Cutaneous B-cell Lymphoma Research Articles

CD20 Negative Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type with Rapidly Progressing Skin Lesions

A 95-year-old man came with violaceous nodular skin lesions of the left shin. The lesions were not painful or pruritic but progressed rapidly over the 3 months. Fever, night sweats, weight loss, and other systemic symptoms were absent. Shave biopsy of the lesion revealed the cells negative for CD20 but positive for BCL2, BCL6, FOX-P1, and CD10. PET/CT showed high uptake in the left leg and a sizeable hypermetabolic lymph node in the left pelvis. The patient was started on rituximab treatment as a single agent. This report highlights some of the diagnostic and treatment difficulties in CD20 negative lymphomas and the importance of a multidisciplinary approach in those cases.

Prevalence of autoimmune diseases in cutaneous B-cell lymphoma: A retrospective cross sectional analysis

Prevalence of autoimmune diseases in cutaneous B-cell lymphoma: A retrospective cross sectional analysis

O-124 - Liquid biopsy assessment in primary cutaneous diffuse large B-Cell lymphoma- leg type

O-124 - Liquid biopsy assessment in primary cutaneous diffuse large B-Cell lymphoma- leg type

44603 Primary cutaneous diffuse large B-cell lymphoma, leg type with spontaneous regression: report of 2 cases

44603 Primary cutaneous diffuse large B-cell lymphoma, leg type with spontaneous regression: report of 2 cases

O-125 - Detection of circulating tumor DNA in liquid biopsies of patients with primary cutaneous diffuse large B-cell lymphoma, leg type

O-125 - Detection of circulating tumor DNA in liquid biopsies of patients with primary cutaneous diffuse large B-cell lymphoma, leg type

O-123 - Single-cell RNA sequencing characterization of primary cutaneous B-cell lymphomas with their systemic counterparts

O-123 - Single-cell RNA sequencing characterization of primary cutaneous B-cell lymphomas with their systemic counterparts

41909 Investigation of clinicopathological, molecular, and immune profile characteristics of Primary Cutaneous B-Cell Lymphomas (PCBCL)

41909 Investigation of clinicopathological, molecular, and immune profile characteristics of Primary Cutaneous B-Cell Lymphomas (PCBCL)

Identifying and addressing unmet clinical needs in primary cutaneous B-cell lymphoma: A consensus-based paper from an ad-hoc international panel.

Primary cutaneous B-cell lymphomas (PCBCLs) are lymphoproliferative disorders that appear on the skin without evidence of extracutaneous manifestations at the time of diagnosis. There is a lack of evidence-based guidelines for their clinical management due to the availability of very few large scale studies and controlled clinical trials. Here we present and discuss a series of major unmet clinical needs (UCNs) in the management of PCBCLs by a panel of 16 experts involved in research and clinical practice of PCBCL. The Panel produced recommendations on the appropriateness of the clinical decisions concerning the identified clinical needs and proposed research for improving the knowledge needed to solve them. Recommendations and proposals were achieved by multiple-step formalized procedures to reach a consensus after a comprehensive analysis of the scientific literature. Recommendations and proposals lay in the domain of classification uncertainties of PCBCL, optimization of diagnosis, optimization of prognosis, optimization of staging and critical issues on therapeutic strategies with particular focus on new treatments. These recommendations are intended for use not only by experts but above all by dermatologists and hematologists with limited experience in the field of PCBCLs as well as general practitioners.

F-18 FDG PET/CT in staging and response assessment of primary cutaneous diffuse large B-cell lymphoma (leg type)

Primary cutaneous Diffuse Large B-Cell Lymphoma-leg type (PCDLBCL-LT) is a rare subtype of cutaneous lymphomas, with high frequency of extra-cutaneous relapse and poor prognosis. We report a case of 70-year-old lady who was diagnosed with PCDLBCL-LT on biopsy and underwent a baseline F-18 FDG PET/CT, followed by interim and post-treatment PET/CTs. With this case report, we highlight the findings of F-18 FDG PET/CT in the staging of this cutaneous lymphoma, and also emphasize on its role in the response assessment.

Clinical characteristics and efficacy analysis of 11 patients with primary cutaneous diffuse large B-cell lymphoma, leg type

Clinical characteristics and efficacy analysis of 11 patients with primary cutaneous diffuse large B-cell lymphoma, leg type

Accessibility and readability of online patient education on cutaneous lymphomas

Patients facing a cutaneous lymphoma diagnosis frequently turn to the internet for information but finding patient-accessible education may be a challenge. To investigate accessibility and readability of patient-oriented online education on cutaneous lymphomas, including cutaneous T-cell and B-cell lymphoma subtypes. 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. 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<.001∗), with higher grade levels for cutaneous T-cell lymphoma subtype webpages than cutaneous B-cell lymphoma subtypes. Webpage quality, accuracy, and language were not assessed. Current online education for cutaneous lymphomas exceeds the American Medical Association's maximum readability recommendation. There is a need for more patient-accessible education amidst predominance of scientific literature, greater dermatology host websites, and enhanced readability of existing online education.

Management strategies and survival in cutaneous B-cell lymphoma: A population based study

Management strategies and survival in cutaneous B-cell lymphoma: A population based study

Eyelid involvement of primary cutaneous lymphomas: A clinical perspective.

Eyelids may be affected in systemic, ocular adnexal and primary cutaneous lymphomas (PCLs). The frequency of eyelid involvement in PCLs is still not well known and it is not a predilection site for any type. While primary cutaneous T-cell lymphomas (CTCLs) are more commonly seen than primary cutaneous B-cell lymphomas (CBCLs), especially mycosis fungoides (MF) as by far the most frequent type, B cell lymphomas are reported to be the commonest type in eyelid localization on the contrary. PCLs may be located on the eyelids, as the sole manifestation or in association with the involvement of other parts of the eye and elsewhere of the body. MF may present with a rich spectrum of clinical features on the eyelids mostly seen in folliculotropic subtype and advanced-stage disease. Erythematous scaly patches or plaques representing the most commonly encountered eyelid MF lesions may mimic many other dermatological conditions. Diffuse thickening, oedema, poikilodermic changes, atrophy and wrinkling are other suggestive findings of eyelid MF. Milia-like papules, madarosis and ectropion are also seen in the folliculotropic variant of MF, as ectropion is more typical for Sezary syndrome. Eyelids are also a typical location for tumoural MF which has been suggested as a poor prognostic indicator in MF. Papulonodular lesions, large tumours, ulceration, diffuse infiltration, oedema and subcutaneous atrophy on the eyelids may also be seen in other types of PCLs. Keep in mind, the rich clinical spectrum of PCLs on the eyelids may be crucial in early diagnosis in this special localization.

Clinical Remission of Primary Cutaneous Marginal Zone B-Cell Lymphoma in a Patient With Crohn's Disease After Helicobacter pylori Quadruple Therapy and Vedolizumab.

Clinical Remission of Primary Cutaneous Marginal Zone B-Cell Lymphoma in a Patient With Crohn's Disease After Helicobacter pylori Quadruple Therapy and Vedolizumab.

Clinician's perspective on the diagnosis of primary cutaneous B-cell lymphoma.

Of all cutaneous lymphomas, 25% are primary cutaneous B-cell lymphomas (PCBCLs). Of these, primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone B-cell lymphoma (PCMZL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) are the most common subtypes. For the diagnosis of PCBCLs, a biopsy combined with immunohistochemistry and histological examination is the gold standard. PCBCLs are categorized into indolent or intermediate to aggressive subtypes based on their clinical behavior in a clinically oriented approach. PCDLBCL-LT has an aggressive course that spreads to extracutaneous sites in about 45% of cases, whereas PCFCL and PCMZL are indolent diseases. As a result, instrumental staging is advised for PCDLBCL-LT but not for extracutaneous disease after a diagnosis of PCMZL or PCFCL. Lastly, dermatoscopy may offer a novel diagnostic tool to improve the clinical recognition of various PCBCL subtypes when used in conjunction with a strong clinical suspicion.

CD30 expression in cutaneous B-cell lymphomas.

CD30 expression has been infrequently described in cutaneous B-cell lymphomas (CBCLs). We examined CD30 expression in reactive lymphoid hyperplasia (RLH) and CBCL and correlated expression with clinicopathologic features. CD30 was examined in 82 CBCL patients and 10 RLH patients that had been evaluated in our cutaneous lymphoma clinics. The CBCL patients included: primary cutaneous follicle center lymphoma (PCFCL), Grade 1/2 systemic/nodal follicular lymphoma (SFL); primary cutaneous marginal zone lymphoma/lymphoproliferative disorder (PCMZL/LPD); systemic marginal zone lymphoma (SMZL); primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT); and extracutaneous/systemic diffuse large B-cell lymphoma (eDLBCL). We scored CD30 expression for intensity and extent and related CD30 expression to age at first diagnosis, sex, site of biopsy, clinical appearance, extracutaneous involvement, multiple cutaneous lesions, B-symptoms, lymphadenopathy, positive positron emission tomography/computed tomography (PET/CT), elevated lactate dehydrogenase (LDH), and positive bone marrow biopsy. CD30 expression was identified in 35% of CBCL, ranging from few, weak, scattered cells to strong and diffuse expression. It was most common in PCFCL and was not expressed in PCDLBCL-LT. Rare PCFCL expressed strong, diffuse CD30. Some cases of PCMZL/LPD, SMZL, FL, and RLH showed scattered, strongly positive cells. CD30 expression in CBCL was associated with favorable clinical features: younger age, negative PET/CT, and an LDH within normal limits. CD30 may be expressed in CBCL, possibly causing diagnostic confusion. CD30 expression was most commonly identified in PCFCL and is associated with favorable clinical features. In cases with strong and diffuse expression, CD30 could be a therapeutic target.

What is new about primary cutaneous B cell lymphomas.

Primary cutaneous B-cell lymphomas (CBCL) are a heterogeneous group of B-cell lymphomas without evidence of extracutaneous disease at the time of diagnosis. The 2022 World Health Organization classification of mature lymphoid neoplasms differentiates the indolent primary cutaneous marginal zone lymphoproliferative disorder, primary cutaneous follicle center lymphoma and Epstein-Barr virus-positive mucocutaneous ulcer, from the more aggressive primary cutaneous diffuse large B-cell lymphoma, leg-type and intravascular large B-cell lymphoma. The new updates in the 2022 classification are based on recent scientific advances in the understanding and characterization of these entities. This article aims to review the main clinical, cellular and molecular features of the five CBCL subsets along with their management and treatment. The exponentially growing evidence for new treatment options for systemic B-cell lymphomas raises expectations for the field of CBCL as well. However, specific prospective high quality research on CBCL is still crucial to further define their management and update international guidelines.

Intralesional oncolytic virotherapy with talimogene laherparepvec in patients with cutaneous lymphomas and non-melanoma skin cancers.

9581 Background: Cutaneous lymphomas (CL) and non-melanoma skin cancers (NMSC) present with skin lesions and can often be treated with local therapies. However, some do not respond or relapse, underlining the need for new treatment options. Intralesional oncolytic virotherapy with genetically modified Herpes simplex 1 virus called Talimogene laherparepvec (T-VEC) was approved for cutaneous melanoma. Given previous reports on oncolytic virotherapy in non-melanoma tumors, we investigated the effect of intralesional T-VEC in patients with CL and NMSC. Methods: Patients with CL and NMSC without extracutaneous spread were eligible (NCT03458117). They were treated according to the standard for up to 8 injections. We assessed clinical response of injected and, whenever possible, non-injected lesions using response criteria for intratumoral therapies (itRECIST). Safety was assessed using CTCAE v5 criteria. To evaluate T-VEC induced changes, we collected tumor biopsies at baseline, after 2 and 5 injections. We performed multiplex immunohistochemistry for CD3, CD8, CD79a, CD56, CD11c and FoxP3 and evaluated the changes using Akoya Bioscience technology. Results: Twenty-seven patients were enrolled into the study: 19pts with cutaneous B-cell lymphoma (CBCL), 5pts with cutaneous T-cell lymphoma, 1pt with cutaneous squamous cell carcinoma and 1pt with Merkel cell carcinoma. The mean age was 61.9yrs. In total, 26pts have received at least one intralesional T-VEC, one patient has withdrawn consent before the first infusion. Patients received median of 7 injections, 11pts (44%) have received 8 planned injections. Therapy was well tolerated with treatment related fever, flu-like symptoms and ulceration of the injected tumor being the most common treatment related adverse events (AE) (34% each). Three patients (12%) had grade 3 AE. Of the injected lesions, 84% demonstrated response, with reduction of the elevation in 84% and reduction of redness of the tumor in 68%. Non-injected response was 40% and overall response was 32%. Tissue samples of 18pts were available for analysis. Following T-VEC injections, responding lesions had an increase of CD8+ and decrease of FoxP3+ cells, while all lesions had an increase of CD56+ cells and slight reduction of CD79a+ cells. Responding CBCL samples had increased CD3+ cells, whereas all samples had an initial reduction of CD79a+ and CD11c+ cells, independent of response. Conclusions: Oncolytic virotherapy with intralesional T-VEC induces clinical responses and is tolerated without any unexpected toxicities in patients with locally advanced cutaneous lymphomas and NMSC. T-VEC induces activation of immune response, with differences in responding and non-responding lesions. T-VEC represents a viable treatment option for patients with CL and NMSC, and should be investigated in larger controlled clinical trials. Clinical trial information: NCT03458117 .

Lymphatic vessel involvement in primary cutaneous B-cell lymphoma: A common finding?

Cutaneous B-cell lymphoma (CBCL) is part of dermatopathological routine diagnostics. However, in contrast to cutaneous T-cell lymphomas, there are only a few studies on the prevalence and possible clinical impact of lymphatic vessel involvement. Therefore, this pilot study aimed to quantify the prevalence of lymphovascular involvement in CBCL and to assess the association between lymphovascular involvement and recurrence. Thirty-nine patients from two tertiary care hospitals diagnosed with CBCL were retrospectively identified and their biopsies were histopathologically examined for the presence of lymphatic vessel involvement using H&E stain, and CD20 and D2-40 immunohistochemistry. Clinical data were retrieved from our digital documentation files. Thirty patients were included in the evaluation (nPCFCL = 15, nPCMZL = 10, and nPCLBCL = 5). Lymphovascular involvement occurred in all three types of lymphoma and was present in 14/30 specimens. The presence of lymphatic involvement did not show a significant impact on recurrence rate (p = 0.150). This immunohistochemical pilot study shows that lymphovascular involvement is a relatively frequent finding in primary CBCL. Although no definitive conclusion can be drawn from our findings because of the small sample size, there were no strong signs of tendencies for recurrence in either group. Future studies with larger sample size are warranted to assess the possible clinical implications.

Cutaneous B-cell lymphomas: 2023 update on diagnosis, risk-stratification, and management.

Approximately one-fourth of primary cutaneous lymphomas are B-cell derived and are generally classified into three distinct subgroups: primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT). Diagnosis and disease classification is based on histopathologic review and immunohistochemical staining of an appropriate skin biopsy. Pathologic review and an appropriate staging evaluation are necessary to distinguish primary cutaneous B-cell lymphomas from systemic B-cell lymphomas with secondary skin involvement. Disease histopathology remains the most important prognostic determinant in primary cutaneous B-cell lymphomas. Both PCFCL and PCMZL are indolent lymphomas that infrequently disseminate to extracutaneous sites and are associated with 5-year survival rates that exceed 95%. In contrast, PCDLBCL, LT is an aggressive lymphoma with an inferior prognosis. PCFCL and PCMZL patients with solitary or relatively few skin lesions may be effectively managed with local radiation therapy. While single-agent rituximab may be employed for patients with more widespread skin involvement, multiagent chemotherapy is rarely appropriate. In contrast, management of patients with PCDLBCL, LT is comparable to the management of patients with systemic DLBCL.