Diseases of the peripheral nervous system are one of the most difficult subjects in neurology partly in that specific etiologies are often not found. Among peripheral neuropathies of unknown causes, an association of monoclonal gammopathies has been observed. Monoclonal gammopathies are disorders characterized by proliferation of a single clone of plasma cell that produces monoclonal proteins. Here we report two cases of chronic sensorimotor polyneuropathy with monoclonal gummopathy of undetermined significance, who showed no evidences of multiple myeloma, macroglobulinemia, systemic amyloidosis, or other neoplasms. One case is also associated with hyperthyroidism and cryoglobulinemia.
Amyloidosis ; Clone Cells ; Cryoglobulinemia ; Hyperthyroidism ; Monoclonal Gammopathy of Undetermined Significance* ; Multiple Myeloma ; Neurology ; Paraproteinemias* ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Plasma Cells ; Polyneuropathies* ; Waldenstrom Macroglobulinemia

No abstract available.
Cryoglobulinemia* ; Gangrene* ; Monoclonal Gammopathy of Undetermined Significance* ; Paraproteinemias*

BACKGROUND: Plasma cell neoplasm is diagnosed by performing bone marrow examination, serum- and urine-protein electrophoresis, and quantification of free light chains of immunoglobulins. We characterized and quantified monoclonal proteins typical of different diagnosed conditions to determine the best screening test(s). METHODS: We retrospectively reviewed diagnosis of and the characteristics of monoclonal proteins from 113 patients with monoclonal gammopathy. Monoclonal proteins were detected by agarose-gel electrophoresis and capillary electrophoresis, and if the results were ambiguous, they were confirmed by immunofixation electrophoresis. Free light chains were measured using nephelometry. RESULTS: The concentrations of monoclonal proteins in 113 patients with different conditions were as follows: multiple myeloma (MM) (67%), 2.66 (0.87-9.48) g/dL; monoclonal gammopathy of undetermined significance (MGUS) (26%), 0.62 (0.08-2.95) g/dL; lymphoma (3%), 3.65 (1.59-6.54) g/dL; Waldenstrom's macroglobulinemia (2%), 1.99 (1.08-2.90) g/dL; amyloidosis (2%), 0.61 g/dL; and POEMS syndrome (1%), 0.99 g/dL. There was a significant difference in the concentration and kappa/lambda ratio (which was based on the immunetype of the monoclonal proteins) of the monoclonal proteins in patients with MM and MGUS (P<0.001 and P=0.004, respectively). The diagnostic sensitivity of serum-protein electrophoresis, free-light-chain assay, and bone marrow analysis was 87.6%, 84.1%, and 84.5%, respectively. The sensitivity of a combination of 2 or 3 of these tests was higher at 100%. CONCLUSIONS: A combination of protein electrophoresis with immunotyping and serum free-light-chain assay may be the best screening method for detecting monoclonal proteins since its non-invasiveness.
Amyloidosis ; Bone Marrow ; Bone Marrow Examination ; Electrophoresis ; Electrophoresis, Capillary ; Humans ; Immunoglobulins ; Light ; Lymphoma ; Mass Screening ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Neoplasms, Plasma Cell ; Paraproteinemias ; Plasma ; Plasma Cells ; POEMS Syndrome ; Proteins ; Retrospective Studies ; Waldenstrom Macroglobulinemia

No abstract available.
Leukemia, Biphenotypic, Acute ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Paraproteinemias ; Thrombocythemia, Essential

OBJECTIVE: To investigate several abnormal genes by the fluorescence in situ hybridization (FISH) in multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS) and reactive plasmacytosis (RP), and to increase the diagnosis and differential diagnosis levels for these common plasma diseases. 
 METHODS: The clinical manifestations, image and laboratory tests and the FISH detection were retrospectively analyzed in 61 cases of newly diagnosed MM, 20 cases of MGUS and 20 cases of RP from August, 2012 to February, 2015 in the Xiangya Hospital of Central South University. 
 RESULTS: Fifty cases among 61 MM patients showed genetic abnormality by FISH technology. The total positive rate was 81.9%. Among them, 19 cases (31.1%) had 1q21 amplification, 18 cases (29.5%) lacked D13S319, 10 cases (16.4%) missed RB1, 10 cases (16.4%) had IGH translocation and 7 cases (11.4%) lacked p53 gene. The positive rate for two or more genes abnormal was 19.8% in 12 cases. However, in 20 cases of MGUS patients, the positive detection rate was 25%, including 4 cases (20%) of 1q21 augmentation and 2 cases (10%) of IGH translocation. There were not two or more abnormal genes in one case. While in RP cases, only 1 case of patients had D13S319 abnormal gene, and the positive rate was only 5%. There was significant difference (P<0.05) among the 3 groups. 
 CONCLUSION The positive detection rate is 81.9% in MM patients by FISH, which is significantly higher than that in patients with MGUS or RP. FISH technology can detect a variety of abnormal genes in MM. It is useful for the differential diagnosis and prognosis for MM, MGUS and RP.
Humans ; In Situ Hybridization, Fluorescence ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Paraproteinemias ; Retrospective Studies ; Translocation, Genetic

No abstract available.
Monoclonal Gammopathy of Undetermined Significance* ; Paraproteinemias* ; Vasculitis

Monoclonal gammopathies are associated with a wide range of renal diseases, including cast nephropathy, light chain amyloidosis, monoclonal immunoglobulin deposition diseases, and so on. We describe seven cases of monoclonal gammopathies involving kidney. The mean age was 61.6+/-3.6 years and male to female ratio was 1:1.3. Among 7 patients, diagnoses were cast nephropathy with light chain deposition disease, two light chain deposition diseases, three light chain amyloidosis and light chain deposition disease with light chain amyloidosis. Two cases were monoclonal gammopathy of undetermined significance and three cases were multiple myeloma in five cases underwent bone marrow biopsy. It showed that renal function was severly decreased in light chain deposition disease. It is clear that monoclonal gammopathies show various renal disease and clinical course in our cases. It is necessary to do renal biospy for adequate diagnosis and treatment even to old patients suspecting monoclonal gammopathy.
Amyloidosis ; Biopsy ; Bone Marrow ; Diagnosis ; Female ; Humans ; Immunoglobulins ; Kidney* ; Male ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Paraproteinemias*

Renal diseases with organized deposits include amyloid, fibrillary, immunotactoid, and cryoglobulinemic glomerulopathies. AL amyloidosis and fibrillary glomerulonephritis are different in the composition of their immunoglobulin deposits. Fibrils of fibrillary glomerulonephritis are usually composed of polyclonal, occasionally oligoclonal or monoclonal, immunoglobin G, but amyloidosis consists of monoclonal light chains. Simultaneous occurrence of fibrillary glomerulonephritis and AL amyloidosis is very rare. We report a case of fibrillary glomerulonephritis combined with AL amyloidosis in a 71-yr-old man.
Amyloid ; Amyloidosis ; Glomerulonephritis ; Immunoglobulins ; Light ; Monoclonal Gammopathy of Undetermined Significance ; Paraproteinemias

Humans ; Paraproteinemias ; Monoclonal Gammopathy of Undetermined Significance ; Kidney ; Mass Spectrometry

Anemia ; Humans ; Male ; Middle Aged ; Monoclonal Gammopathy of Undetermined Significance ; Paraproteinemias