Primary Cutaneous B-cell Lymphoma Research Articles

Primary Cutaneous CD4 Small/Medium T-Cell Lymphoproliferative Disorder Following COVID-19 Vaccination-What Do We Know about Lymphoproliferative Disorders and Cutaneous Lymphomas after COVID-19 Vaccination? A Report of an Atypical Case and a Review of the Literature.

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.

A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL).

Primary cutaneous B-cell lymphomas (PCBCLs) are B-cell lymphomas that can occur in the skin without evidence of extracutaneous involvement. The 2005 WHO/EORTC classification of cutaneous lymphomas and its 2018 update have distinguished three main categories based on clinicopathological, immunohistochemical, and genetic characteristics: primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle centre lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT). PCMZL and PCFCL are clinically indolent, while PCDLBCL-LT is an aggressive lymphoma. Due to its low incidence and lack of prospective studies, it is difficult to establish a standard treatment for each subgroup. The objective of our study was to describe the clinical and pathological characteristics of 103 patients with cutaneous B-cell lymphoma from 12 centres belonging to the Spanish Lymphoma Oncology Group. The median age was 53 years (40-65). According to skin extension, 62% had single-site lymphoma, 17% had regional lymphoma, and 20% had multifocal lymphoma. Histology: 66% had PCMZL, 26% had PCFCL, and 8% had PCDLBCL-LT. Twenty-three percent of the patients were treated exclusively with surgery, 26% with radiotherapy only, 21% with surgery plus radiotherapy, 10% with polychemotherapy, and 5% with rituximab monotherapy. Overall, 96% of patients achieved a complete response, and 44% subsequently relapsed, most of them relapsing either locally or regionally. The 10-year OS was 94.5% for the entire cohort, 98% for the PCMZL cohort, 95% for the PCFCL cohort, and 85.7% for the PCDLBCL-LT cohort. Our data are comparable to those of other published series, except for the high frequency of PCMZL. The expected heterogeneity in therapeutic management has been observed.

Increased incidence of thyroid, renal, lung, melanoma, bladder, and prostate cancers after diagnosis of primary cutaneous B-cell lymphoma: A SEER database analysis

Increased incidence of thyroid, renal, lung, melanoma, bladder, and prostate cancers after diagnosis of primary cutaneous B-cell lymphoma: A SEER database analysis

Health-related quality of life and its influencing factors in patients with primary cutaneous B-cell lymphomas: A multicentric study in 100 patients.

Primary cutaneous B-cell lymphomas (CBCL) are a group of rare malignant skin diseases that represent approximately 20%-30% of all primary cutaneous lymphomas (PCL). Previous studies revealed impaired health-related quality of life (HRQoL) in patients diagnosed with primary cutaneous T-cell lymphoma (CTCL). Currently, only small-sized studies investigated HRQoL in CBCL patients and lacked detailed analysis of respective subtypes. This study aims to investigate HRQoL in CBCL patients to identify independent factors of HRQoL impairment in CBCL patients. One hundred CBCL patients were recruited from eight German PCL centres in this multicentric, cross-sectional study from 2021 to 2022. The patients completed the dermatologic HRQoL questionnaire Skindex-29 and an investigator-designed 'CBCL-Questionnaire' with additional questions on HRQoL and clinical characteristics. The Skindex-29 revealed that HRQoL in CBCL patients is impaired on a mild to moderate level. The multiple regression analysis identified parameters like worries about dying, feeling prejudiced/discriminated and impairment of daily activities to be independently associated with impairment of HRQoL. Highest scores for HRQoL impairment were found in patients with primary cutaneous follicle centre lymphoma while on rituximab treatment and in patients with primary cutaneous marginal zone lymphoma while on watchful waiting. HRQoL is impaired in CBCL patients, even though, in the face of indolent disease course and favourable prognosis in the majority of cases. Of note, our investigator-designed tool identified worries about dying, feeling prejudiced/discriminated, and the type of treatment to have a negative impact on patients' HRQoL. Our study highlights the importance of a thorough patient-doctor communication to capture overall disease burden because generic HRQoL tools might lack of disease-specific items.

Primary Cutaneous Follicle Center Lymphoma in an Adult African American Male

Primary cutaneous B-Cell Lymphomas (PCBCL) is a form of a lymphoproliferative neoplasm consisting of proliferation localized to the skin. Cutaneous lymphomas originate from mature T-lymphocytes, B-lymphocytes, or NK cells. The incidence of PCBCL is significantly lower in patients with Skin of Color (SoC) and is quite rare among African American (AA) individuals, compared to their Caucasian counterparts. The clinical course of PCFCL in SoC is not widely studied or reported in literature due to the low incidence in the AA population. We present a case of PCBCL in an AA individual along with a brief review of the clinical presentation, pathology, and treatment options of the condition.  According to the U.S. Census Bureau, approximately half of the U.S population will have SoC by 2050; by adding our case of PCFCL to the body of literature, we hope to promote awareness of the presentation and course of this cutaneous disorder in SoC patients. 

Primary cutaneous diffuse large B-cell lymphoma NOS with short-term relapse

Primary cutaneous B-cell lymphomas are a unique, heterogeneous group of lymphoproliferative dis¬orders without evidence of extra-cutaneous manifestations. Primary cutaneous diffuse large B-cell lymphomas (PCDLBCLs) are aggressive neoplasms with poor prognosis. The infiltrate in some cases could involve the underlying subcutaneous tissues, but rarely reported. Accurate diagnosis is man¬datory as these patients respond well to systemic anthracycline-based chemotherapy (R-CHOP). In this article, we report a case of PCDLBCL/NOS that presented with skin manifestations and was diagnosed based on clinical features, pathology, and immunohistochemical staining. The patients were treated with systemic chemotherapy, CHOP-R, and responded well and the PET scan showed no evidence of residual disease after the 5th cycle. After 3 months of follow-up, the patient succumbed to his disease involving almost all his skin.

New Perspectives for Patients with Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type

New Perspectives for Patients with Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type

Increased Incidence of Melanoma, Prostate, Lung, Bladder, Renal and Thyroid Cancer after Diagnosis of Primary Cutaneous B-Cell Lymphoma: A SEER Database Analysis

Background: Primary cutaneous B-cell lymphomas (PCBCLs) are a subset of non-Hodgkin lymphomas originating in the skin. The risk of developing other malignancies in PCBCL patients is not known. We evaluated the risk of developing a second primary malignancy after a PCBCL diagnosis. Methods: We utilized the surveillance, epidemiology, and end results (SEER-17) data registry of 9,208,295 patients from 2000-2020 to generate a cohort of 5,435 patients with PCBCL to identify patients at risk for a second primary malignancy. Relative risk was calculated by using the Standardized Incidence Ratio (SIR), defined as the ratio between observed cases (O) and expected cases (E) of malignancies (SIR = O/E). Statistical significance was established based on 95% confidence intervals (CI). Results: Of the 5,435 patients with PCBCL, 847 (16%) received diagnoses of a second malignancy, identifying a higher risk than the general population (SIR, 1.54; 95% CI, 1.43-1.64). Males had a higher chance of developing prostate cancer (SIR, 1.29; 95% CI, 1.07-1.54), melanoma (SIR, 1.49; 95% CI, 1.03-2.09), and bladder cancer (SIR, 1.42; 95% CI, 1.01-1.94). Females were more predisposed to cancers of the lung (SIR, 1.69; 95% CI, 1.25-2.24), thyroid (SIR, 3.06; 95% CI, 1.63-5.23), and kidney (SIR, 2.13; 95% CI 1.06-3.81). For males with PCBCL, there was an increased risk of prostate cancer in patients 60-69 (SIR, 1.53; 95% CI, 1.14-2.02). For females, there was an increased risk of lung cancer in patients over 70, especially in the age group of 70-79 (SIR, 1.84; 95% CI, 1.12-2.84). For cutaneous melanoma, bladder, renal, and thyroid cancer, there was no age group identified to be at a significantly greater risk. Prostate cancer risk was increased within the first year (SIR, 2.09; 95% CI, (1.28 - 3.23) and between 5-10 years after PCBCL diagnosis (SIR, 1.45; 95% CI, 1.04-1.97) .For males, melanoma and bladder cancer were associated with an increased risk between 1-5 years after PCBCL (SIR, 1.91; 95% CI, 1.09-3.11 and SIR, 1.7; 95% CI, 1.01-2.68). Females were at the highest risk of developing thyroid cancer within the first year of PCBCL diagnosis (SIR, 21.11; 95% CI, 10.12-38.83) and lung cancer 1-5 years after PCBCL diagnosis (SIR, 1.7; 95% CI, 1.04-2.62). For renal cancer, there was no latency identified carrying an increased risk. Conclusion: These data, should inform screening strategies for melanoma, prostate, bladder, lung, renal, and thyroid cancer in patients with PCBCL. Males 60-69 within 10 years of PCBCL can benefit from prostate screening. Males within 1-5 years of PCBCL diagnosis can benefit from screening for melanoma and bladder cancer. Females can benefit from thyroid, lung, and renal cancer surveillance. Specifically for females over 70, screening for lung cancer should be considered, and for females within the first year of PCBCL diagnosis, thyroid cancer screening should be considered.

Characteristics of Primary Cutaneous Lymphoma in Italy: A Tertiary Care, Single-Center Study.

Data on primary cutaneous lymphomas (PCLs) patients in the Italian population are limited, and, despite the existence of several treatment options, the management of those patients remains challenging. Our study aimed to investigate the clinical and therapeutic features of PCL patients in a referral center in Italy. We conducted a retrospective study on 100 consecutive PCL patients between January 2017 and December 2022. The mean (SD) age of our cohort was 70.33 (14.14) years. Cutaneous T-cell lymphomas (CTCLs) represented 65% of all cases; the majority were mycosis fungoides (42%), followed by cases of Sezary syndrome (10%) and primary cutaneous anaplastic large cell lymphoma (4%). Cutaneous B-cell lymphomas (CBCLs) accounted for 35 % of PCLs, with 15 cases of primary cutaneous follicle center lymphoma, 10 cases of primary cutaneous diffuse large B-cell lymphoma leg type, and 9 cases of marginal zone B-cell lymphoma. A higher frequency of pruritus (p = 0.008) and higher peripheral blood levels of beta-2 microglobulin (p ≤ 0.001) and lactate dehydrogenase (p = 0.025) were found in CTCLs compared to those of CBCLs. Considering all therapeutic lines performed, treatments were extremely heterogeneous and skin-directed therapies represented the most frequently used approach. Our study confirms the distribution of PCL subtypes formerly reported in the literature and highlights the utility of real-life data in treatments to improve the current management of PCL patients.

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

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

Spontaneous Regression of Primary Cutaneous Diffuse Large B-Cell Lymphoma on Sequential FDG PET.

Primary cutaneous diffuse large B-cell lymphoma, leg type is a rare, aggressive lymphoma characterized by skin involvement predominantly in the lower extremities. We present a case of an elderly woman with pathology diagnosis of primary cutaneous diffuse large B-cell lymphoma, leg type with spontaneous regression without systematic therapy documented by sequential FDG PET scans and clinical follow-up.

Diffuse Large B-cell Lymphoma (Leg Type) Originating in the Skin of the Right Cheek Region: A Case Report and Literature Review.

Primary diffuse cutaneous large B-cell lymphoma, leg type (PCDLBCL-LT), usually affecting one or both lower legs, with a 5-year disease-free survival rate of less than 60%. Solitary facial lesions are extremely rare. Our report is about a 93-year-old woman whose clinical examination revealed a 4 cm × 5 cm × 3 cm mass with a soft texture and smooth margins on the right side of her cheek. Immunohistochemical analyses were consistent with a diagnosis of PCDLBCL-LT. The surgical method for this patient was: extensive resection of the tumor and repair of the defect with an adjacent flap. Neither local recurrence nor systemic invasion was observed during postoperative follow-up (8 months). The clinician must be very careful when making a correct diagnosis based on the clinical and immunohistochemical findings of PCDLBCL-LT. For this type of PCDLBCL-LT isolated in 1 site without invasion of the rest of the body, extensive surgical resection may result in a favorable prognosis.

AN ATYPICAL PRESENTATION OF PRIMARY CUTANEOUS FOLLICLE CENTER B-CELL LYMPHOMA

Background: Primary cutaneous follicle center B-cell lymphoma(PCFCL) is a low-grade lymphoma that affects adults around the age of fifty. We report the case of an atypical presentation. Cas Report:4 years after the complete excision of a lesion above the left eyebrow leading to the diagnosis of a lymphocytic infiltrate in favor of a lymphocytoma. A 55-year-old woman developed bilateral lesions in the ears, treated surgically. One year later, two masses, pseudo tumoral temporal and frontalappeared. After excisional biopsy with immunohistological study and an extension assessment, the diagnosis of PCFCL was retained and polychemotherapy of the CHOP type was undertaken. Discussion :The two localizations, at the level of the ear and fronto-temporal, the antecedent of lymphocytic infiltrate, conferred to our observation its atypical character.

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.

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)

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

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.