Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Abnormal urinary proteins were investigated in 365 patients with serum M-components; 250 urines were available for study. All specimens were concentrated and processed by electrophoresis and immunoelectrophoresis. An overall frequency of Bence Jones proteinuria was found in 63%, whereas other M-components were detected in 18%. In patients with multiple myeloma, Bence Jones proteinuria occurred in 71%, while M-components in the form of whole molecules of immunoglobulins or as Fc and F'c fragments were observed in 20%. Bence Jones globulin was found in 60% of patients with IgG myeloma, 69% of those with IgA myeloma and 100% of patients with IgD myeloma and light chain disease. In regard to other diseases, Bence Jones proteinuria was found in 44% of patients with macroglobulinemia, in 60% of patients with nonplasmacytic neoplastic diseases and in 28% of patients with non-malignant conditions. Other M-components were detected in the urine of 13%, 14% and 6% respectively.The detection of abnormal urinary proteins in patients with M-components in the serum is an important diagnostic tool and may have prognostic significance.
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PMID:Anomalous urinary proteins in patients with serum M-components. 499 75

A series of 21 patients with IgD myeloma was studied retrospectively, to assess which parameter present at the time of diagnosis was of prognostic importance for survival and whether the clinical staging system of Durie and Salmon had predictive value for the survival time of these patients. Survival time did not appear to be correlated with haemoglobin concentration, thrombocytopenia, initial level of M-protein, amount of Bence-Jones proteinuria, hypercalcaemia, serum creatinine level, presence of osteolytic lesions or hepatosplenomegaly. Neither did the staging system of Durie and Salmon predict the survival time. It is concluded that clinical staging is of limited value in the management and prediction of the survival time of IgD myeloma patients.
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PMID:Prognostic factors in IgD myeloma: a study of 21 cases. 651 30

IgD myeloma is relatively rare. We wish to report 4 new cases investigated in this laboratory during the past 18 months. Extra-osseous involvement was present in 2 patients. Total serum protein concentrations were normal in all cases, while serum paraprotein peaks were inconspicuous in 2 patients. Bence Jones proteinuria of the lambda type was present in all, while free light chains could be detected in the blood in 3 patients.
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PMID:IgD myeloma. A report of 4 new cases. 706 15

Out of a group of 57 patients with the diagnosis of multiple myeloma fourteen (25%) with different degrees of renal disease were selected. Bence-Jones (BJ) proteinuria, infections, and, above all, the tumoral cell mass were the three main factors implicated in the development of myeloma associated renal disease. Only 13% of IgG myelomas presented with renal failure as compared to 27% of IgA myelomas. The patients with BJ and IgD myeloma, classically those with a higher tendency to develop renal disease (in our series 37% of BJ myelomas and 50% of IgD myelomas had renal disease), had the biggest tumoral cell mass of all patients studied. The relationship between tumor cell mass and renal disease in myeloma is supported by the recovery of renal function in a patient with chronic renal failure after a treatment-induced reduction of the tumoral cell mass from 1.71 to 0.82 x 10(12) cells/m2 body surface.
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PMID:[Renal disease in myeloma. Role of the tumor cell mass (author's transl)]. 725 52

On initial presentation of a patient with IgD multiple myeloma there were no features to suggest an unusual variant. Two months later she developed spinal cord compression due to an IgD plasmacytoma. This complication of IgD myeloma has rarely been reported. During the course of the disease and using the routine laboratory protocol for investigating and identifying paraproteins, including IgD, the patient's results became indistinguishable from those in Bence-Jones proteinuria myeloma.
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PMID:IgD multiple myeloma with thoracic spine compression due to epidural extra-osseous tumour spread. 808 29

Three patients suffering from IgD myeloma, which a rare variant of multiple myeloma which often has an aggressive course, were studied retrospectively in order to elucidate the existence of clinical or laboratory features in relationship to survival time. The patients were monitored in follow-up for a time variable for 8 to 52 months. All patients received courses of chemotherapy using an association of Melphalan and Prednisone (MP); one patient also received recombinant interferon alpha in association. Response to chemotherapy, with a > 50% reduction of serum M component, disappearance of Bence Jones proteinuria and permanent control of the disease was achieved in all patients. The median duration of survival in IgD myeloma is shorter than that currently observed in patients with other myeloma types: in our series one patient died 8 months after diagnosis but other two patients are still alive 8 and 52 months after diagnosis, respectively. Great difficulty was encountered in analysis of unfavourable prognostic clinical and laboratory data: in our series, in spite of the small number of cases, the Authors observe that only the relief of increased serum levels of Lactate Dehydrogenase (LDH) seem to be in relationship with a trend of shorter survival. The authors, confirming the particular clinical and laboratory aspects of this myeloma, stress that there may coexist cases in which standard chemotherapy failed to control the diseases: these seem to indicate neoplasia with fast growth kinetics. Further studies are necessary in order to identify new prognostic index which allows the identification of selected groups of patients who can profit from a combination chemotherapy regimen other than the standard MP association.
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PMID:IgD multiple myeloma. A report of three cases. 866 86

We reviewed the clinical features and outcome of 56 patients with myeloma and severe renal failure managed in a single institution over a 15-year period. Renal failure was recognized within 2 months of the diagnosis of myeloma in 75% of patients, and was the initial presentation of myeloma in 50%. Patients were staged by the Durie and Salmon classification. Light-chain and IgD myeloma accounted for 46% of cases, and Bence-Jones proteinuria was identified in > 90%. In 43%, a potential precipitant of renal failure was identified, usually hypercalcaemia or a non-steroidal anti-inflammatory agent. A preserved corrected calcium at presentation was characteristic (2.40 +/- 0.15 mmol/l, n = 42), even after excluding those with hypercalcaemia requiring specific intervention (n = 14, 2.76 +/- 0.51; p < 0.01): this finding in patients with unexplained acute renal failure should alert clinicians to the possibility of myeloma. Forty-seven patients (84%) required dialysis. Only seven (15%) ever regained renal function. Median survival (all patients) was 8 months. One-third died within 3 months of referral and one-third survived > 1 year. Hypoalbuminaemia and reduced platelet count at presentation were associated with reduced survival, but hypercalcaemia, infection, dialysis, (urgent or long-term), and dialysis modality were not. Chemotherapy was associated with increased survival, but progression of myeloma and infection were the two most frequent causes of death. Severe renal failure was associated with advanced myeloma stage and light-chain/IgD paraproteinaemia. Survival was related to severity of myeloma and not requirement for dialysis per se.
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PMID:Presentation and survival of patients with severe renal failure and myeloma. 953 42

Nonsecretory myeloma, which accounts for 1% to 5% of all myelomas, is characterized by the absence of detectable M-protein in serum and urine. The presenting features of nonsecretory myeloma are similar to those in patients with a detectable M-protein, except for the absence of renal function impairment. The response to therapy and survival of patients with nonsecretory myeloma are similar to those of patients with measurable M-protein. Immunoglobulin D myeloma represents 2% of all myelomas. Patients with IgD myeloma usually present with a small band or no evident M-spike on serum electrophoresis and heavy light-chain proteinuria. Thus, IgD myeloma can be considered a variant of Bence Jones myeloma; the presence of the IgD M-protein and the predominance of the lambda light chain are the only distinctive features. The median survival of patients with IgD myeloma is almost 2 years, with one fifth of them surviving for more than 5 years. Plasma cell leukemia is also a rare form of plasma cell dyscrasia (2% to 4% of all myelomas). The primary form accounts for 60% of the cases. In primary PCL, the constellation of adverse biologic prognostic factors in patients with advanced aggressive myeloma is already present at diagnosis. In fact, primary PCL has a more aggressive clinical presentation than MM, with a higher frequency of extramedullary involvement, anemia, thrombocytopenia, hypercalcemia, and renal failure. Treatment with a single alkylating agent plus prednisone is not appropriate. Combination chemotherapy with VAD, cyclophosphamide and etoposide, or VCMP/VBAP is a better initial option. Given the poor prognosis of primary PCL, intensification with high-dose therapy followed by stem cell rescue should be offered to affected patients.
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PMID:Nonsecretory myeloma, immunoglobulin D myeloma, and plasma cell leukemia. 1062 49

The study of IgD myeloma remains a challenging field. In terms of the initial detection of the IgD paraprotein, great care must be exercised in the interpretation of electrophoresis patterns and immunoglobulin profiles. Laboratory staff have a very important role to play in this, as it is likely that many IgD myeloma cases are uncovered following the involvement of laboratory staff. They must help to ensure that suggestive electrophoresis and immunoglobulin levels are properly investigated and that Bence Jones myeloma is not diagnosed without excluding the presence of an IgD paraprotein. In clinical terms, IgD myeloma remains a rare but aggressive tumour affecting younger people and with presenting features that include most of those common to all myeloma cases. However, renal problems, amyloidosis and the occurrence of Bence Jones lambda light chain proteinuria complicate matters to a far greater extent than in most other forms of the disease. There are now increasing numbers of case reports describing patients with associative symptoms and only time will tell whether these relationships are predictive or useful in nature. It is important however, given the rarity of the condition, that these cases continue to be reported. There do not appear to be any treatment regimes that are specifically tailored for IgD myeloma and the response to chemotherapy does not seem to differ from other forms of the disease. However, the management of any associated renal failure will always remain a challenge, I suspect. The progress being made in the treatment of myeloma as a whole, is bound to have a positive impact on the treatment of IgD myeloma.
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PMID:IgD myeloma: clinical, biological and laboratory features. 1246 46

Multiple myelomas (MM) of the immunoglobulin D (IgD) subtype is rare amongst plasma cell malignancies. It can present a diagnostic challenge because of the low amount of immunoglobulin in the serum. The amount of monoclonal (M)-protein is often undetectable on electrophoresis. Historically, survival in these patients was typically shorter compared to the immunoglobulin A (IgA) and immunoglobulin G (IgG) subtypes due to advanced disease upon presentation. With the advent of better diagnostic techniques, the prognosis of this disease is changing. We describe a case of an extramedullary testicular plasmacytoma (EMP) of the IgD subtype as the primary feature of MM, which responded well to novel therapy. A 72-year-old White male presented to the emergency room with a right testicular mass for three months. He subsequently underwent right radical orchiectomy. Pathology of the specimen revealed plasmacytoid cells positive for cluster of differentiation (CD79a), lambda free light chain, IgD, and BCL-1 (Cyclin D1) on immunochemical stains. Urine and serum immunofixation were positive for monoclonal IgD with lambda light chain specificity and Bence Jones proteinuria. Bone marrow biopsy showed large sheets of plasma cells with greater than 90% cellularity. Flow cytometry displayed atypical plasma cells expressing cluster of differentiation (CD38, CD20, and CD56) with cytoplasm and lambda light chain, approximately 20%, consistent with a plasma cell dyscrasia. Stage 3 IgD lambda multiple myeloma was diagnosed. He received novel treatment with Bortezomib and dexamethasone for three months, followed by Lenalidomide. His performance status and lab data improved significantly. He had progression-free survival (PFS) of approximately three years and remained in complete remission low-dose dose of Lenalidomide daily. IgD myeloma was considered a diagnostic challenge due to undetectable M-protein levels on serum protein electrophoresis (SPEP). With the advent of serum free light chain assay and serum and cytologic examinations, diagnostic accuracy has significantly improved. The IgD subtype is commonly associated with poor clinical outcomes. However, the use of novel agents and autologous transplant has changed the prognosis of this disease.
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PMID:An Extremely Rare Manifestation of Multiple Myeloma: An Immunoglobulin D Secreting Testicular Plasmacytoma. 2884 77


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