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Nephrology (Carlton, Vic.) Dec 2007The primary glomerulopathies with a deposit of ultrastructural fibrillary structure, which are negative for Congo-red stain but positive for immunoglobulins, include... (Review)
Review
The primary glomerulopathies with a deposit of ultrastructural fibrillary structure, which are negative for Congo-red stain but positive for immunoglobulins, include fibrillary glomerulonephritis and immunotactoid glomerulopathy. Several paraproteinemias, including cryoglobulinemia, monoclonal gammopathy and light chain deposition disease as well as haematopoietic disorders including plasmacytoma, plasma cell dyscrasia and B cell lymphoproliferative disorders involve glomerulopathy with an ultrastructural fibrillary structure. A rare glomerulopathy with fibrillary structure showing negative stain for Congo-red and for immunoglobulins has been also reported. The pathological diagnoses of these glomerulopathies with ultrastructural fibrillary deposits can include either glomerular diseases, or paraproteinemic diseases, or haematopoietic diseases. The terminology is still confusing when glomerular diseases can be combined with paraproteinemic diseases and/or haematopoietic diseases. Therefore, the generic term, 'glomerular deposition disease', has been proposed by pathologists with a requirement for clinicians to detect autoantibodies, paraproteins and to carry out a bone marrow check. An attempt has been made to elucidate the correlation among the glomerular deposition disease, paraproteinemia and haematopoietic disorder.
Topics: Glomerulonephritis; Humans; Kidney Glomerulus; Lymphoproliferative Disorders; Paraproteinemias
PubMed: 17995523
DOI: 10.1111/j.1440-1797.2007.00877.x -
Der Internist Jan 2012Amyloidoses are rare protein folding disorders, in which proteins are deposited as insoluble fibrillar aggregates due to a conformational change. This can occur in a...
Amyloidoses are rare protein folding disorders, in which proteins are deposited as insoluble fibrillar aggregates due to a conformational change. This can occur in a local or systemic form. Systemic amyloidoses are life-threatening complications of monoclonal gammopathy, chronic inflammatory diseases or within hereditary diseases. The causative treatment of amyloidosis is the reduction of the amyloid precursor protein by chemotherapy, anti-inflammatory treatment, or liver transplantation. Early diagnosis of the disease is essential in order to effectively treat patients and avoid further deterioration of organ functions.
Topics: Amyloidosis; Humans; Inflammation; Paraproteinemias
PubMed: 22033913
DOI: 10.1007/s00108-011-2952-y -
Neurologic Clinics Feb 2007Paraproteinemia frequently is associated with peripheral neuropathy. The clinical manifestations can be protean owing to the potential for multiple organ involvement. A... (Review)
Review
Paraproteinemia frequently is associated with peripheral neuropathy. The clinical manifestations can be protean owing to the potential for multiple organ involvement. A methodical diagnostic approach to patients who have a plasma cell dyscrasia and neuropathy is necessary to ensure the appropriate detection of more widespread systemic involvement.
Topics: Amyloidosis; Demyelinating Diseases; Humans; Immunoglobulin A; Immunoglobulin E; Immunoglobulin G; Immunoglobulin M; Multiple Myeloma; Paraproteinemias; Peripheral Nervous System Diseases; Polyradiculopathy; Waldenstrom Macroglobulinemia
PubMed: 17324720
DOI: 10.1016/j.ncl.2006.12.002 -
Amyloid : the International Journal of... Sep 2001A case of 79 year-old man suffering from nephrotic syndrome, infiltrative cardiomyopathy and sensitive neuropathy of the lower limbs, associated with biclonal gammopathy... (Review)
Review
A case of 79 year-old man suffering from nephrotic syndrome, infiltrative cardiomyopathy and sensitive neuropathy of the lower limbs, associated with biclonal gammopathy (IgG K and IgA A), is described. There was a history of non-insulin dependent diabetes mellitus and of two lung nodules considered as benign lesions on the basis of cytologic, hematologic and instrumental examination. A rectal biopsy positive for amyloid deposition (Congo red histology and immunofluorescence study) led to the diagnosis of AL amyloidosis. Considering that the patient did not fulfill diagnostic criteria for lymphoproliferative diaseases (myeloma, lymphoma or Waldenström's macroglubulinemia), nor for secondary malignant paraproteinemia, a diagnosis of idiopathic AL amyloidosis with biclonal gammopathy was made. Very few cases of idiopathic AL amyloidosis with double component are reported in the literature. Our review suggests that idiopathic AL amyloidosis with biclonal gammopathy is similar to idiopathic AL amyloidosis with monoclonal paraproteinemia in terms of clinical features, response to therapy and prognosis. Further studies, however, are necessary to clarify the true incidence and the clinical features of idopathic AL amyloidosis associated with biclonal gammopathy.
Topics: Aged; Amyloid; Amyloidosis; Humans; Immunoglobulin A; Immunoglobulin G; Immunoglobulin Idiotypes; Male; Paraproteinemias; Prognosis
PubMed: 11676298
DOI: 10.3109/13506120109007364 -
Journal of the American Academy of... Dec 2017Cutaneous manifestations secondary to paraprotein deposits in the skin include a group of different disorders that although rare, may be the first clinical manifestation... (Review)
Review
Cutaneous manifestations secondary to paraprotein deposits in the skin include a group of different disorders that although rare, may be the first clinical manifestation of the underlying hematologic dyscrasia. In this article we review the clinical manifestations and histopathologic findings of the processes that result from specific deposition of the paraprotein in different structures of the skin. Paraneoplastic processes frequently associated with hematologic malignancies will not be covered in this review. Some of the disorders included here result from deposition of the intact paraprotein in the skin, whereas in other cases the lesions are due to deposition of modified paraproteins in the form of amyloid substance, cryoglobulins, or crystalglobulins. Cutaneous amyloidoma refers to nodular dermal deposits of amyloid derived from immunoglobulin light chains produced by local plasma cells in the absence of systemic amyloidosis. Dermatologists and dermatopathologists should be aware of the clinical and histopathologic features of these rare disorders because sometimes the cutaneous lesions are the first sign of an underlying silent hematologic malignancy with paraproteinemia.
Topics: Humans; Paraproteinemias; Paraproteins; Skin; Skin Diseases
PubMed: 28985955
DOI: 10.1016/j.jaad.2017.07.039 -
American Journal of Kidney Diseases :... Mar 2018
Topics: Aged; Blood Urea Nitrogen; Clinical Laboratory Techniques; Creatinine; False Positive Reactions; Glomerular Filtration Rate; Humans; Male; Paraproteinemias; Sensitivity and Specificity
PubMed: 29477177
DOI: 10.1053/j.ajkd.2017.09.027 -
Klinische Monatsblatter Fur... 2002Necrobiotic xanthogranuloma is a rare disorder which predominantly affects the periorbital region and is frequently associated with paraproteinemia and... (Review)
Review
BACKGROUND
Necrobiotic xanthogranuloma is a rare disorder which predominantly affects the periorbital region and is frequently associated with paraproteinemia and lymphoproliferative diseases.
PATIENT AND METHODS
A 49-year old woman presented with bilateral, yellowish, subcutaneous lesions involving all four eyelids. These lesions have developed over the last year. Additionally, similar skin lesions developed on the trunk and the lower extremities. Two years ago, the patient had a bilateral episcleritis. An incisional biopsy was taken form the lesion. In addition, we reviewed the literature concerning similar cases.
RESULTS
Histologic examination revealed a granulomatous process consisting of histiocytes, foamy cells, multinucleated giant cells (touton- and foreign body-type) and associated with necrobiotic collagen and cholesterol clefts. General examination revealed the presence of a paraproteinemia of the IgG-typ associated with a hyperlipidemia. The patient was treated with a low dose chemotherapy which resulted in improvement of signs and symptoms. We could identify in the literature 75 similar cases. The mean age of those patients was 53 years.
CONCLUSION
The clinical and histologic findings in our patient were typical for a necrobiotic xanthogranuloma with associated paraproteinemia. The most favorable treatment response has been reported with low doses of systemic administered chemotherapeutic agents in combination with radiation therapy; however, the mortality due to underlying diseases is high.
Topics: Biopsy; Eyelid Diseases; Eyelids; Female; Granuloma, Giant Cell; Humans; Hyperlipidemias; Immunoglobulin G; Middle Aged; Necrobiosis Lipoidica; Paraproteinemias; Xanthomatosis
PubMed: 11932812
DOI: 10.1055/s-2000-23502 -
Journal of Neurology 1985Paraproteinaemias are frequently associated with peripheral neuropathies. "Benign" paraproteinaemia, myeloma and Waldenströms macroglobulinaemia may present clinically... (Review)
Review
Paraproteinaemias are frequently associated with peripheral neuropathies. "Benign" paraproteinaemia, myeloma and Waldenströms macroglobulinaemia may present clinically as polyneuropathy. Therefore immunoelectrophoresis is strongly recommended in the routine diagnosis of polyneuropathies of unknown origin. Peripheral neuropathies associated with paraproteinaemia are clinically, electrophysiologically, pathologically and probably also pathogenetically heterogeneous. There are subgroups such as demyelinating neuropathy associated with IgM paraproteinaemia, which show quite distinctive features. This survey describes the different types of paraproteinaemia and their associated peripheral neuropathies. The incidence, pathogenesis and therapy of peripheral neuropathy associated with monoclonal gammopathies are discussed.
Topics: Aged; Blood Viscosity; Cryoglobulinemia; Electrophysiology; Humans; Immunoglobulin G; Immunoglobulin M; Middle Aged; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Paraproteinemias; Peripheral Nervous System Diseases; Waldenstrom Macroglobulinemia
PubMed: 2995596
DOI: 10.1007/BF00313781 -
The Journal of Clinical Investigation Nov 1976Many of the clinical features of paraproteinemia result from impairment of blood flow through the vascular tree because of blood hyperviscosity. Studies were carried out...
Many of the clinical features of paraproteinemia result from impairment of blood flow through the vascular tree because of blood hyperviscosity. Studies were carried out in 65 patients with serum paraproteins (31 with IgG, 25 with IgM, and 9 with IgA) to examine the relationship between the blood viscosity and the frequency of selected clinical features. The blood and plasma viscosities were measured at low rates of shear. Blood hyperviscosity was present in 91% of the patients and plasma hyperviscosity in 75% of the patients. In each of the three immunoglobulin classes both the blood and plasma viscosities increased logarithmically with the paraprotein concentration being greatest in the case of IgM. In addition, the relationship between the hematocrit and the logarithm of blood viscosity tended to be linear at any given protein concentration. In patients with very high levels of paraprotein the blood viscosity was modified by low hematocrits; the latter was below 30 in 70% of patients in whom the concentration of paraprotein was above 4 g/100 ml. The prevalence of clinical complications involving the retinal circulation, the peripheral vascular system, and the central nervous system increased markedly with increasing blood viscosity, measured at 0.18 S-1. One or more of these regions was affected in greater than 80% of patients with blood viscosity above 60 centipoise and in less than 23% of patients with blood viscosity below 40 centipoise. These observations illustrate the complex relationship between blood viscosity, concentration of paraprotein, immunoglobulin class and hematocrit, and emphasize the importance of measuring the whole blood viscosity at low rates of shear in determining the risk of vascular complications.
Topics: Blood Viscosity; Hematocrit; Humans; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Paraproteinemias; Paraproteins; Plasma Volume
PubMed: 993338
DOI: 10.1172/JCI108568 -
Muscle & Nerve Jan 2015The paraproteinemias are a heterogeneous group of disorders in which monoclonal plasma cells cause the proliferation of monoclonal proteins. They are of importance to... (Review)
Review
The paraproteinemias are a heterogeneous group of disorders in which monoclonal plasma cells cause the proliferation of monoclonal proteins. They are of importance to clinicians because they often occur in association with neuropathies. Neurologists play a particularly important role when the neuropathy is the presenting feature, in which case they may uncover clinical, laboratory, radiologic, electrodiagnostic, or biopsy findings that lead to identification of the underlying paraproteinemia. The frequency of neuropathies in these patients, and the extent to which such neuropathies dominate the clinical picture, varies significantly between the different paraproteinemias. Treatments may be aimed specifically at the neuropathy, or against the underlying hematologic disorder. In all patients with paraproteinemias, the neurologist can work collaboratively with the hematologist to formulate therapeutic plans and goals and can provide follow-up and monitoring to determine the response of the neuropathy to treatment.
Topics: Humans; Immunoglobulin M; Immunoglobulins; Myelin-Associated Glycoprotein; Paraproteinemias; Peripheral Nervous System Diseases
PubMed: 25288371
DOI: 10.1002/mus.24471