DeCure for Nodal marginal zone B-cell lymphoma

Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

AbstractPrimary cutaneous B-cell lymphomas are rare entities that develop primarily in the skin. They constitute a heterogeneous group that represents around a quarter of primary cutaneous lymphomas. The 2018 update of the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification differentiates primary cutaneous marginal zone lymphoma and primary cutaneous follicle center lymphoma with an indolent course from primary cutaneous diffuse large B-cell lymphoma, leg type with an aggressive behavior. The broad spectrum of clinical presentations and the disease course marked by frequent relapses are diagnostic and therapeutic challenges. The classification of these diseases has been refined in recent years, which allows to better define their immunopathogenesis and specific management. In the present article, we review the main clinico-biological characteristics and the current therapeutic options of these three main subsets. Based on the recent therapeutic advances in nodal B-cell lymphomas, we focus on the development of novel treatment options applicable to primary cutaneous B-cell lymphomas, including targeted therapies, combination treatments and immunotherapeutic approaches, and cover basic, translational and clinical aspects aiming to improve the treatment of cutaneous B-cell lymphomas.

AbstractMarginal zone lymphoma (MZL) is commonly underrepresented in clinical trials collectively studying mostly nodal indolent lymphomas.In this manuscript we propose new inclusion and response criteria defined by MZL subtype and disease location for those with extranodal MZL. Progression of disease within 24 months is associated with poor outcomes in MZL and future studies should assess the efficacy of novel agents in this population.

A roadmap for clinical trial design in marginal zone lymphoma

AbstractMarginal zone lymphoma (MZL) is commonly underrepresented in clinical trials collectively studying mostly nodal indolent lymphomas.In this manuscript we propose new inclusion and response criteria defined by MZL subtype and disease location for those with extranodal MZL. Progression of disease within 24 months is associated with poor outcomes in MZL and future studies should assess the efficacy of novel agents in this population.

A case of nodal marginal zone B-cell lymphoma of the lower eyelid

AbstractA marginal zone B-cell lymphoma is a type of B-cell lymphoma which is normally located at the margins of the secondary lymph node follicles. According to 2008 World Health Organization (WHO) classification, there are three main types classified according to the location of invasion: nodal lymphoma in the lymph nodes, splenic lymphoma in the spleen, and extranodal lymphoma in other locations. Recently, we have experienced a rare case of primary nodal marginal zone lymphoma that arose in the lower eyelid. Therefore, we report this case with a review of literature.

Clinical Features and Prognostic Assessment of Nodal Marginal Zone B-Cell Lymphoma, a Rare Disease with Follicular-Like Behaviour.

AbstractAbstract Background. Primary nodal marginal zone B-cell lymphoma (MZL) is a rare entity recognized by the WHO classification. Diagnosis requires a lymph node localization in the absence of prior or concurrent involvement of extranodal sites. Most studies reported so far focus mainly on histopathology, while the clinical features and outcome of this uncommon lymphoma remains less defined. Aim. To define the clinical features and to assess prognosis of primary nodal marginal zone B-cell lymphoma. Methods. We studied a series of 47 newly diagnosed patients with primary nodal marginal zone B-cell lymphoma. Diagnosis was made on histologic examination of lesional tissues integrated with immunohistochemical data. No patient showed MALT or splenic localisation of lymphoma at diagnosis. Results. Patients: 17 males and 30 females, median age 63 years (25–79) with 64% aged more than 60 years. 13% of patients had stage I disease, 10% stage II, 32% stage III, 45% stage IV (bone marrow involvement). 11% had peripheral blood involvement, 11% had bulky disease, 15% B symptoms, 6% ECOG score ≥ 2. 23% had hemoglobin <12 g/dl. LDH was above normal in 15% and β2-microglobulin in 45%. 11% had an autoimmune background. HCV serology was positive in 24% (9/38). With the IPI score 37% ranked in the low risk, 22% in the low-intermediate, 35% in the intermediate-high, and 7% in the high risk category. Using the FLIPI score, 33% were classified as low risk, 34% as intermediate risk, and 33% as high risk. After treatment, 57% achieved a complete response and 24% a partial response, for an overall response rate of 81%. At a median follow-up of 2.6 years, no patient developed splenic or MALT involvement. 5-years and 10-years OS is 69% (95% CI 52–86%). Death occurred in 10 pts (related to NHL in 9, to another neoplasm in one). In univariate analysis the following factors were associated with shorter event-free survival (EFS): B symptoms (p=0.001), high vs intermediate vs low risk FLIPI score (p=0.009). The following factors were associated with worse overall survival: high vs intermediate vs low risk FLIPI score (p=0.02), age > 60 years (p=0.05), LDH above normal (p=0.05). HCV positivity was of borderline significance (p=0.06). In multivariate analysis hemoglobin < 12 g/dl (p=0.02, HR 14.3) was predictive of shorter EFS. Concerning overall survival, only the FLIPI retained statistical significance in predicting a worse outcome (p=0.02, HR 3.5). Positive HCV serology was of borderline significance (p=0.06, HR 4.4). Conclusions: among marginal zone neoplasms, primary nodal marginal zone lymphoma appears a distinct disorder with an indolent behaviour. The association with HCV infection (25%) is particularly high in comparison with non-marginal zone lymphomas. Considering the prognostic assessment of this rare disease, the FLIPI score is effective in detecting patients at worse prognosis with the same power as in follicular lymphoma. Thus, the application of the FLIPI may be of clinical value for treatment decision also in primary nodal marginal zone lymphoma.

Nodal marginal zone lymphoma – etiopathology, diagnosis and treatment

AbstractNodal marginal zone lymphoma (NMZL) is an unfrequent type of indolent non-Hodgkin lymphoma. NMZL affects main­ly the lymph node, but it presents with histologic as­pects similar with splenic marginal zone lymphoma or extranodal marginal lymphoma, usually without af­fec­ting other structures. Nevertheless, NMZL remains a con­di­tion that needs to be further studied in order to better under­stand its pathogenesis, to better diagnose it and treat the patients with this affliction.