ABSTRACT
Swallowing can be affected by a variety of systemic diseases. The etiology of dysphagia in the geriatric population is usually overlooked due mainly to a presumed diagnosis of presbyphagia or difficulty in revealing the direct cause. On the other hand, dysphagia can be a meaningful clinical sign of premalignant systemic disease. A 78-year-old man, without any prior medical or family history, was admitted with the chief complaint of dysphagia with recent aspiration pneumonia. Instrumental swallowing tests revealed a severe degree of dysphagia due to decreased laryngopharyngeal sensation and weakness of the pharyngeal constrictor muscles. Extensive workup, including electromyography and laboratory tests, revealed severe sensorimotor peripheral polyneuropathy related to monoclonal gammopathy. Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant precursor of multiple myeloma, which is characterized by the proliferation of monoclonal proteins. These conditions are often associated with peripheral polyneuropathy, ataxia, and sometimes even muscle weakness. Although dysphagia can occur in other systemic disorders, such as vasculitis or paraneoplastic syndrome-related malignancies, there are few reports of dysphagia related to MGUS. The patient was followed up for three years. The MGUS showed no further progression, but the patient showed no improvement, indicating a protracted clinical course and poor prognosis when dysphagia is related to MGUS.
Subject(s)
Aged , Humans , Ataxia , Deglutition , Deglutition Disorders , Diagnosis , Electromyography , Hand , Monoclonal Gammopathy of Undetermined Significance , Multiple Myeloma , Muscle Weakness , Muscles , Paraproteinemias , Pneumonia, Aspiration , Polyneuropathies , Prognosis , Sensation , VasculitisABSTRACT
Lymphoplasmacytic lymphoma (LPL) is a low-grade B-cell neoplasm, composed of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow and sometimes lymph nodes or spleen. LPL with bone marrow involvement and an IgM monoclonal gammopathy of any concentration is designated as Waldenström macroglobulinemia (WM). LPL associated with non-IgM monoclonal gammopathy or biclonal gammopathy is rarely observed. LPL diagnosis was based on clinical, morphological, and immunophenotypic findings. Recently, the test for L265P mutation of the myeloid differentiation factor 88 (MYD88) gene has been helpful in the diagnosis of LPL. Here, we reported the first case of LPL/WM with IgM-κ/IgA-λ biclonal gammopathy in Korea.
Subject(s)
B-Lymphocytes , Bone Marrow , Diagnosis , Immunoglobulin M , Korea , Lymph Nodes , Lymphocytes , Lymphoma , Multiple Myeloma , Myeloid Differentiation Factor 88 , Paraproteinemias , Plasma Cells , Spleen , Waldenstrom MacroglobulinemiaABSTRACT
Abstract Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy.
Subject(s)
Humans , Male , Middle Aged , Dermis/pathology , Scleromyxedema/pathology , Cell Proliferation , Fibroblasts/pathology , MucinsABSTRACT
An 8-year-old male Austrian Pinscher and a 14-year-old male Golden Retriever were presented for evaluation due to unexplainable high fructosamine values despite euglycemia and epistaxis in combination with polydipsia/polyuria, respectively. Blood analysis revealed severe hyperglobulinemia, hypoalbuminemia and markedly elevated fructosamine concentrations in both dogs. Multiple myeloma with IgA-monoclonal gammopathy was diagnosed by serum and urine electrophoresis including immunodetection with an anti-dog IgA antibody and bone marrow aspirations. Diabetes mellitus was excluded by repeated plasma and urine glucose measurements. Fructosamine values were positively correlated with globulin, but negatively correlated with albumin concentrations. These cases suggest that, as in human patients, monoclonal IgA gammopathy should be considered as a possible differential diagnosis for dogs with high fructosamine concentrations.
Subject(s)
Animals , Dogs , Male , Blood Proteins/analysis , Dog Diseases/blood , Fructosamine/blood , Immunoglobulin A/metabolism , Melphalan/therapeutic use , Multiple Myeloma/complications , Myeloablative Agonists/therapeutic use , Paraproteinemias/bloodABSTRACT
PURPOSE: To investigate the role of nitric oxide (NO) on the migration of cultured human Tenon's capsule PURPOSE: Necrobiotic xanthogranuloma is a very rare disease characterized by the presence of yellow subcutaneous skin lesions and systemic paraproteinemia. We report a case of post traumatic necrobiotic xanthograuloma that presented with a preseptal mass. CASE SUMMARY: A 31-year-old man presented with ptosis and a mass on the left superior orbit that developed after trauma 3 weeks prior to admission. A computed tomography scan showed a contrast-enhancing preseptal mass, and surgical exploration was performed. CONCLUSIONS: Histopathological examination revealed a tumor that had all the major features of necrobiotic xanthogranuloma, including fat necrosis with granulomatous inflammation, giant multinucleated cells, foamy cells, and several lymphoid follicles with germinal centers. Hematologic evaluation revealed polyclonal gammopathy.
Subject(s)
Adult , Humans , Fat Necrosis , Germinal Center , Inflammation , Necrobiotic Xanthogranuloma , Nitric Oxide , Orbit , Paraproteinemias , Rare Diseases , Skin , Tenon CapsuleABSTRACT
Paraproteinemia potentially causes peripheral neuropathy via an unknown underlying pathogenetic mechanism. We report a case of pathologically proven amyloid neuropathy with AL amyloidosis with an IgA kappa light chain, which was initially diagnosed as neuropathy associated with monoclonal gammopathy of undetermined significance. This case indicates that in cases of neuropathy with paraproteinemia, the other potential causes should be excluded by appropriate means, especially pathological evaluations.
Subject(s)
Amyloid Neuropathies , Amyloidosis , Immunoglobulin A , Monoclonal Gammopathy of Undetermined Significance , Paraproteinemias , Peripheral Nervous System Diseases , PolyneuropathiesABSTRACT
Hyperphosphatemia is an unusual manifestation in patients with multiple myeloma without a significantly reduced glomerular filtration rate. Serum phosphate may be falsely elevated when a large amount of paraproteins is present in the serum, because ultraviolet light absorbance is elevated with the phosphomolybdate ultraviolet assay, which is most commonly used for serum phosphate measurement. This pseudohyperphosphatemia can be confirmed by deproteinization of the serum of patients. We report a case of multiple myeloma presenting with spurious hyperphosphatemia revealing pseudohyperphosphatemia by deproteinization of serum using sulfosalicylic acid.
Subject(s)
Humans , Glomerular Filtration Rate , Hyperphosphatemia , Multiple Myeloma , Paraproteinemias , Paraproteins , Ultraviolet RaysABSTRACT
Necrobiotic xanthogranuloma(NXG) is a characteristic cutaneous manifestation associated with paraproteinemia. A case of NXG associated with an IgG (lambda) monoclonal gammopathy occurred in a 48-year-old man. Skin lesions were dome-shaped, hard palpable nodules, 2x3 cm to 4x4 cm sized, on both arms and forearms. They were arranged in a linear pattern. Also, hard palpable tumors, 1x2 cm to 2x3 cm in size, were present on the left leg and the dorsum of the left foot. They were violaceous, slightly protruded, and hard on palpation. Histologically, the lesion was characterized by inflammatory xanthogranuloma with broad hands of hyaline necrobiosis. Many foreign body type of bizarre giant cells, Touton type of giant cells and foamy histiocytes were infiltrated into the dermis and subcutaneous fat tissue. Three days after medication with ledercort and immuran, the cutaneous masses decreased in size and became soft.