Cutaneous B-cell Lymphoma Research Articles

Helicobacter pylori and skin disorders: a comprehensive review of the available literature.

Helicobacter pylori is a Gram-negative bacterium identified for the first time about 30 years ago and commonly considered as the main pathogenic factor of gastritis and peptic ulcer. Since then, it was found to be associated with several gastrointestinal and extra-gastrointestinal diseases. Helicobacter pylori is also associated with many skin disorders including, but not limited to, chronic urticaria, rosacea, lichen planus, atopic dermatitis, psoriasis, pemphigus vulgaris, vitiligo, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, primary cutaneous marginal zone B-cell lymphomas and cutaneous T-cell pseudolymphoma. Literature up to September 2020 shows that clear evidence exists only for some of the mentioned associations, while in the majority of cases, data appear contrasting. The aim of this review is to summarize the available studies on the topic and draw possible conclusions. Further clinical and laboratory studies are needed to assess the real plausibility and relevance of these associations, as well as the possible role of Helicobacter pylori with the underlying pathogenic mechanisms.

Primary cutaneous diffuse large B-cell lymphoma, leg type: case report

ABSTRACT The report describes the clinic of an aggressive non-Hodgkin lymphoma, primary cutaneous diffuse large-B cell lymphoma, leg type (PCDLBCL-LT), and shows its relevance for addressing the case of a rare lymphoma, with complex diagnosis and, in most cases, late as there are few studies reported on the subject. Since it is an aggressive pathology, it is essential to have the dissemination of knowledge to assist in the early diagnosis of the disease and, consequently, in the choice of a more effective treatment. Therefore, we seek to demonstrate the main clinical characteristics, as well as the diagnostic approach, staging, and the symptomatic and specific treatment of PCDLBCL-LT.

Systemic chemotherapy for primary cutaneous b-cell lymphoma, leg-type: a case report

Primary cutaneous lymphomas are a group of extranodal B-cell non-Hodgkins lymphomas. Primary cutaneous diffuse B-large cell lymphoma, leg-type, is an extremely rare and aggressive variant of primary cutaneous lymphoma. Due to the contradictory nature, poor prognosis, and high frequency of recurrence of this disease, the treatment of patients is multidisciplinary, based on an accurate histological and immunohistochemical classification as well as risk factor assessment. In this report, we present a clinical case of primary cutaneous B-large cell skin lymphoma, leg-type, with a positive response to chemotherapy.
 Clinical case. A clinical observation of chemotherapy of a patient with primary B-large cell skin lymphoma, leg-type, is presented. The patient underwent 6 R-CHOP chemotherapy courses and achieved an uncertain complete disease remission.
 Conclusion. The results obtained confirm the limited literature data on the need for timely administration of systemic chemotherapy in the management of this pathology.

PO-0922: De-escalated radiotherapy for indolent primary cutaneous B-cell lymphoma

PO-0922: De-escalated radiotherapy for indolent primary cutaneous B-cell lymphoma

Primary Cutaneous Follicle Center Lymphoma (PCFCL) with Parotid Gland Involvement

Abstract Introduction/Objective Primary cutaneous follicle center lymphoma (PCFCL) is the most common primary cutaneous B-cell lymphoma. It arises from mature germinal center B lymphocytes. Here we report two cases of PCFCL with parotid gland involvement. Methods First case is a 66-year-old male with an enlarging forehead mass for nine months and a painless nodule on the right pre-auricular skin. Second is a 39-year-old male with a history of a recurring scalp spindle cell B-cell lymphoma now presenting with an enlarging lesion on the scalp and “fullness” in the right neck. There was no nodal or other extranodal involvement found in either case. Skin and parotid gland biopsies were obtained in both cases. Results Case one: The skin and parotid gland demonstrated sheets of predominantly medium sized infiltrating lymphoma cells, positive for CD20, Bcl-6, CD5 (dim) and Bcl-2 (dim), and negative for CD10, Bcl-1, and MUM-1. The epidermis was spared. Case two demonstrated medium sized, spindle shaped lymphoma cells. The skin showed a vague follicular growth pattern, sparing the epidermis. The parotid gland showed diffuse infiltration by lymphoma cells, positive for CD20 and Bcl-6, Bcl-2 (dim) and no definite positivity for CD10. Fluorescent in situ hybridization for t(14;18) translocation was absent in both cases. Conclusion Dissemination of PCFCL to extracutaneous sites is uncommon (~10% of cases) and to our knowledge, has not been reported in the parotid gland. Here we present two unique cases, which in the absence of nodal disease, prove the diagnosis of PCFCL with parotid gland involvement.

Expression profile of immunological and vascular mediators in an unusual case of paravertebral primary cutaneous non-Hodgkin lymphoma

Primary cutaneous follicle center lymphoma (PCFCL) is a rare type of indolent, low-grade cutaneous B-cell lymphoma, most frequent in trunk, particularly the head and scalp.

Linfomas cutáneos. Parte II: otros linfomas cutáneos

Primary cutaneous T-cell lymphomas other than mycosis fungoides, Sézary syndrome, and lymphoproliferative CD30+ disorders are few, accounting for less than 5% of all cutaneous lymphomas. A cytotoxic phenotype is characteristic of these tumors, and their clinical behavior is usually aggressive. Patients often present with extracutaneous symptoms or develop them shortly after diagnosis. Management is usually multidisciplinary, and intensive systemic therapy and bone marrow transplantation should be considered. Cutaneous B-cell lymphomas account for approximately 30% of primary cutaneous lymphomas. They make up a heterogeneous group of tumors that have different clinical and pathological features. Clinical course also varies. Presenting as papules, nodules, or tumors of variable reddish–violaceous coloring, the lesions may be solitary or multiple and occasionally form clusters. There may also be generalized lesions, present at multiple sites on the trunk, head, or extremities. Three well-defined groups of primary cutaneous lymphoma have been reported: follicle center lymphoma; marginal zone lymphoma, which follows an indolent course; and a diffuse large B-cell lymphoma, leg type, which follows an aggressive course.

Prognostic significance of Bcl-2 expression in primary cutaneous B-cell lymphoma: a reappraisal

Bcl-2 family protein plays an important role in apoptosis and its overexpression is protects neoplastic cell from apoptotic stimuli. Cutaneous B-cell lymphoma are rare non-Hodgkin lymphomas and can be classified in primary forms, featuring an exclusive skin-involvement at diagnosis, and cutaneous spread of a nodal disease. Such a distinction is not trivial, owing to different prognosis (indolent vs. aggressive) and therapeutic management. Bcl-2 expression at immunohistochemistry can be crucial in differential diagnosis between cutaneous and systemic disease, as well as between the different primary cutaneous forms. In the last few years, an animated debate on the prognostic role of Bcl-2 overexpression at molecular analysis have been developed in cutaneous B-cell lymphoma. To conclude, Bcl-2 expression have a diagnostic role more than prognostic in primary cutaneous B-cell lymphomas.

Value of Teledermoscopy in Primary Healthcare Centers: Preliminary Results of the TELESPOT Project in Belgium.

IntroductionTeledermoscopy using smartphone-based applications is becoming more and more important in a setting of increasing frequency of skin cancer and difficult access to specialized care. The TELESPOT project aimed to provide rapid diagnosis and speed up patient flow between primary healthcare centers and a tertiary care center in Belgium. The aim of the present study is to describe the development of an in-house smartphone-based dermoscopy application, evaluate its real-life value in a series of primary healthcare centers, and present preliminary diagnostic data.MethodsModified Likert scales were used to assess patient and general practitioner (GP) satisfaction rates for the system. Furthermore, a total of 105 photographic and dermoscopic images were acquired in a series of 80 patients at participating centers.ResultsOverall, patient and GP satisfaction levels were 89% and 94%, respectively. High-priority management was recommended in 7.6% of cases (8/105: 3 basal cell carcinoma, 1 primary cutaneous B-cell lymphoma, 1 Spitz melanocytic nevus, 1 congenital nevus, 1 in situ melanoma, and 1 invasive melanoma, proven by histology).ConclusionsThe primary healthcare centers were highly satisfied with the TELESPOT project in terms of user-friendliness, efficacy, and reliability as well as in providing a reinforced image of first-line medicine efforts in combating skin cancer.

Kappa and lambda immunohistochemistry and in situ hybridization in the evaluation of atypical cutaneous lymphoid infiltrates.

Atypical cutaneous lymphoid infiltrates are challenging lesions in dermatopathology. We present a summary of the literature regarding kappa and lambda immunohistochemistry (IHC) and in situ hybridization (ISH) in the evaluation of atypical cutaneous or mucosal lymphoid infiltrates. Relevant articles from 1967 to 2018 in the English language were identified and summarized. In the absence of larger studies, case series of n ≥ 3 were included. Sixty-three articles assessing kappa and lambda IHC and/or ISH were identified. Most focused on marginal zone lymphomas. Other lymphomas included follicle center lymphoma, diffuse large B-cell lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma, mantle cell lymphoma, lymphoplasmacytic lymphoma, plasmablastic lymphoma, multiple myeloma, monoclonal gammopathy of undetermined significance, and polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS). Non-neoplastic lesions included reactive lymphoid hyperplasia, cutaneous plasmacytosis, connective tissue disease, IgG4-related disease, acrodermatitis chronic atrophicans, Zoon balanitis, dermatitides, and infiltrates around epithelial dysplasias/neoplasias. Kappa and lambda IHC and ISH are useful tools in the evaluation of cutaneous B-cell lymphomas and plasma cell neoplasms. The literature supports that the detection of light-chain restriction by IHC and ISH is one of the most useful findings in the differential diagnosis of reactive lymphoid hyperplasia vs B-cell lymphoma with plasmacytic differentiation.

Cutaneous B-cell lymphomas: 2021 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. Both 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, multi-agent chemotherapy is rarely appropriate. In contrast, management of patients with PCDLBCL, LT is comparable to the management of patients with systemic DLBCL.

Primary Cutaneous B-Cell Lymphomas in Patients With Impaired Immunity.

Primary Cutaneous B-Cell Lymphomas in Patients With Impaired Immunity.

UNCOMMON MANIFESTATION OF DIFFUSE CUTANEOUS LARGE B-CELL LYMPHOMA LEG-TYPE IN THE FACE - CASE REPORT

Primary cutaneous large B-cell lymphoma, leg-type is the most aggressive form of primary cutaneous B-cell lymphoma. It is characterized by progressive nodules and tumors initially localized to 1 anatomic region, most commonly the legs. A 48-year-old woman with melanoderma attended the service of oral and maxillofacial surgery reporting weight loss, fever, and swelling in the lower third of the face 3 months ago, followed by episodes of improvement and aggravation. Clinical extraoral examination revealed an increase in volume in the lower third of the face, reddish, diffuse plaque, and elevated and irregular surface in the chin and submental regions, associated with bilateral superficial cervical lymphadenomegaly. The incisional biopsy of the lesion was submitted to histopathological and immunohistochemical analysis, which revealed the diagnosis of diffuse cutaneous large B-cell lymphoma leg-type. The patient is in post-chemotherapy follow-up of 2 years and 4 months without evidence of relapse of the disease.

Innovative Therapeutic Approaches in Primary Cutaneous B Cell Lymphoma.

Background: Primary cutaneous B-cell lymphomas (pCBCL) include an infrequent group of non-Hodgkin lymphomas that are limited to skin sites at the time of diagnosis. They comprise roughly 20–25% of all cutaneous lymphomas and are subdivided into primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large cell B cell lymphoma, leg type (PCDLCBCL, LT). The first two show a rather indolent course while PCDLCBCL, LT carries a worse prognosis. Intravascular large cell B-cell lymphoma is the most infrequent subtype, and its therapy is not covered in this review.Topical Therapy: For solitary, single-site PCMZL and PCFCL, several topical treatment options exist. They include, but are not limited to, excision, radiotherapy, and intralesional therapies, discussed in this review. However, in selected cases, even “watchful waiting” is reasonable.Systemic Therapy: Indolent types of pCBCL rarely require systemic treatment. However, in extended cases and more importantly DLCBCL, LT, systemic treatment is the first choice. Monoclonal anti-CD20-antibody rituximab is often used as monotherapy in PCMZL and PCFCL or combined with chemotherapy in PCDLBCL, LT. Newer options are monoclonal anti-CD40 antibody dacetuzumab, anti-PD-1 and anti-PD-L1 checkpoint inhibitors, and Bruton tyrosine kinase inhibitors.Conclusion: Indolent pCBCL are treated with a risk-adapted strategy using intralesional steroids, RT, and interferon-α as first-line treatments. Relapsing cases may profit from rituximab. In aggressive PCDLCBCL, LT, rituximab with polychemotherapy is recommended. Innovative therapies include intralesional oncolytic virotherapy, systemic monoclonal antibodies, and small molecules.

Oncolytic Virotherapy Mediated Anti-Tumor Response: A Single-Cell Perspective

Talimogene laherparepvec (T-VEC) is a genetically modified herpes simplex 1 virus (HSV-1), approved for cancer therapy. We investigated its effect on the clinical, histological, single-cell transcriptomic and immune repertoire level using repeated fine-needle aspirates (FNAs) of injected and noninjected lesions in primary cutaneous B-cell lymphoma (pCBCL). Thirteen patients received intralesional T-VEC, eleven of which demonstrated tumor response in the injected lesions. Upon single cell sequencing of the FNAs, we identified the malignant population and separated three pCBCL subtypes. HSV-1T-VEC transcripts were detected 24h after injection in malignant and non-malignant cells of the injected lesion but were absent in the noninjected lesion. Oncolytic virotherapy resulted in a rapid eradication of malignant cells by more than one death mechanism, IFN pathway activation, early influx of NK cells, monocytes, dendritic cells, followed by an enrichment in clonal cytotoxic T-cells and decrease of regulatory T-cells in both injected and noninjected lesions.

Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type: A Narrative Review

Primary cutaneous lymphomas are rare manifestations of extranodal lymphomas. Comprising the majority of cutaneous lymphoma cases with neoplastic B-cell origins, three main subtypes exist. In general, grossly and microscopically these subtypes are similar. However, these are three distinct variants with diverse clinicopathologic, cytogenetic, molecular, and prognostic features. Primary cutaneous diffuse large B-cell lymphoma, leg type is an exceedingly rare and aggressive variant of primary cutaneous B-cell lymphomas. Thus, increased clinical awareness is needed to differentiate between the three subtypes because earlier identification not only leads to the appropriate treatment, but also improved survival. Here, characteristic features of the three predominant variants of primary cutaneous B-cell lymphomas are presented while remaining focused on the most aggressive subtype- primary cutaneous diffuse large B-cell lymphoma, leg type.

Systemic rituximab for the treatment of the indolent forms of primary cutaneous B-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry

Systemic rituximab for the treatment of the indolent forms of primary cutaneous B-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry

伴显著浆细胞分化的原发性边缘区B细胞淋巴瘤一例

伴显著浆细胞分化的原发性边缘区B细胞淋巴瘤一例

Multifocal cutaneous non-epitheliotropic B-cell lymphoma in a cat

Case summarySkin tumours are the second-most common form of feline cancer after haematopoietic neoplasms and are often malignant. Cutaneous lymphoma is uncommon in cats and can be classified as epitheliotropic (typically of T-cell origin) or non-epitheliotropic (either of T-cell or B-cell origin). The present study describes a case of multifocal cutaneous non-epitheliotropic B-cell lymphoma. The skin nodules were multiple and variable in size; showed rapid progression; were alopecic and erythematous in appearance and pruritic and ulcerated; and were mostly located on the trunk. Nodule biopsies revealed the presence of uniform medium-to-large round neoplastic cells that infiltrated the dermis and subcutis. The neoplasias were consistent with a round cell cutaneous tumour and did not show evidence of epitheliotropism. Furthermore, immunohistochemical assessments indicated an immunophenotype characterised by round cells with a strong membrane and cytoplasmic positivity for the CD20 antigen, consistent with a lymphocyte of B-cell origin.Relevance and novel informationCutaneous non-epitheliotropic B-cell lymphoma in cats is rare and was previously reported to appear as single dermal and subcutaneous masses that are variable in size and generally develop in the tarsal region. To our knowledge, this is the first report to describe multifocal cutaneous non-epitheliotropic B-cell lymphoma in a cat.

Primary cutaneous diffuse large B-cell lymphoma, leg type – case report and literature review

Primary cutaneous diffuse large B-cell lymphoma, leg type – case report and literature review