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Screening for neurodegeneration in Langerhans cell histiocytosis with neurofilament light in plasma.

Patients with Langerhans cell histiocytosis (LCH) may develop progressive neurodegeneration in the central nervous system (ND-CNS-LCH). Neurofilament light protein (NFL) in cerebrospinal fluid (CSF) is a promising biomarker to detect and monitor ND-CNS-LCH. We compared paired samples of NFL in plasma (p-NFL) and CSF in 10 patients (19 samples). Nine samples had abnormal CSF-NFL (defined as ≥380 ng/l) with corresponding p-NFL ≥ 2 ng/l. Ten samples had CSF-NFL < 380 ng/l; eight (80%) with p-NFL < 2 ng/l (p < 0.001; Fisher's exact test). Thus, our results suggest that p-NFL may be used to screen

Epigenetic remodeling by vitamin C potentiates plasma cell differentiation.

Ascorbate (vitamin C) is an essential micronutrient in humans. The severe chronic deficiency of ascorbate, termed scurvy, has long been associated with increased susceptibility to infections. How ascorbate affects the immune system at the cellular and molecular levels remained unclear. From a micronutrient analysis, we identified ascorbate as a potent enhancer for antibody response by facilitating the IL-21/STAT3-dependent plasma cell differentiation in mouse and human B cells. The effect of ascorbate is unique as other antioxidants failed to promote plasma cell differentiation. Ascorbate is e

Plasma cell gingivitis, an underestimated condition in patients with Sjögren's syndrome?

OBJECTIVES: To explore the correlation between the severity of radiotherapy-induced glossitis (RTG) and endothelial cell injury in local tissues in a rat model. STUDY DESIGN: The RTG animal model was designed and used by our team. The Oral mucositis index(OMI) was documented daily. Immunohistochemistry (IHC) Staining of CD34 was utilized to identify endothelial cells in the RTG tissues. Apoptosis of endothelial cells in local lesions due to RTG was detected by the TUNEL assay. The dynamic relationship between the OMI and apoptotic endothelial cells was statistically analyzed by time. RESULTS

Plasma Cell Leukemia: A Review of 3 Cases Managed in Kenya.

Plasma Cell Leukemia (PCL) is a rare and aggressive form of plasma cell dyscrasia that can arise either de novo (primary plasma cell leukemi … anomegaly. The diagnosis is made based on at least 20% of total white blood cells being circulating plasma cells with a peripheral blood absolute plasma cell count of at least 2 × 109/l. Treatment with novel agents followed by autologous stem cell transplant in those who are transplant eligible leads to better outcomes.

Clinical and Biological Characteristics of Medullary and Extramedullary Plasma Cell Dyscrasias.

Background: Extramedullary plasma cell (PC) disorders may occur as extramedullary disease in multiple myeloma (MM-EMD) or as primary extramedullary plasmocytoma (pEMP)/solitary osseous plasmocytoma (SOP). In this study, we aimed to obtain insights into the molecular mechanisms of extramedullary spread of clonal PC. Methods: Clinical and biological characteristics of 87 patients with MM-EMD (n = 49), pEMP/SOP (n = 20) and classical MM (n = 18) were analyzed by using immunohistochemistry (CXCR4, CD31, CD44 and CD81 staining) and cytoplasmic immunoglobulin staining combined with fluorescence in s

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… CD31 (JC70) expression in plasma cells: An immunohistochemical analysis of reactive and neoplastic plasma cells. J. Clin. Pathol. 1997;50:490–493. doi: 10.1136/jcp.50.6.490. 12. Plocharczyk E., Wakely P.E. CD31 expression in plasmacytic/plasmablastic lesions. Ann. Diagn. … CD44 isoforms are differentially regulated in plasma cell dyscrasias and CD44v9 represents a new independent prognostic parameter in multiple myeloma. Leuk. Res. 2001;25:1051–1057. doi: 10.1016/S0145-2126(01)00075-3. 23. …

Plasma circulating cell-free mitochondrial DNA in depressive disorders.

BACKGROUND: Plasma circulating cell-free mitochondrial DNA (ccf-mtDNA) is an immunogenic molecule and a novel biomarker of psychiatric disorders. Some previous studies reported increased levels of ccf-mtDNA in unmedicated depression and recent suicide attempters, while other studies found unchanged or decreased ccf-mtDNA levels in depression. Inconsistent findings across studies may be explained by small sample sizes and between-study variations in somatic and psychiatric co-morbidity or medication status. METHODS: We measured plasma ccf-mtDNA in a cohort of 281 patients with depressive disor

An atypical presentation of plasma cell gingivitis with generalized skin lesions.

This article aims to present a report of an atypical clinical presentation of a plasma cell gingivitis (PCG) case with unusual skin lesions. PCG is a rare benign inflammatory condition which can be classified into Type 4 hypersensitivity reaction. It occurs due to reaction to unknown antigen, often flavoring agents or spices found in chewing gums, toothpastes and lozenges. Histologically, the lesion shows dense plasma cells infiltrate in the connective tissue. Early diagnosis of PCG is essential to differentiate from variety of conditions, namely, leukemia, HIV infection, discoid lupus erythem…

Cross-segment spinal plasma cell granuloma:a case report.

BACKGROUND: Plasma cell granuloma (PCG) is a rare non-neoplastic entity, with the precise etiology remaining unclear. Vertebra-affected spin … inconclusive, thereby necessitating complete excision of the lesions. Finally, the infiltration of plasma cells observed by pathological examination of the surgical specimen confirmed the diagnosis of PCG. CONCLUSIONS: To the authors' knowledge, this was the first case of cross-segment spinal PCG with osteolytic property. The possibility of PCG should be con …

A patient with Gaucher disease and plasma cell dyscrasia: bidirectional impact.

Patients with Gaucher disease (GD), a rare autosomal recessive glycosphingolipid storage disease, commonly present to hematologists with unexplained splenomegaly, thrombocytopenia, anemia, and bone symptoms. Patients with GD may develop other manifestations, such as autoimmune thrombocytopenia, monoclonal gammopathy, multiple myeloma, or, even more rarely, other hematological malignancies; sometimes they are first diagnosed during an assessment of those disorders. Although the diagnosis and management of patients with GD have significantly evolved over the last 30 years, some patients remain p

Plasma Cell Gingivitis Mimicking Granulomatous Disease: A Diagnostic Dilemma.

INTRODUCTION: Plasma cell gingivitis is an uncommon benign inflammatory condition of the gingiva which is considered to arise as a hypersensitivity reaction to certain allergens. The distinguishing feature of this condition is massive infiltration of plasma cells into the subepithelial connective tissue. This case report discusses a case of plasma cell gingivitis which produced a diagnostic dilemma due to its unusual histopathologic appearance. CASE PRESENTATION: A 13-year-old female patient presented with diffuse gingival enlargement in relationship to upper anterior teeth. The case was mana…

Plasma cell cheilitis: the diagnosis of a disorder mimicking lip cancer.

Plasma cell cheilitis (PCC) is an inflammatory disorder of unknown etiology that affects the lip. It is characterized histologically by a dense infiltrate of plasma cells with a variety of clinical features. The response to different therapeutic modalities is controversial, especially regarding the effectiveness of corticosteroids. We present a case of a 56-year-old Caucasian man with a painful ulcerated and crusted area in the lower lip, resembling a squamous cell carcinoma or actinic cheilitis. Topical corticosteroid was used for one week, which resulted in partial regression and motivated a…

Thrombocytosis as an Initial Presentation of Plasma Cell Neoplasm: A Case Report.

Plasma cell neoplasms are usually associated with normal or decreased platelet count. The association of thrombocytosis and multiple myeloma is exceedingly rare, with only six such cases reported in the literature until now. Differentiating clonal from secondary causes of thrombocytosis can be extremely difficult, yet the distinction has important therapeutic implications. We report the case of a woman presenting with thrombocytosis that led to the diagnosis of multiple myeloma. The possible etiological link between both these entities is also discussed.

ReactELISA method for quantifying methylglyoxal levels in plasma and cell cultures.

Methylglyoxal (MG) is a toxic glycolytic by-product associated with increased levels of inflammation and oxidative stress and has been linked to ageing-related diseases, such as diabetes and Alzheimer's disease. As MG is a highly reactive dicarbonyl compound, forming both reversible and irreversible adducts with a range of endogenous nucleophiles, measuring endogenous levels of MG are quite troublesome. Furthermore, as MG is a small metabolite it is not very immunogenic, excluding conventional ELISA for detection purposes, thus only more instrumentally demanding LC-MS/MS-based methods have dem

Is lenalidomide the standard-of-care after an autotransplant for plasma cell myeloma?

Three randomized controlled trials and a meta-analysis reported lenalidomide given after high-dose therapy and an autologous hemopoietic cell transplantation is associated with increase in progression-free survival (PFS) and survival in persons with plasma cell myeloma (PCM). Based on these data, posttransplant lenalidomide is considered by many a standard-of-care in this setting. However, decisions on the use of new therapies should consider not only results of such trials and meta-analyses but also other factors including quality-of-evidence, anticipated desired and undesired effects of the

Oligosecretory Primary Plasma Cell Leukemia with Atypical Morphological Abnormality.

Plasma cell leukemia (PCL) is a rare variant of multiple myeloma. The detection of plasma cells in the peripheral blood and monoclonal protein in the serum or urine is important for the diagnosis of PCL. However, it is sometimes difficult to diagnose PCL in patients with atypical plasma cell morphology and/or those without detectable monoclonal protein. We herein report a case of oligosecretory PCL showing atypical morphology in leukemic cells with a convoluted nucleus and basophilic cytoplasm but without detectable monoclonal protein, except for serum free light chain. A flow cytometric analy…

Analyzing T-Cell Plasma Membrane Lipids by Flow Cytometry.

Plasma membrane lipid rafts are highly ordered membrane microdomains enriched for glycosphingolipids and cholesterol, which play an important role during T-cell antigen receptor (TCR) signaling. Our previous work has demonstrated that plasma membrane lipid composition is an important determinant of human CD4+ T-cell function and that defects in lipid raft expression contribute to CD4+ dysfunction in patients with autoimmunity. In this chapter we share three flow cytometry-based methods to quantitatively analyze plasma membrane lipid composition in primary human CD4+ T cells. We describe the qu

How I treat elderly patients with plasma cell dyscrasias.

Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders which are more prevalent in the older part of the population. The introduction of novel agents, improved understanding of disease biology and better supportive management have improved outcomes considerably and in the era of the aging population the question of how to best manage older patients with plasma cell dyscrasias has never been more relevant. Data on how to treat these patients comes mostly from subgroup analysis as they are underrepresented in clinical trials. This review will cover issues, available evi

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… Introduction Plasma cell dyscrasias are a rare heterogeneous group of disorders characterized by the expansion of monoclonal bone marrow plasma cells. … Figure adjusted from ESMO guidelines for WM 2018 and EMN recommendations for treatment of rare plasma cell dyscrasias. …