Amyloid Tumors of Breast, Lung and RIB in Nonsecretory Multiple Myeloma
Gilles Grateau, Robert A. Kyle, Martha Skinner in Amyloid and Amyloidosis, 2004
In this case serum and urine protein electrophoresis were negative for monoclonal gammopathy. Serum immunoelectrophoresis was normal. Immunofixation of the concentrated urine revealed κ-light chains more likely consistent with a lowsecretory myeloma. Development of amyloid tumors in NSMM has been described only occasionally in the literature. Azar et al. described 123 patients with MM, seven patients of them had a NSMM and only one of whom developed amyloidosis [1]. The patient described above suffered from NSMM with AL amyloidosis affecting the breast and lung. AL amyloidosis is characterized by fibrils composed of the variable portion of a monoclonal light chain. Bladé et Kyle observed that in nonsecretory multiple myeloma the light chain is of κ-type in 75% similar to our case [2]. Amyloid deposits are known to have three major components: the amyloid fibril protein, the amyloid P- component and glycosaminoglycans (GAGs). GAGs are important in the amyloidogenic pathway. They accelerate the formation of fibrils and protect them against proteolytic degradation [4]. The CEC method with constant toluidine blue concentration but variable salt (MgCl2) concentration serves to show various GAGs within the amyloid deposits: hyaluronic acid (0,1M), chondroitin sulphate (0,5M), keratan sulphate (1M) and heparan sulphate (1,8/1,9 M).
Differential diagnoses of systemic sclerosis
Aparna Palit, Arun C. Inamadar in Systemic Sclerosis, 2019
Scleromyxedema (Figure 5.5) is a primary cutaneous mucinosis characterized by diffuse or localized dermal mucin infiltration leading to a thickening and a smooth waxy appearance of the skin. The infiltration is often accompanied by smooth waxy, pruritic, closely-set papules. Most of the cases are associated with monoclonal gammopathy. The typical sites involved are the mid-upper back and forehead with deep glabellar furrows. Involvement of the face, forearms, and hands gives a smooth ironed-out appearance, as in SSc. However, such an appearance in SSc is due to sclerosis rather than infiltration. Further, reduced mouth opening and sclerodactyly due to infiltration also simulates SSc.17 Severe esophageal dysmotility due to mucin infiltration of the esophageal walls leading to significant dysphagia has also been reported with scleredema.20
B Cells and Humoral Immunity
Constantin A. Bona, Francisco A. Bonilla in Textbook of Immunology, 2019
This gammopathy results from proliferation in the bone marrow of IgM-secreting plasmacytoid cells. The clinical presentation is non-specific, most often generalized weakness, fatigue, and weight loss. Diagnosis is made by detecting the M component in immunoelectrophoresis. Common associated symptoms are anemia and hepatosplenomegaly. Bence-Jones proteinuria occurs in about 10% of patients. Osteolytic bone lesions are very rare. The disease is relatively indolent, and even though incidence is rarely before age 60, the mean survival after diagnosis is three to five years, and occasionally ten years or more. Death attributable to this disease is usually due to increased blood viscosity. This can lead to renal failure, hemorrhagic purpura of mucous membranes (paraproteinemia may interfere with coagulation), and occasionally congestive heart failure.
Case report: gastric plasmacytoma resistant to radiation therapy
Published in Postgraduate Medicine, 2022
An 83-year-old woman was noted by her primary care physician to have elevated total serum protein on routine labwork, and subsequent serum immunofixation confirmed IgG/Kappa paraprotein with normal hemoglobin, renal function, and calcium. She was referred to an oncologist, who noted gradually increasing total serum protein. On evaluation, there was no evidence of end-organ damage characteristic of myeloma: hypercalcemia, renal dysfunction, anemia, or bone lesions. The patient was diagnosed with monoclonal gammopathy of undetermined significance with IgG/Kappa monoclonal protein and was placed on observation. Seven years later, she was noted to have mild anemia. Because of her increasing M spike and the possibility of smoldering myeloma, she was referred to the NIH for a smoldering myeloma clinical trial.
Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review
Published in Expert Review of Cardiovascular Therapy, 2018
Noor Ul-Ain Baloch, Marvi Bikak, Abdul Rehman, Omar Rahman
A role of immune system dysregulation in the pathogenesis of SCLS has also been suggested by some reports. An increase in the number of circulating CD25+ T cells was observed in some patients with SCLS [34,35]. Furthermore, skin biopsies taken from patients with SCLS have shown perivascular infiltration with mononuclear cells [36]. However, in nearly all cases, complement or immunoglobulin deposition was absent on immunofluorescence staining [37]. Nevertheless, a clear association between SCLS and monoclonal gammopathy of undetermined significance (MGUS) has been described in the literature. Amoura et al. (1997) reported a cohort of 13 patients with SCLS, of which all 13 (100%) patients had evidence of MGUS [38]. In the cohort of Kapoor et al. (2010), 76% of patients with SCLS had MGUS [9]. Druey and Greipp reported in their narrative review that approximately 79–82% of patients with SCLS have associated MGUS [6]. In the systematic review by Eo et al. [2], 88% of patients were noted to have immunoglobulin G (IgG) MGUS with κ-light chain IgG-MGUS in 64.2% and λ-light chain IgG-MGUS in 22.1% cases. In addition, immunoglobulin A (IgA) MGUS was detectable in 2.1% of cases, while biclonal gammopathy and triclonal gammopathy were only noted in one and two patients, respectively.
Enhancing prognostication and personalizing treatment of extranodal marginal zone lymphoma
Published in Expert Review of Hematology, 2023
Juan Pablo Alderuccio, Izidore S. Lossos
Analyzing the University of Miami EMZL dataset, we found shorter PFS (HR = 1.82, P = 0.016) and OS (HR = 2.25, P = 0.033) in patients presenting with monoclonal gammopathy (MG) at diagnosis [58]. MG was present in 10.7% of the patients and was characterized by predominance of IgG as the most common paraprotein. In other series, IgM has been described as the most common MG in EMZL, but this difference could be secondary to difference in EMZL disease location, analysis of small cohorts, and possible inclusion of patients with lymphoplasmacytic lymphomas which occasionally may be difficult to differentiate from EMZL [63,64]. We also observed a higher incidence of MG in patients with gastrointestinal (GI)-non gastric (12.5%) and multiple mucosal sites (MMS) (11.6%) presentations. Other studies support this observation, associating the presence of MG at diagnosis with worse outcome in EMZL [65].
Related Knowledge Centers
- Antibody
- Globulin
- Hepatitis A
- Measles
- Serum Protein Electrophoresis
- Immunodeficiency
- X-Linked Agammaglobulinemia
- Hyper Igm Syndrome
- Vaccine
- Immune Thrombocytopenic Purpura