagnosis of mogamulizumab and mechlorethamine gel cutaneous adverse events.
PubMed: 38164221
DOI: 10.3389/fmed.2023.1243459 -
Indian Journal of Cancer Apr 2023Primary extranodal lymphomas (pENL) are lymphomas with minimal nodal involvement and dominant extranodal disease. We aimed to study the prevalence and...
BACKGROUND
Primary extranodal lymphomas (pENL) are lymphomas with minimal nodal involvement and dominant extranodal disease. We aimed to study the prevalence and clinicopathological characteristics of pENL presenting at our center over 5 years from January 2015 to January 2020.
METHODS
This is a cross-sectional study of pENL patients in which relevant clinical and laboratory data was collected including demography, site, stage, international prognostic index-revised, imaging findings, hematological, and biochemical parameters and comorbidities including underlying immunodeficiency. The paraffin blocks were subjected to routine hematoxylin and eosin stain and immunohistochemistry with standard lymphoma panel.
RESULTS
Of 341 lymphomas, 73 (21.4%) were pENL with commonest site being gastrointestinal tract (31.5%) followed by head and neck (23.2%) and soft tissues (9.6%). Diffuse large B-cell lymphoma (DLBCL) (39.7%) was the commonest histological type (germinal center type-48%, nongerminal center-52%) followed by marginal zone lymphomas (MZL) (23.3%) and primary CNS lymphoma (8.2%). Primary breast lymphoma, primary bone marrow lymphoma, and follicular lymphoma constituted 4.1, 5.4, and 4.1% of pENL, respectively. There was a case of high grade B cell lymphoma of ileum with features intermediate between DLBCL and Burkitt. Other unusual pENL were anaplastic DLBCL of tonsils, DLBCLs of bone marrow with M band, MZL of base of tongue, Richter's transformation of tonsillar small lymphocytic lymphoma, and follicular lymphoma presenting as pericardial mass. Of 12 cases of T-non-Hodgkin lymphoma, commonest were mycosis fungoides (4/12) followed by mediastinal T-lymphoblastic lymphoma (2/12) and peripheral T-cell lymphoma (2/12).
CONCLUSION
pENL has unique clinical presentation depending on the location with site-specific distribution of histological subtypes.
PubMed: 38155455
DOI: 10.4103/ijc.IJC_1267_20