Search

Laryngeal oedema caused by acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a life-threatening condition. The swelling is bradykinin mediated and will not respond to the usual treatment with antihistamines, corticosteroids, or epinephrine. Instead, kallikrein-bradykinin-targeted therapies should be used promptly to prevent asphyxiation.

Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Crystalglobulinemia is an extremely rare pathology that is associated in most cases with plasma cell dyscrasia, mainly multiple myeloma. In most cases, it may be the manifestation of incipient gammopathy or it manifests shortly after diagnosis. We report a patient with ischemic lesions of thrombotic origin in lower limbs. Subsequently, renal involvement occurs, in view of this involvement, it is suspected that the patient may have an associated vasculitis. After performing the biopsy and with the subsequent diagnosis of monoclonal gammopathy of uncertain significance, the diagnosis is made. We review the most recent bibliography of patients who have been diagnosed with crystalglobulinemia associated with plasma dyscrasia focusing in those with thrombotic vasculopathy or acute renal failure. In our case, in addition to being associated with monoclonal gammopathy of undetermined significance that is less frequent, the debut of the symptoms is years before the detection of the monoclonal peak. This could speak of patients with a low peak of monoclonal component (not detected by immunoelectrophoresis) who could have kidney and vascular damage. [ABSTRACT FROM AUTHOR]

Associations between previous joint replacement and B‐cell lymphoid malignancies have been reported, but despite numerous reports, associations with the disease subtypes have received little attention. Using a UK‐based register of haematological malignancies and a matched general population‐based cohort, joint replacements from linked hospital inpatient records were examined. Cases diagnosed 2009–2015 who were aged 50 years or more were included; 8,013 mature B‐cell neoplasms comprising myeloma (n = 1,763), diffuse large B‐cell lymphoma (DLBCL, n = 1,676), chronic lymphocytic leukaemia (CLL, n = 1,594), marginal zone lymphoma (MZL, n = 957), follicular lymphoma (FL, n = 725) and classical Hodgkin lymphoma (CHL, n = 255), together with monoclonal gammopathy of uncertain significance (MGUS, n = 2,138) and monoclonal B‐cell lymphocytosis (MBL, n = 632). Odds ratios (OR) and 95% confidence intervals (95%CI) were calculated relative to 10 age‐ and sex‐matched controls using conditional logistic regression. Having had a joint replacement before diagnosis was associated with myeloma (OR = 1.3, 95% CI 1.1–1.5, p = 0.008) and MGUS (OR = 1.3, 95% CI 1.1–1.5, p < 0.001). Excluding replacements in the year before diagnosis, the MGUS risk remained, elevated where two or more joints were replaced (OR = 1.5, 95% CI 1.2–2.0, p = 0.001), with hip (OR = 1.2, 95% CI 1.0–1.5, p = 0.06) or knee replacements (OR = 1.5, 95% CI 1.2–1.8, p < 0.001). Associations with CHL and two or more replacements (OR = 2.7, 95% CI 1.3–5.6, p = 0.005) or hip replacements (OR = 1.9, 95% CI 1.0–3.4, p = 0.04); and between DLBCL and knee replacements (OR = 1.3, 95% CI 1.0–1.6, p = 0.04) were also observed. Our study reports for the first time a relationship between joint replacements and MGUS; while absolute risks of disease are low and not of major public health concern, these findings warrant further investigation. What's new? While lymphoid malignancies are increased in persons with previous joint replacements, data on associations with particular diagnostic subtypes is lacking. Here, the authors investigated the relationship between joint replacement and mature B‐cell neoplasms and their precursor conditions in an established cohort of patients with hematological malignancies linked to national healthcare records. Previous joint replacement was associated with subsequent elevated risk of myeloma, monoclonal gammopathy of uncertain significance, and Hodgkin lymphoma subtypes of hematological disease. While the findings indicate that absolute risks are low, joint replacement procedures are increasing and disentangling the underlying reasons behind these associations warrants further investigation. [ABSTRACT FROM AUTHOR]

Introduction: Monoclonal gammopathy of uncertain significance (MGUS) is highly prevalent in older adults and affects bone structure, with osteoporosis and increased risk of fractures in up to 14% of affected patients. Dual-energy X-ray absorptiometry (DXA), the standard technique for diagnosing osteoporosis, is ineffective to reveal microstructure and bone quality in this disease. Materials and methods: We conducted a cross-sectional study of patients with MGUS, recruited consecutively from the Hematology and Internal Medicine Departments of Hospital del Mar, Barcelona, between January 2011 and January 2018. Medical records, clinical results and spinal X-ray images were collected. Bone mineral density (BMD) at hip and spine was measured by DXA and Bone Material Strength index (BMSi) by impact microindentation on the tibial mid-shaft. Results: Thirty-nine patients with MGUS and 65 age-matched controls without previous fractures were included. In the MGUS group, 11 (28.2%) patients had prevalent fractures, nearly half of them vertebral (n = 5, 45.45%). Compared to controls, MGUS patients had significantly lower BMSi, a mean (SD) of 70.72 (9.70) vs. 78.29 (8.70), p = 0.001, and lower spinal BMD values (0.900 [0.159] vs. 1.003 [0.168], respectively, p = 0.012), but no significant differences at femoral neck and total hip. No association was observed between BMSi and DXA. Bone remodeling markers (procollagen type-1 N propeptide, bone-alkaline phosphatase and C-terminal telopeptide of type I collagen) did not differ between the two groups. Conclusions: Spinal BMD and mechanical properties of bone tissue, as measured by impact microindentation, were impaired in patients with MGUS. These changes in bone tissue mechanical resistance were independent of DXA levels. [ABSTRACT FROM AUTHOR]

Protein and immunofixation electrophoresis are the laboratory backbone assays for testing multiple myeloma (MM) and related plasma cell proliferative diseases. The secreted monoclonal proteins act as serological tumor markers, and the electrophoretic assays identify monoclonal proteins for diagnosis as well as quantitate these markers for monitoring clinical course and response to therapy. However these techniques present limitations, especially in patients with nonsecretory MM, light chain MM, and amyloid light chain amyloidosis. Freelite, a nephelometric/turbidimetric serum assay for the measurement of serum free light chains, has improved the management of patients with monoclonal gammopathies and has been included in international myeloma working group guidelines for the screening, prognostic, monitoring and as response criteria. Recently, a new assay, Hevylite; , was also approved by Food and Drug Administration for the monitoring of IgA and IgG MM. It allows the quantification of each immunoglobulin subtype (i.e., IgGκ, IgGλ, IgAκ, IgAλ), enables analysis of specific involved/monoclonal and uninvolved/polyclonal immunoglobulins heavy/light chain pairs, which produce immunoglobulin heavy/light chain ratios and may improve sensitivity for identifying residual disease and relapse in some patients. Together, these assays are important tools which will help us to achieve the correct diagnostic and follow-up of B cell dyscrasias.

Many case reports have indicated the occurrence of monoclonal gammopathy of uncertain significance (MGUS) or multiple myeloma (MM) in patients with Ph-negative myeloproliferative neoplasms (MPN), but few cohorts of patients have been published. This study concerns 667 patients newly diagnosed with polycythemia vera (PV) or essential thrombocythemia (ET) who were tested for monoclonal (M) protein at diagnosis (13.9% of patients). The overall survival of patients with M protein was dramatically lower than that of patients without M protein (12.7 versus 22.4 years; p = .0132). Univariate analysis identified the presence of M protein as a potential risk factor for the secondary occurrence of myelofibrosis (p = .02), myelodysplastic syndrome (p = .043), and MM/Waldenstrom macroglobulinemia (p < .0001). Our cohort shows that the presence of M proteins in patients with PV or ET leads to a poor prognosis. We believe that testing for M protein could help practicians to identify such patients. [ABSTRACT FROM AUTHOR]

Monoclonal gammopathy of uncertain significance is a premalignant plasma cell neoplasm with a high prevalence in the population over 50 years of age and an annual risk of progression of 1%. Multiple recent studies have led to advances in understanding both the pathogenesis of these disorders and their risk of progression to other diseases. Patients require lifelong follow-up, and a multidisciplinary and risk-adapted approach is essential. In recent years, an increasing number of entities associated with a paraprotein, known as clinically significant monoclonal gammopathies, have been recognized., (Copyright © 2023. Published by Elsevier España, S.L.U.)

Objective To investigate the use of peripheral nerve ultrasound (PNUS) in addition to nerve conduction studies (NCS) in the diagnosis of paraproteinemic neuropathies (PN). Methods PNUS/NCS of predefined peripheral nerves and the 5th/6th cervical roots were performed in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) (+/− paraprotein), patients with anti-MAG neuropathy, and patients with neuropathy and multiple myeloma or monoclonal gammopathy of uncertain significance (MGUS) — summarized as M-protein associated neuropathies (MPAN) and compared to controls (+/− paraprotein). Results 39 patients and 27 age-matched controls were included. Nerve enlargement was most marked in patients with CIDP, while in anti-MAG neuropathies enlargement was significant in the legs. In MPAN, no nerve enlargement is found regularly. However, in two cases, the diagnostic steps were influenced by the finding of multiple enlarged nerves and finally immunotherapy response was successfully initiated. By the use of the ultrasound pattern sum score (UPSS), differentiation of PN can be simplified. Discussion Due to the heterogeneous findings in NCS, correct diagnosis of PN, and straightforward therapeutic decisions often may be controversial. Particularly in cases of M-protein related neuropathy, the finding of multiple nerve enlargements facilitates the decision for therapeutic approaches or nerve biopsy. The UPSS enables the distinction of different PN from each other. Conclusion The use of an ultrasound quantification tool in addition to NCS facilitates a differentiation of PN. [ABSTRACT FROM AUTHOR]

Abstract: Flow cytometric (FC) enumeration of abnormal plasma cells (APCs) for diagnosis and prognostication of plasma cell dyscrasias (PCD) is challenging. We studied antigen expression in normal plasma cells (NPC) (N =34) and APC in a series of unselected PCD (N =59). NPC subpopulations often demonstrated CD19(−), CD20(+), CD45(−) or dim and CD56(+), an immunophenotype observed in PCD. However abnormal CD81 was only observed in APCs (APC detection sensitivity 95%; specificity 100%). We evaluated differences in antigen expression patterns among MGUS (N =14), SMM (N =35) and MM (N =10), finding the combination of CD45 and CD56 helpful in differentiating MGUS from SMM and MM (p =0.0002). [Copyright &y& Elsevier]

Multiple myeloma (MM) is a clonal B-cell malignancy characterised by excess bone marrow plasma cells, serum and/or urine paraprotein, immune paraesis, renal failure and lytic bone lesions. Dendritic cells (DC) are key players in the adaptive and innate immune responses, but reside in tissues, so are difficult to quantify in vivo. By enumerating the blood DC pool, we aim to examine the influence of MM disease and accompanying therapy on the DC system. We have shown, using the BDCA DC detection kit, that blood pDC and mDC numbers are suppressed at diagnosis in MM, and uniquely, monoclonal gammopathy of uncertain significance (MGUS) and patients with plasmacytoma. B-cell numbers were also significantly reduced in MM, MGUS and patients with plasmacytoma (p ≤ 0.005). Standard chemotherapy did not improve the number of mDC1 or pDC seen in the blood of patients with MM. The number of blood mDC1 improved transiently following auto hemopoietic stem cell transplantation, as numbers returned to within the normal range at engraftment and were maintained until D100. The number of blood mDC1 in patients taking thalidomide was also significantly higher than at relapse. These studies suggest that the defects in the B cell and blood DC pool is present in MGUS and plasmacytoma as well as patients with MM and can recover following therapy. [ABSTRACT FROM AUTHOR]

An 87 year old woman with dementia presented with multiple digitate hyperkeratoses on her face, limbs and chest. She had a previous diagnosis of a paraproteinaemia, with a serum monoclonal band of immunoglobulin G-κ and anaemia being detected. The skin biopsy of these hyperkeratotic lesions was characterized by multiple areas of orthokeratosis with immunoglobulin deposition. There was no association with hair follicles. These multiple digitate hyperkeratoses have been associated with underlying systemic disorders including malignancies. [ABSTRACT FROM AUTHOR]

Monoclonal gammopathy of uncertain significance (MGUS) may become symptomatic for autoimmune manifestations. We report on the prevalence and clinical course of immune thrombocytopenic purpura (ITP) observed in a consecutive series of 228 MGUS patients. At MGUS diagnosis, ITP was determined in 6/228 cases, accounting for a prevalence of 2630/100 000 [95% confidence interval (CI): 1210–5620]. One incidental ITP case occurred after 21 months of follow-up. After a follow-up of 681·3 patient-years, the crude incidence of ITP in MGUS was 146·8 per 100 000 patient-year (95% CI: 3·7–817·8). Overall, these observations point to an association between MGUS and ITP. [ABSTRACT FROM AUTHOR]

Martín P, Santón A & Bellas C (2004) Histopathology 44, 375–380 Neural cell adhesion molecule expression in plasma cells in bone marrow biopsies and aspirates allows discrimination between multiple myeloma, monoclonal gammopathy of uncertain significance and polyclonal plasmacytosis Differential diagnosis between multiple myeloma (MM), monoclonal gammopathy of uncertain significance (MGUS), and polyclonal plasmacytosis may be difficult in cases with not much bone marrow infiltration. Normal plasma cells express the antigens CD138, CD38, CD19, CD10 and D-related human leucocyte antigen (HLA-DR). Myelomatous plasma cells lack B lymphoid-associated markers and may express cell surface antigens associated with other haematopoietic lineages such as NCAM/CD56 (neural cell adhesion molecule). Recently, a monoclonal antibody, anti-CD56, has become available that can be used in fixed tissues embedded in paraffin, and it has been reported that osteoblastic cells of trabecular bone strongly express NCAM/CD56. We analysed NCAM molecule expression in 35 samples from patients with plasma cell disorders: 14 cases of MM, 16 cases of MGUS, and five cases of polyclonal plasmacytosis using immunohistochemistry in parallel in bone marrow core biopsies processed routinely and in bone marrow smears from the same patients. Of the MM samples 78% were CD56+ in smears and 92% positive in biopsies. We did not find strong CD56 expression in MGUS samples. One of five samples of polyclonal plasmacytosis was CD56+. A case was considered to be positive for CD56 expression if >50% of the CD138+ plasma cells expressed NCAM with an intensity on a par with that of the osteoblasts. We conclude that CD56 antibody is a very useful marker in the study of plasma cell proliferation in bone marrow biopsies and in bone marrow aspirates and is a great help in discriminating between MM, MGUS, and polyclonal plasmacytosis, especially in those cases with low infiltration. [ABSTRACT FROM AUTHOR]

A 53-year-old woman with necrobiotic xanthogranuloma presented as infiltrated eyelid plaques, with later development of arm and thigh lesions. Clinical features, including association with immunoglobulin G-κ paraprotein, and pathological findings were typical of this disorder. Treatment for 15 months with varying combinations of prednisone and multiple chemotherapeutic agents (melphalan, cyclophosphamide and chlorambucil) has led to minimal or no improvement in the clinical lesions or paraprotein. The case demonstrates some of the difficulties in managing this unusual disorder. [ABSTRACT FROM AUTHOR]

Responds to comments made by Jerry M. Winkler, Philip R. Greipp and Rafael Fonseca on the paper 'FGFR3 dysregulation in multiple myeloma: frequency and prognostic relevance,' which appeared in a 2002 issue of the 'British Journal of Haematology.' Proof of the presence of t(4;14) translocation in multiple myeloma patients; Reciprocal translocations involving the immunoglobulin genes.