Project description:Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma characterized by infiltration of the subcutaneous tissue by neoplastic cytotoxic T cells mimicking panniculitis. There is a strong association between SPTCL and lupus erythematosus panniculitis (LEP). However, patients who were diagnosed with LEP with a preceding diagnosis of SPTCL have been scarcely reported. We herein reported a 21-year-old Thai woman presenting to a dermatology clinic for evaluation of a 1-month history of a painful mass on the right buttock and bilateral upper eyelid swelling. A subcutaneous mass which was 5 by 2 cm in diameter, tender, firm, and fixed with a smooth surface was palpated over the upper outer quadrant of her right gluteal area. After a diagnosis of SPTCL had been made based on the histological and immunohistochemical studies, treatment with oral dexamethasone and ciclosporin A was initially started. Because of intolerance to adverse reactions of dexamethasone, only ciclosporin A was given. Improvement was not achieved at 6-month follow-up. She then underwent the re-incisional biopsy at the same gluteal area and the histological features were consistent with LEP. After hydroxychloroquine was given, the lesion resolved within 3 months, and no recurrence was detected during the following 6-month follow-up. We emphasize that long-term follow-up of patients with SPTCL is required. Additionally, in case of poor response to the given treatment, a repeat skin biopsy should be considered in order to determine the proper management.
Project description:BackgroundSLE is associated with increased risk of diffuse large B-cell lymphoma (DLBCL). DLBCL is routinely classified by cell of origin (COO), with germinal centre B-cell (GCB) being more common and indicating better prognosis in the general population. We studied COO subtyping in patients with SLE diagnosed with DLBCL and their survival.Patients and methodsWe evaluated 20 cases of SLE with DLBCL. Immunohistochemistry analysis was performed (BCL2, MYC, BCL6, CD10, CD20, FOXP1, GCET1, MUM1) in tissue microarrays. We examined associations between molecular and clinical features, including overall survival.ResultsOf the 20 DLBCL SLE cases, 12/20 cases (60%) were classified as non-GCB using Hans or Choi algorithms. MYC and BCL2 protein expression was positive in 6/20 (30%) and 8/20 (40%) SLE cases, respectively, with 2/20 (10%) co-expressing both markers. Seven (7/20) had only extranodal involvement at DLBCL diagnosis. As expected, non-GCB cases had worse survival. Cases presenting exclusively with extranodal disease were associated with shorter SLE duration and better survival despite higher BCL2 protein expression.ConclusionsWe present novel data characterising DLBCL in SLE. Sixty per cent of the DLBCL in patients with SLE were non-GCB. The nodal and extranodal distribution in SLE was similar to what is known in the general population, but extranodal disease occurred more often with short SLE duration and was associated with longer overall survival. More research on cancer in SLE is the key to further understanding the complex interplay between cancer and the immune system.
Project description:Autoreactive B cells are one of the key immune cells that have been implicated in the pathogenesis of systemic lupus erythematosus (SLE). In addition to the production of harmful auto-antibodies (auto-Abs), B cells prime autoreactive T cells as antigen-presenting cells and secrete a wide range of pro-inflammatory cytokines that have both autocrine and paracrine effects. Agents that modulate B cells may therefore be of potential therapeutic value. Current strategies include targeting B-cell surface antigens, cytokines that promote B-cell growth and functions, and B- and T-cell interactions. In this article, we review the role of B cells in SLE in animal and human studies, and we examine previous reports that support B-cell modulation as a promising strategy for the treatment of this condition. In addition, we present an update on the clinical trials that have evaluated the therapeutic efficacy and safety of agents that antagonize CD20, CD22 and B-lymphocyte stimulator (BLyS) in human SLE. While the results of many of these studies remain inconclusive, belimumab, a human monoclonal antibody against BLyS, has shown promise and has recently been approved by the US Food and Drug Administration as an indicated therapy for patients with mild to moderate SLE. Undoubtedly, advances in B-cell immunology will continue to lead us to a better understanding of SLE pathogenesis and the development of novel specific therapies that target B cells.