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Poikilodermatous mycosis fungoides is a rare variant of cutaneous T-cell lymphoma that is often misdiagnosed given its diverse clinical presentation. Often diagnosed as vitiligo or morphea, poikilodermatous mycosis fungoides can be asymptomatic or present as pruritic lesions. Discrepant signs and symptoms can lead to diagnostic delays. No consensus on its treatment currently exists, but treatment options include corticosteroids, phototherapy, and radiation. Here, we present a case of poikilodermatous mycosis fungoides in an otherwise healthy young man who showed limited improvement after years of treatment with topical antifungals, topical steroids, and phototherapy. Improvement was seen following a single session of radiation therapy, highlighting radiations potential in cases resistant to traditional first-line treatments. We propose that radiation may be efficacious for the treatment of poikilodermatous mycosis fungoides in cases of delayed diagnosis or resistance, and further research is needed to investigate radiation monotherapy as a treatment option for poikilodermatous mycosis fungoides.

Background Methods Results Conclusions Primary cutaneous B‐cell lymphomas (PCBCLs) are rare cutaneous neoplasms with limited literature regarding treatment options and associated treatment outcomes. This study aimed to investigate and present real‐world treatment outcomes in patients with PCBCLs.All patients with PCBCL who were treated in five major referral centers for cutaneous lymphoma in Greece over 10 years were retrospectively included with their baseline characteristics and treatment‐associated outcomes collected and analyzed.In total, 235 PCBCL patients, of whom 125 (53.2%) were females, were included. The median age at diagnosis was 60 years (IQR 47–72), and the median follow‐up duration was 3 years (IQR 1.3–6.4). The most common subtype was primary cutaneous marginal zone lymphoma (PCMZL) (52.3%), followed by primary cutaneous follicle center lymphoma (PCFCL) (40.9%), and primary cutaneous large B‐cell lymphoma, leg type (PCDLBCL, LT) with 16 (6.8%) cases. Complete responses (CRs) were observed in 77.3% after first‐line treatment. Both radiotherapy (RT) and surgical excision (SE) achieved superior outcomes, with 88.9% and 89% achieving CR, respectively. Relapses occurred in 22.6% of initial complete responders. The median time to the next treatment (TTNT) for the PCMZL and PCFCL was 349 days. RT and topical/intralesional steroids demonstrated longer TTNT compared to SE (445 and 359 vs. 154 days). For PCMZL and PCFCL, the 1‐year progression‐free survival (PFS) was 84.2% (75.7–89.9) and 85% (75.1–91.2), and the 5‐year PFS was 66.5% (55.2–75.5) and 58.8% (44.4–70.7), respectively.PCBCLs have favorable outcomes. RT demonstrates significantly increased TTNT compared to SE, suggesting RT as the preferable option. After careful evaluation, “watch and wait” may be a reasonable option for asymptomatic patients. [ABSTRACT FROM AUTHOR]

Background and Design: Primary cutaneous B-cell lymphomas (PCBCLs) have not been well characterized due to their relatively low incidence and heterogeneous clinical features. Materials and Methods: Data of 29 patients with primary cutaneous marginal zone lymphoma (PCMZL) (n=18), primary cutaneous follicle center B-cell lymphoma (PCFCL) (n=7), and primary cutaneous diffuse large B-cell lymphoma-leg type (PCDLBCL-LT) (n=4) who were followed and treated at Ankara University Faculty of Medicine between 2006 and 2022 were retrospectively evaluated. Clinical characteristics, treatment modalities, and outcomes of all patients were analyzed. Results: Our study supports the data that there are geographical differences in the distribution of B-cell lymphomas and that PCMZL is predominant in Asian countries compared to Europe. The mean age at the time of diagnosis in the PCDLBCL-LT group was significantly older than the indolent PCBCL group (p=0.022). The lesion size was predominantly less than 2 cm in PCMZL, between 2 and 5 cm in PCFCL, and larger than 5 cm in the PCDLBCL-LT group (p=0.006). While skin-directed treatments were the most common approach in the PCMZL and PCFCL groups, multi-agent chemotherapy was the preferred treatment in the PCDLBCL-LT group (p=0.003 and p=0.001, respectively). No significant correlation was found between age, gender, clinical features of the lesion, cutaneous lymphoma international prognostic index score, stage and treatment modalities, recurrence, and overall survival. The World Health Organization and European Organization for Research and Treatment of Cancer classification of cutaneous lymphoma remains the primary guideline for understanding the clinical behavior and prognostic parameters of this heterogeneous patient group. Conclusion: Given the relative rarity of these types of lymphoma, our patient collective provides an additional value to the existing literature. [ABSTRACT FROM AUTHOR]

Background: Hypopigmented mycosis fungoides (HMF) is a recognized variant of Mycosis fungoides (MF) that resembles several inflammatory dermatoses. Clinical and histopathological features of HMF must be well-characterized to ensure early recognition, especially that such information is lacking in Jordan. Methods: We retrospectively reviewed medical records of 10 HMF patients, from 2015 to 2022. Clinical and histopathological features were extracted, reviewed, and summarized. Results: Our cohort comprised 6 males and 4 females. Male: female ratio 3:2. Mean age at diagnosis was 21.2. The most common location was the trunk. Half reported mild itching. All were treated with NB-UVB, 90% achieved a complete response to treatment, one lost follow up. All biopsy specimens displayed hyperkeratosis, epidermal hyperplasia, mild superficial dermal perivascular lymphoid infiltrate, and disproportionate spongiosis. Lining of atypical lymphocytes at the dermal-epidermal junction was encountered in 90% and papillary dermal fibrosis in 60%. Immunohistochemical staining was performed on 7 specimens, with 57.1% CD8 predominance and 42.8% CD4 predominance. Conclusion: HMF in the Jordanian population shares histopathological and clinical features with published reports. Our report emphasizes the importance of obtaining a skin biopsy when considering HMF. A common theme was the variability in latency periods before diagnosis emphasizing the delay in diagnosis. Further data and techniques must be studied and integrated to ensure timely diagnosis. [ABSTRACT FROM AUTHOR]

Mycosis fungoides (MF) has been widely reported to mimick a considerable number of different dermatoses, including scarring alopecia, bullous dermatoses or cysts, and comedones. In atypical presentations, histopathology is essential for the diagnosis. We present two cases of MF with clinical urticarial lesions and a striking blood involvement that responded to mogamulizumab treatment. Histopathologically, both cases had classic MF features and shared a peculiar immunophenotype, with positivity for CD25 and FOXP3. Differential diagnoses included urticarial lymphomatoid drug reactions and other lymphomas, like T‐cell prolymphocytic leukemia, atypical Sézary syndrome, or adult T‐cell lymphocytic leukemia. A low suspicion threshold is necessary for the diagnosis of atypical presentations of MF. [ABSTRACT FROM AUTHOR]

Summary: The recently published 5th edition of the "World Health Organization classification of hematolymphoid tumors: lymphoid neoplasms" provides a hierarchical reorganization. In general, new (definitive) entities as well as tumor‐like lesions were included. Primary cutaneous B‐cell lymphomas (CBCL) received a thorough review. A new class/family of cutaneous follicle center lymphomas was defined. Primary cutaneous marginal zone lymphoma is now presented as a separate entity independent from extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue. In primary cutaneous T‐cell lymphoma, former provisional entities were upgraded to definite entities. Sézary Syndrome was sorted into the class/family of mature T‐cell and NK‐cell leukemias. Additionally, a newly formed entity of primary cutaneous peripheral T‐cell lymphoma, NOS was created for CTCL entities that do not fit into the already described CTCL entities. The increasing importance of genomic and molecular data has already been recognized in classifying leukemias and systemic lymphomas. However, in PCL the genomic landscape has not yet been fully described and validated. Therefore, future research is necessary to describe the genomic and molecular mechanisms underlying the disease entities more clearly. This would both meet a diagnostic need and valuably contribute to future classification schemes. [ABSTRACT FROM AUTHOR]

Background and Design: Primary cutaneous B-cell lymphomas (PCBCLs) have not been well characterized due to their relatively low incidence and heterogeneous clinical features. Materials and Methods: Data of 29 patients with primary cutaneous marginal zone lymphoma (PCMZL) (n=18), primary cutaneous follicle center B-cell lymphoma (PCFCL) (n=7), and primary cutaneous diffuse large B-cell lymphoma-leg type (PCDLBCL-LT) (n=4) who were followed and treated at Ankara University Faculty of Medicine between 2006 and 2022 were retrospectively evaluated. Clinical characteristics, treatment modalities, and outcomes of all patients were analyzed. Results: Our study supports the data that there are geographical differences in the distribution of B-cell lymphomas and that PCMZL is predominant in Asian countries compared to Europe. The mean age at the time of diagnosis in the PCDLBCL-LT group was significantly older than the indolent PCBCL group (p=0.022). The lesion size was predominantly less than 2 cm in PCMZL, between 2 and 5 cm in PCFCL, and larger than 5 cm in the PCDLBCL-LT group (p=0.006). While skin-directed treatments were the most common approach in the PCMZL and PCFCL groups, multi-agent chemotherapy was the preferred treatment in the PCDLBCL-LT group (p=0.003 and p=0.001, respectively). No significant correlation was found between age, gender, clinical features of the lesion, cutaneous lymphoma international prognostic index score, stage and treatment modalities, recurrence, and overall survival. The World Health Organization and European Organization for Research and Treatment of Cancer classification of cutaneous lymphoma remains the primary guideline for understanding the clinical behavior and prognostic parameters of this heterogeneous patient group. Conclusion: Given the relative rarity of these types of lymphoma, our patient collective provides an additional value to the existing literature.

Abstract Background Primary cutaneous follicle centre lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma (PCMZL) are indolent radiosensitive lymphomas, with national guidelines recommending 24 Grey (Gy) in 12 fractions, despite shorter regimens showing no difference in overall survival. Multiple studies demonstrate 4 Gy in two fractions is effective in other indolent lymphomas. Shorter regimens may result in less toxicity for patients. Objectives We aim to characterise local control rates in PCFCL and PCMZL treated with radiation therapy (RT) with 4 Gy in two fractions. Methods From a single institution database, we retrospectively identified all patients with PCFCL and PCMZL treated with 4 Gy in two fractions between 2011 and 2022. Prior topical or systemic treatments were permitted. T‐cell or high‐grade B‐cell cutaneous lymphomas, or those with less than 6 months follow‐up, were excluded. Primary outcomes were rates of response and local failure per lesion as determined by a radiation oncologist and oncologic dermatologist. Results Forty‐three consecutive patients with 98 lesions treated were identified. Twenty‐four (56%) of the patients had PCMZL, and the remainder had PCFCL. Most lesions (97%) received RT with electrons. The complete response or partial response rate was 94%. Six local failures were identified (6%). With a median follow‐up of 2.75 years, 1‐, 2‐ and 3‐year local failure‐free survival were 94%, 94% and 92%, respectively. There was minimal acute toxicity, no late toxicity and no cosmesis issues. No clinical features were predictive of local failure. Conclusions Ultra‐low dose radiation was associated with high rates of local control and minimal toxicity and may reduce personal burden and financial toxicity in appropriately selected patients.

Primary cutaneous gamma/delta (γδ) T‐cell lymphoma (PCGDTCL) is a rare, aggressive malignant neoplasm of γδ T lymphocytes arising within the skin and subcutis. We present a challenging case of PCGDTCL diagnosed in a 35‐year‐old male soldier who presented with constitutional symptoms, pancytopenia, hemophagocytic lymphohistiocytosis (HLH), disseminated lymphadenopathy, and cutaneous lesions on his extremities and back following a deployment to Iraq and Syria. Histopathologic evaluation of an excisional biopsy showed that PCGDTCL can be focal, localized to the subcutaneous adipose tissue, and obscured by predominant HLH in the surrounding tissues. Pathologists should recognize that the diagnosis of PCGDTCL may be confounded by florid HLH and require multiple biopsies and a comprehensive immunohistochemical panel. [ABSTRACT FROM AUTHOR]

AbstractThere are no established maintenance protocols for cutaneous lymphomas. We aim to determine patient treatments and outcomes during the COVID-19 pandemic in order to uncover the most effective maintenance protocols for cutaneous lymphomas and impact of treatment interruption. Data was collected retrospectively from nine international institutions, including 149 patients. Younger patients had earlier stages of disease and were most frequently treated with skin-directed therapies including topical steroids, mechlorethamine gel, and phototherapy. Treatment interruption varied by treatment type and stage, with patients on topical therapies and earlier stages of disease being least likely to experience interruption. Treatment interruption was significantly associated with progression of disease and worse outcomes, with twice as many patients progressing who had interruption compared to those without interruption. This study may demonstrate the significance of continuous maintenance therapies, even in younger patients with early stages of disease. [ABSTRACT FROM AUTHOR]

Background Objective Methods Results Conclusion Primary cutaneous CD4+ small/medium T‐cell lymphoproliferative disorder (PCSM‐LPD) is an increasingly recognized entity with heterogeneous management strategies that may include radiotherapy.Our aim was to characterize treatment options for PCSM‐LPD, with a focus on the role of radiotherapy.This is a retrospective review of 46 patients seen in the Cutaneous Lymphoma Program at the University of Texas MD Anderson Cancer Center, with a clinicopathologic review consistent with PCSM‐LPD. All patients were biopsied and underwent observation, topical/intralesional steroids, and/or radiotherapy. Patients were confirmed to have residual disease prior to radiotherapy.All patients achieved a complete response (CR). Sixteen patients (35%) received focal radiotherapy, with a CR in 15 (94%). The CR rate following ultra‐low‐dose radiotherapy (4 Gy in 1–2 fractions) was 92%. There was no grade 3 toxicity after radiotherapy. Thirty patients were managed without radiotherapy, with excision and observation or steroids.Primary cutaneous CD4+ small/medium T‐cell lymphoproliferative disorder has excellent outcomes, and management strategies may include observation following biopsy, steroids, or radiation. Ultra‐low‐dose radiotherapy results in excellent outcomes with limited toxicity and is effective for persistent lesions after steroidal therapy. [ABSTRACT FROM AUTHOR]

Copyright of Conocimiento Enfermero is the property of Colegio Oficial de Enfermeria de Madrid and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Epitheliotropic cutaneous T‐cell lymphoma (ETCL) is rarely described in golden or Syrian hamsters (Mesocricetus auratus), and no effective treatments or outcomes have previously been published. This study includes two cases of epitheliotropic cutaneous T‐cell lymphoma that were diagnosed via skin biopsy. Clinical presentation, diagnosis, treatment and outcome were reported. Both cases received chemotherapy, including prednisolone, lomustine, L‐asparaginase and chlorambucil. One Syrian hamster that received prednisolone (0.5 mg/kg per os every 24 hours) and chlorambucil (0.4 mg/kg per os every 24 hours) survived 276 days after diagnosis. Another Syrian hamster treated with prednisolone (1 mg/kg per os every 24 hours) survived 81 days after diagnosis. The effectiveness of lomustine and L‐asparaginase remains unknown for Syrian hamsters, and more studies are needed to determine appropriate dose ranges for chlorambucil. The combination of chlorambucil (0.4 mg/kg per os every 24 hours) and prednisolone (0.5–1 mg/kg per os every 24 hours) may assist in managing epitheliotropic cutaneous T‐cell lymphoma in Syrian hamsters. [ABSTRACT FROM AUTHOR]

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This article discusses a case of folliculotropic mycosis fungoides (FMF), a type of cutaneous lymphoma, that was successfully treated with the oral selective JAK1 inhibitor abrocitinib. The patient, a 46-year-old Chinese male, had previously tried interferon and phototherapy with minimal improvement. After adding abrocitinib to the treatment plan, there was significant improvement in the skin lesions and reduction in pruritus. The study suggests that selective JAK1 inhibitors may be a promising adjunct therapy for refractory FMF. [Extracted from the article]

Current treatment guidelines for cutaneous T cell lymphoma (CTCL) advocate a stage-driven approach, considering clinical presentation, symptom burden, and patient comorbidities. Therapy selection hinges on factors like disease subtype, severity, and treatment availability. The primary goal is to enhance the quality of life by mitigating symptoms, as achieving lasting complete remission is infrequent. Over the past decade (2013–2023), the therapeutic landscape of CTCL has experienced substantial transformation with the introduction of innovative therapies. This review explores the main pivotal developments in traditional treatment schedules and recently introduced drugs, aiming to offer clinicians and researchers a thorough perspective on the decade's progress in the field. Despite the progress made in CTCL therapeutics, ranging from topical chemotherapeutics to immunomodulatory agents, several unmet needs persist. Firstly, there is a pressing need for the incorporation of readily available predictors for treatment response, encompassing clinical, pathological, and molecular features. Secondly, a more profound comprehension of the tumor microenvironment is imperative to optimize the landscape of targetable molecules. Lastly, the undertaking of studies on combination regimens should be encouraged as it enhances therapy efficacies by synergistically combining agents with diverse modes of action. [ABSTRACT FROM AUTHOR]

Introduction and aim. Primary musculocutaneous lymphoma is extremely rare and associated with poor prognosis. Here, we present a case of refractory primary muscular lymphoma with overlying cutaneous involvement with the aid of 18F-Fluorodeoxyglucose positron emission tomography/computed tomography (18F FDG PET/CT) for staging and treatment. Description of the case. We present the case of a 52-year-old man presented with musculocutaneous Hodgkin's lymphoma having swelling and discolored areas over the left leg and right arm associated with itchiness. Upon examination, there was scaling, erythema over the body with Gottron's papules on knuckles and heliotrope rash. Skin and muscle biopsy were suggestive of Hodgkin's lymphoma. This patient was had primary musculocutaneous lymphoma. His diagnosis was delayed. PET/CT was performed for staging and response to treatment, which was suggestive of refractory disease. Conclusion. PET/CT plays a vital role in diagnosis, staging, response to therapy and helps in optimized treatment for these specific patients. [ABSTRACT FROM AUTHOR]

The association between Primary cutaneous CD4 small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) and COVID-19 immunization has been sparsely documented in the medical literature. Reviewing the literature, albeit infrequently, we can find cases of the recurrence and new onset of lymphoproliferative processes and cutaneous lymphomas following the COVID-19 vaccine. Many of the entities we encounter are classified as cutaneous lymphoproliferative disorders. The prevailing hypothesis suggests that the predominant cutaneous reactions to SARS-CoV-2 vaccines may stem from T-cell-mediated immune activation responses to vaccine components, notably messenger RNA (mRNA). Specifically, it is posited that the presence of cutaneous lymphoid infiltrates may be linked to immune system stimulation, supported by the absence, to date, of instances of primary cutaneous B-cell lymphoma following mRNA vaccination. Within this context, it is imperative to underscore that the etiological association between PCSM-TCLPD and COVID-19 vaccination should not discourage vaccination efforts. Instead, it underscores the necessity for continuous surveillance, in-depth investigation, and comprehensive follow-up studies to delineate the specific attributes and underlying mechanisms of such cutaneous manifestations post vaccination. [ABSTRACT FROM AUTHOR]

Simple Summary: This systematic review concludes that a higher neutrophil-to-lymphocyte ratio (NLR), a robust indicator of adverse prognosis across the common metastatic cancers including malignant melanoma, is also a promising biomarker of tumor aggressiveness in all advanced stage non-melanoma skin cancers. A distinct exception with still inconclusive evidence is the cutaneous T-cell lymphoma, although there are some data indicating that higher NLR may predict poorer prognosis in patients with earlier stages mycosis fungoides. Finally, given the promising data concerning the impact of NLR as a prognosticator of the outcome of the treatment with the current immunotherapies, there is presently a need for studies focusing on the role of NLR in the framework of the treatment of advanced non-melanoma skin cancer. With the ongoing progress of basic research along with the introduction of new pharmaceutical options spanning almost all therapeutic areas, the need for biomarkers that will be implemented into the personalized medical approach is higher than ever. Their use can be incorporated into clinical practice and can be applied to the classification of disorders and the evaluation of disease severity but also to the monitoring of the progress of therapeutic/pharmaceutical interventions. This systematic review collects the findings of hematologic biomarkers in various cutaneous malignancies, excluding malignant melanoma, to support their potential use in the prognosis but also in the assessment of therapeutic strategies for the specific category of skin disorders. [ABSTRACT FROM AUTHOR]

Cutaneous T-cell lymphomas (CTCL) are a rare group of T-cell neoplasms which infiltrate the skin and can result in substantial morbidity and mortality. Risk factors for CTCL are still poorly understood though recent studies suggest chemical exposures may play a role in its development. To further characterize patient-centered risk factors for CTCL, especially compared with matched controls, we performed one of the largest prospective cohort survey studies to date to examine patient-reported exposures and health-related quality of life (HRQoL) in association with concurrent clinical disease characteristics. Patient demographics, lifestyle factors, and chemical exposures were collected via clinical data and surveys. Descriptive statistics, ANOVA, Chi-square tests and t tests were utilized to compare patient-reported exposures and HRQoL in patients with CTCL versus matched healthy controls (HC). Statistically significant differences were identified between patients and HC in terms of race (non-white race 22.4% in CTCL patients vs. 18.8% in HC, P = 0.01), and education level (high school or less 41.6% in CTCL patients vs. 14.3% in HC, P = 0.001), but not with Fitzpatrick skin type (P = 0.11) or smoking status (P = 0.28). Notably, 36.0% of the CTCL patients reported exposures to chemicals, a near threefold increased percentage when compared to HC (12.9%). Among various chemical exposures, 27.0% of the CTCL patients specifically reported industrial chemical exposure, a more than two-fold increased percentage when compared to HC (12.9%). Itch and pain were significantly associated with skin disease severity (as evaluated by CTCL-specific mSWAT score) in advanced stage disease (stages IIB–IVB) (r = 0.48 and 0.57, P < 0.05). Itch and body mass index (BMI) were weakly associated with skin disease severity in early-stage disease (stages IA–IIA) (r = 0.27 and 0.20, P < 0.05). [ABSTRACT FROM AUTHOR]

Background: Patients facing a cutaneous lymphoma diagnosis frequently turn to the internet for information but finding patient-accessible education may be a challenge. Objective: To investigate accessibility and readability of patient-oriented online education on cutaneous lymphomas, including cutaneous T-cell and B-cell lymphoma subtypes. Methods: This study queried a search engine for 11 cutaneous lymphoma terms, resulting in 1083 webpages. Webpages were screened using defined inclusion/exclusion criteria; literature directed to physicians and scientists was excluded. Webpages were stratified by academic/nonacademic and dermatology/nondermatology hosts and assessed by order of appearance. Readability, including text complexity, was analyzed for grade level understanding using 5 established calculators. Overall readability was assessed by Flesch–Kincaid Reading Ease. Results: Academic webpages had earlier order of appearance. There was a dearth in dermatology-hosted webpages. Rarer cutaneous lymphomas yielded fewer patient-accessible results. Search term readability significantly exceeded the American Medical Association–recommended sixth grade level (P

Rare subtypes of cutaneous T-cell lymphomas (CTCL) include four entities, primary cutaneous γδ T-cell lymphoma, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, and primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders, primary cutaneous acral CD8+ T-cell lymphoma, which were previously considered provisional and are now included in the new 5th World Health Organization classification of hematolymphoid tumors as distinct entities. An updated summary of the clinical, histological, and genomic characteristics of these uncommon CTCL subtypes is given in this review, with a focus on the growing body of knowledge regarding their classification and possible treatment strategies. [ABSTRACT FROM AUTHOR]

Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare subtype of reactive histiocytosis which is seldom associated with Hodgkin's and non‐Hodgkin's lymphomas. To date, the coexistence in the same patient of extra nodal SHML and primary cutaneous B‐cell lymphoma (PCBCL) has been reported in the literature, as metachronous diagnosis in the anatomical area of the original PCBCL or synchronous occurrence in the same lesions. However, no data have been published as for synchronous occurrence of the two pathological entities in distinct anatomical sites. Herein, we report the first ever described synchronous occurrence of PCBCL and SHML, detected in distinct lesions, affecting the same patient. The complete resolution of the patient's PCBCL after rituximab treatment and the concomitant regression of SHML suggest that this clinically benign reactive histiocytic proliferation, potentially triggered by the lymphoma microenvironment itself, may take place not only in the site of the PCBCL lesion, but also in other distant areas not directly affected by the primary cutaneous lymphoma. [ABSTRACT FROM AUTHOR]

Individual reports described lymphoproliferative disorders (LPDs) after COVID-19 vaccination; however, the relationship between cases is unexamined. We aim to determine if there are cases of cutaneous LPDs associated with COVID-19 vaccination and their outcomes. We present a review of world literature, vaccine registries, and two unreported cases of LPDs after COVID-19 vaccination. Review of the medical literature, VAERS, and our two cases reveal predominance of Pfizer-BioNTech vaccine, younger patients, and males. All cases resulted in favorable outcomes. Approximately 84% of cases demonstrated CD30+ positivity in their skin biopsies, suggesting that an antigenic trigger may lead to a type IV adaptive immune response, with clonal expansion of CD30+ T-cells and subsequent oncogenic mutational hits eventuating in transient LPDs. LPDs after COVID-19 vaccination appear in the context of the same vaccines (proportionally to their global market shares), share clinical and pathological findings, and have indolent, self-limited character. [ABSTRACT FROM AUTHOR]

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 (P =.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% (P =.042) and their PSWQ scores by 11% (P =.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. [ABSTRACT FROM AUTHOR]

Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a rare subtype of non-Hodgkin lymphoma, CD30 positive that does not exhibit extracutaneous manifestations at the time of diagnosis. The emergence of solitary papules or nodules, on the trunk and extremities, characterizes the disease. This case reports a 58-year-old female who presented with a rapidly enlarging nodule on her right calf. The biopsy revealed a malignant neoplasm of large cells. The morphological features, combined with the immunohistochemical profile, revealed a CD30-positive and anaplastic lymphoma kinase-negative lymphoproliferative disorder, consistent with C-ALCL. Shortly after the first excision, new lesions manifested in violaceous papules and nodules, and a new biopsy was performed, confirming the initial diagnosis. The patient underwent radiotherapy for 4 weeks and the lesions regressed but recurred about a year after. The patient is currently under treatment. The main goal is to emphasize the importance of considering this diagnosis as a possibility in large-cell cutaneous lymphomas. [ABSTRACT FROM AUTHOR]

Primary cutaneous CD4+ small to medium T-cell lymphoproliferative disorder is rare, accounting for only 2-3% of all primary cutaneous lymphomas. Patients usually present in their 50s with a rapidly growing yet asymptomatic nodule on the face or neck. In this report, we describe the case of a 50-year-old man with an asymptomatic, small, round nodule on his temple area appearing in the preceding two months without any systemic involvement. Surgical excision was done, and histopathological and immunohistochemical studies revealed high infiltration of CD4+ lymphocytes in the dermis, confirming the diagnosis of primary cutaneous CD4+ small to medium T-cell lymphoproliferative disorder. An excellent outcome was achieved, with no signs of recurrence after two months of follow-up. As the disease is often overlooked in the differential diagnoses of suddenonset, rapidly growing lesions, this report reminds physicians to keep it in mind and avoid unnecessary aggressive interventions. [ABSTRACT FROM AUTHOR]

Copyright of Die Pathologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Rare subtypes of cutaneous T-cell lymphomas (CTCL) include four entities, primary cutaneous γδ T-cell lymphoma, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, and primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders, primary cutaneous acral CD8+ T-cell lymphoma, which were previously considered provisional and are now included in the new 5th World Health Organization classification of hematolymphoid tumors as distinct entities. An updated summary of the clinical, histological, and genomic characteristics of these uncommon CTCL subtypes is given in this review, with a focus on the growing body of knowledge regarding their classification and possible treatment strategies.

Mycosis fungoides (MF) and Sezary syndrome (SS) are important representatives of the heterogeneous group of cutaneous T-cell lymphoma. The diagnosis of MF/SS is based on the clinical, histopathologic and immunohistochemical findings, flow cytometric analysis of peripheral blood and T-cell receptor gene rearrangement, as well as other genetic analysis. The treatments for MF/SS are always stage-oriented and are classified into topical (skin-directed) and systemic therapies. Chinese and international experts in this field have updated recommendations and guidelines for the diagnosis and treatment of MF/SS. There are tremendous evolvements in the diagnosis of MF/SS, including molecular diagnostic methods, molecular biomarkers, dermoscopic imaging and artificial intelligence. Novel disease-specific targeted therapy, biologic therapy and immunomodulatory drugs have emerged. This review summarizes advances in the diagnosis and treatments of MF/SS, which may provide a reference for clinical practice and future research.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), is a rare and aggressive variant of primary cutaneous lymphoma that typically expresses B cells as well as MUM1/IRF4, BCL2, and FOXP1, whereas BCL6 may be present or undetectable. We present a case of CD5+ PCDLBCL-LT presenting as a 6 mm pink-bluish nodule on the mid-left thigh, which was concerning for basal cell carcinoma. The histological examination reveals the presence of an intradermal proliferation of large, atypical CD5+, CD20+ BCL2+, BCL6+, MUM-1+, and Cyclin-D1+ lymphocytes in a nodular, diffuse interstitial and perivascular distribution. Because the patient presented with a small, single nodule, the systemic treatment of multiagent chemotherapy was avoided and localized electron beam radiation therapy with rituximab was initiated instead, achieving complete response. Early identification of PCDLBCL-LT is key for maximal therapeutic benefit and prognosis; it is important to consider PCDLBCL-LT on the differential when evaluating small, single nodules on the lower extremities of elderly patients.

Abstract Primary cutaneous follicle centre lymphomas (PCFCLs) are cutaneous proliferation of centrocytes and centroblasts, with a generally indolent evolution. Secondary systemic spread of disease is a rare event, which significantly impairs prognosis. Recently published study by Zhou and al. proposed criteria aiming to predict such evolution at initial stage: (i) rearrangement of Bcl2, (ii) Ki67% index 30% Ki67% indexes and no mutations among the four proposed genes. These findings run contrary to these previously proposed criteria, highlighting the molecular heterogeneity of this rare disease.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (CD4+PCSM-LPD) is a low-grade cutaneous T cell disorder. There is no standardized approach to treatment of CD4+ PCSM-LPD due to its rarity. Herein, we discuss a 33-year-old woman with CD4+PCSM-LPD which resolved after a partial biopsy. We highlight that conservative and local treatment modalities should be considered prior to utilizing more aggressive and invasive treatment options.

A 72-year-old man with a history of squamous cell carcinoma presented to the Portland VA with forehead discoloration. He was initially diagnosed with actinic damage and prescribed topical treatment. However, he returned to clinic months later with a large, violaceous forehead plaque. Upon biopsy, he was diagnosed with blastic plasmacytoid dendritic cell neoplasm (BPDCN), a rare hematological malignancy. This case report illustrates the importance of keeping BPDCN in the differential diagnosis for ecchymotic plaques that fail to respond to first line therapy.

The large cell transformation of mycosis fungoides (MF-LCT) is a phenomenon observed in the advanced stages of mycosis fungoides (MF), which is the most common primary cutaneous lymphoma. The diagnostic criteria of MF-LCT are a minimum of 25% of large cells or a formation of microscopic nodules of them in the histological examination of skin samples. The clinical outcomes for MF-LCT are poor, as less than 20% of patients survive 5 years after diagnosis, but the expression of the CD30 antigen is generally considered to be associated with a better prognosis. We present a case of a patient with the diagnosis of MF with LCT, with an ulcerated tumor lesion approximately 30 × 20 cm in size on the right lateral abdominal wall. Brentuximab vedotin (BV) treatment was started due to the presence of the CD30 antigen, with a quick and impressive regression of the cutaneous lesion and tumor mass and good treatment tolerance. After follow-up of 20 months, patient remains in complete remission. A schedule of treatment for MF-LCT is directed mainly by the clinical stage of the disease and the comorbidities; the more severe clinical course of the disease requires systemic treatment. If at least 5% of the cells found in the skin lesions biopsy sample express the CD30 antigen, a beneficial effect of BV treatment could be expected. It may seem that the use of BV is one of the optimal therapeutic options in patients with advanced MF-LCT showing expression of CD30.

Primary cutaneous follicle center lymphoma (PCFCL) differs from follicular lymphoma in biological behavior and molecular profile and is treated as a distinct entity, according to the 5th edition of the World Health Organization classification of hematolymphoid tumors. It is an uncommon cutaneous B-cell lymphoma that is considerably rare in children and adolescents. To date, only 13 cases of individuals younger than 20 years of age have been reported in the literature. The lack of relevant clinical epidemiological data in this population has hampered the investigation of its clinical and diagnostic aspects. Here we report the case of a 17-year-old male with PCFCL, who may be the first PCFCL patient under 20 years of age reported in China. He was admitted to the hospital with a solitary nodule on his face. After complete surgical excision, the patient's facial mass was histologically identified as PCFCL. The patient's prognosis was favorable, with no recurrence at 17 months of follow-up after the surgical resection. We present a case of an adolescent PCFCL patient and systematically review the literature with a view to increase the awareness of the disease and inform the diagnosis and treatment of this age group. [ABSTRACT FROM AUTHOR]

Purpose of Review: This review focuses on updates in prognosis, pathogenesis, and treatment of cutaneous T cell lymphoma (CTCL). Recent Findings: Cohort studies indicate imaging may be necessary in early-stage CTCL. Risk factors for progression of CTCL have been identified. Interactions between malignant cells and the tumor microenvironment (TME) and the skin microbiome advance the understanding of pathogenesis and tumor cell dissemination. Studies support a hypothesis of circulating malignant tumor cells. MicroRNA (miR) influence tumor progression and prognosis; the IL22-STAT3-CCL20 cascade may be a novel target. IL-4, IL-5, and IL-31 cytokines are relevant for pruritus and could be targets for therapeutic interventions. Systemic therapies, such as JAK inhibitors, targeted antibodies, and checkpoint inhibitors, show promise in advanced stages. Allogenic hematopoietic stem cell transplantation provides a potential curative option for patients. Summary: Further investigations of prognosis and translational research are necessary to improve stratification of patients for treatment. [ABSTRACT FROM AUTHOR]

Cutaneous T-cell lymphomas (CTCL) are characterized by focal infiltration of malignant T cell clones in solitary skin lesions. Many CTCL patients experience an indolent disease, but some progress to advanced disease with high fatality. We hypothesized that natural killer (NK) cells participate in local control of tumor growth in CTCL skin. Immunohistochemistry and flow cytometry analysis of the density, localization, phenotype and function of NK cells in twenty-nine fresh or formalin-fixed skin biopsies from twenty-four CTCL patients and twenty-three biopsies from twenty healthy controls highlighted higher numbers of CD56+CD3-NK cells in CTCL skin. A reduced fraction of CTCL skin NK cells expressed the maturation marker CD57, the cytotoxic protein granzyme B and the activation marker CD69, indicating reduced tumor-killing abilities of the NK cells. Retained expression of immune checkpoint proteins or inhibitory proteins including PD1, TIM3, LAG3, CD73 and NKG2A and the activating receptors CD16 and NKp46 indicated maintained effector functions. Indeed, the capacity of NK cells to produce anti-tumor acting IFNg upon PMA+ionomycin stimulation was similar in cells from CTCL and healthy skin. Co-cultures of primary human NK cells or the NK cell line NKL with CTCL cells resulted in reduced levels of granzyme B and CD69, indicating that close cellular interactions with CTCL cells induced the impaired functional NK cell phenotype. In conclusion, increased numbers of NK cells in CTCL skin exhibit a partially impaired phenotype in terms of activity. Enhancing NK cell activity with NK cell activating cytokines such as IL-15 or immune checkpoint blockade therefore represents a potential immunotherapeutic approach in CTCL. [ABSTRACT FROM AUTHOR]

Mycosis fungoides (MF) and Sezary syndrome (SS) are important representatives of the heterogeneous group of cutaneous T-cell lymphoma. The diagnosis of MF/SS is based on the clinical, histopathologic and immunohistochemical findings, flow cytometric analysis of peripheral blood and T-cell receptor gene rearrangement, as well as other genetic analysis. The treatments for MF/SS are always stage-oriented and are classified into topical (skin-directed) and systemic therapies. Chinese and international experts in this field have updated recommendations and guidelines for the diagnosis and treatment of MF/SS. There are tremendous evolvements in the diagnosis of MF/SS, including molecular diagnostic methods, molecular biomarkers, dermoscopic imaging and artificial intelligence. Novel disease-specific targeted therapy, biologic therapy and immunomodulatory drugs have emerged. This review summarizes advances in the diagnosis and treatments of MF/SS, which may provide a reference for clinical practice and future research. [ABSTRACT FROM AUTHOR]

Definitive radiation therapy is an effective local treatment for several cutaneous malignancies. Patients with diffuse or generalized skin manifestations might require total skin electron beam therapy (TSEBT) as an alternative treatment to the chasing technique. In this short communication, we highlight the evolving role of TSEBT and present its role in various forms of skin malignancies. [ABSTRACT FROM AUTHOR]

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), is a rare and aggressive variant of primary cutaneous lymphoma that typically expresses B cells as well as MUM1/IRF4, BCL2, and FOXP1, whereas BCL6 may be present or undetectable. We present a case of CD5+ PCDLBCL-LT presenting as a 6 mm pink-bluish nodule on the mid-left thigh, which was concerning for basal cell carcinoma. The histological examination reveals the presence of an intradermal proliferation of large, atypical CD5+, CD20+ BCL2+, BCL6+, MUM-1+, and Cyclin-D1+ lymphocytes in a nodular, diffuse interstitial and perivascular distribution. Because the patient presented with a small, single nodule, the systemic treatment of multiagent chemotherapy was avoided and localized electron beam radiation therapy with rituximab was initiated instead, achieving complete response. Early identification of PCDLBCL-LT is key for maximal therapeutic benefit and prognosis; it is important to consider PCDLBCL-LT on the differential when evaluating small, single nodules on the lower extremities of elderly patients. [ABSTRACT FROM AUTHOR]

Primary cutaneous lymphomas (CLs) represent a heterogeneous group of T-cell lymphomas and B-cell lymphomas that present in the skin without evidence of extracutaneous involvement at time of diagnosis. CLs are largely distinct from their systemic counterparts in clinical presentation, histopathology, and biological behavior and, therefore, require different therapeutic management. Additional diagnostic burden is added by the fact that several benign inflammatory dermatoses mimic CL subtypes, requiring clinicopathological correlation for definitive diagnosis. Due to the heterogeneity and rarity of CL, adjunct diagnostic tools are welcomed, especially by pathologists without expertise in this field or with limited access to a centralized specialist panel. The transition into digital pathology workflows enables artificial intelligence (AI)-based analysis of patients' whole-slide pathology images (WSIs). AI can be used to automate manual processes in histopathology but, more importantly, can be applied to complex diagnostic tasks, especially suitable for rare disease like CL. To date, AI-based applications for CL have been minimally explored in literature. However, in other skin cancers and systemic lymphomas, disciplines that are recognized here as the building blocks for CLs, several studies demonstrated promising results using AI for disease diagnosis and subclassification, cancer detection, specimen triaging, and outcome prediction. Additionally, AI allows discovery of novel biomarkers or may help to quantify established biomarkers. This review summarizes and blends applications of AI in pathology of skin cancer and lymphoma and proposes how these findings can be applied to diagnostics of CL. [ABSTRACT FROM AUTHOR]