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Query: UMLS:C0020437 (hypercalcemia)
 10,293 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Findings of a 56 year old woman suffering from tumoral calcinosis, who was treated for 6 years, are presented. Under conditions known to lead to negative calcium-phosphorus balance, a reduction in tumor size was seen. Transient hypercalcemia was attributed to immobilization. The process of tumor reduction was not definitely accelerated by treatment with ethane-hydroxy-diphosphonate (EHDP; 500 mg/day for 20 months). Nephrotic syndrome as a consequence of amyloidosis developed. Amyloidosis seems to have resulted from the aseptic histiocytic inflammatory process in the tumors. The possible importance of high cholesterol in very low density lipoproteins in the serum of the patient is discussed.
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PMID:Tumoral calcinosis. Observations during six years. 677 68

Paraproteinemias can be subdivided in 1. obligatory paraproteinemias (myeloma, macroglobulinemia, heavy chain diseases); 2. accompanying paraproteinemias (Non-Hodgkin's lymphomas, myeloproliferative diseases, immune deficiency diseases, autoimmune diseases, transitory paraproteinemias after infection, paraproteinemias in association with nonlymphatic neoplasms); 3. benign paraproteinemias: a) with symptoms (primary amyloidosis, chronic cold agglutinin disease, paraproteinemias with further autoantibody function, monoclonal cryoglobulinemia); b) asymptomatic forms. Myeloma is the most common type of obligatory paraproteinemias. Characteristic findings are: Paraproteinemia and/or paraproteinuria in 98%, increase of plasma cells in the bone marrow in 84%, alterations in the roentgenograms of the skeleton in 79%. Clinical staging is of importance for the prognosis (amount of paraproteins, Hb level, renal disease, hypercalcemia, lytic lesions of bone). Neurologic complications, hemostasis dysfunction, cryopathies may be other symptoms. The terminal phase of the disease is determined by plasma cell proliferation, immune deficiency and renal disease or myelomonocytic leukemia. As to Non-Hodgkin's lymphomas the accompanying paraproteinemia is to be found in immunocytomas and in CLL. At last it has to be mentioned that B-cell disorders will influence the T-cell populations and vice versa.
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PMID:[Clinical aspects of monoclonal gammopathies in diseases of the lympho-plasmacytic cell system]. 681 57

The clinical, laboratory and pathologic findings were studied in 62 consecutively autopsied patients with multiple myeloma between 1954 and 1975. All patients were 40 years of age or older. Bone pain was the initial symptom in 2/3 of patients. Anemia (81%), thrombocytopenia (29%), azotemia (41%), hypercalcemia (46%) and hyperuricemia (52%), were common laboratory findings at diagnosis. Ninety-seven percent had a monoclonal protein in serum or urine. Extensive plasma cell replacement of marrow was invariably seen at autopsy although in 15% of patients no abnormality was found on skeletal survey. Extraskeletal spread (67%) was due to direct extension to paraosseous tissue resulting from cortical destruction and to distant organ involvement mainly of splenic red pulp and hepatic sinusoids. The patients were susceptible to bacterial infection, mainly gram-negative, of the lung (56%), urinary tract (35%), and blood (24%). Fungal infection was less frequent and usually consisted of superficial candidal overgrowth of gastrointestinal tract ulcerations (18%). Amyloidosis (10%) was perivascular and associated with light chain proteinuria. Renal failure as a cause of death (21%) was secondary only to infection (52%). Severity of histologic findings in the kidney at autopsy had little correlation to initial BUN concentration. The median survival was 11.5 months with alkylating agent therapy (responders, 29 months; non-responders, 6 months), and 6 months with urethan. Initial azotemia (greater than 80 mg/dl) and hypercalcemia (greater than 12 mg/dl) were important prognostic indicators (median survival, less than 1 month and 3 months, respectively). A good response to alkylating agent therapy, initial BUN less than 40 mg/dl and serum calcium less than 12 mg/dl were favorable to prognostic indicators.
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PMID:Multiple myeloma: a clinicopathologic study of 62 consecutively autopsied cases. 743 54

Multiple myeloma accounts for approximately 10% of hematologic cancers; it is characterized by an uncontrolled malignant growth of plasma cells occurring usually in the bone marrow or sometimes in other body sites as well. Malignant myeloma cells produce monoclonal immunoglobulins appearing as monoclonal spikes in the serum and/or urine. Patients with multiple myeloma suffer from various clinical features including bone destruction, bone marrow suppression, impaired renal function, hypercalcemia, serious infection, and amyloidosis. The combination of melphalan and prednisolone has been used as a standard therapy for this disease for over 30 years. The median survival of patients with multiple myeloma is 2.5-3 years, and only 10% of them survive longer than 10 years. To improve the prognosis for multiple myeloma, some strategies have been attempted. In this article, we introduce combination chemotherapy, interferon therapy, and bone marrow transplantation as newer approaches to therapy for multiple myeloma based on the biological characteristics of intractable multiple myeloma. Future therapeutic approaches are also discussed.
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PMID:[Current and future approaches to therapy for multiple myeloma]. 782 84

The renal concern in a multiple myeloma (MM) case has a frequency of 50% and causes a worsening of the disease with a survival average of about 12 months. The monoclonal light free chains (CLL) produced in excess by the plasmacytes are present in the urine as proteinuria of Bence Jones (PBJ) in 60-70% of patients affected by MM. They represent the major pathogenetic factor of the nephropathy in course of MM as they can deposit in shape of intratubular "casts" in the myeloma casts nephropathy (MCN). In some worse cases, dehydration or hypercalcaemia can cause an irreversible acute renal insufficiency (RI). It is therefore important in a patient affected with MM with PBJ to prevent, locate and opportunely treat these situations which worsen the nephropathy. Beside the tubular cast nephropathy, the CLL "accumulate" in the kidney even though with a lower frequency compared to MCN, in the light chains deposition disease (LCDD) and in the amyloidosis AL (AL). LCDD is characterized by a deposit of nodular amorphous materials PAS positive in the glomerulus and sometimes even in the tubulus. It usually presents itself as a chronic RI and a proteinuria causing nephrotic syndrome (NS). This quickly evolves into uraemia and its evolution can be lessened by the MM treatment. AL in course of MM also reveals with a chronic RI and NS. CLLs deposit in the typical fibrillar structure, on the vessel walls, in the glomerulus, in the mesangium and can be marked out with the Congo red colouring and the subsequent green birefringence through microscope with polarized light. Prognosis of AL is extremely severe and no benefit is given by the treatment of the hematological illness. It is therefore absolutely necessary to study the renal histology through biopsy when MM is grade B, that is, with serumal creatinine above 2 mg/dl as: MCN imposes the MM treatment programme in order to reduce the tubular excess of PBJ and to attempt to make RI reversible; MCN with tubular atrophy and interstitial fibrosis results in an unfavourable prognosis as it expresses a nephropathic irreversibility due to the loss nephrons. It will therefore necessary to start on a renal substitutional treatment programme. Renal damage in course of MM is not always tubular, rather an unexpected glomerular damage of LCDD or amyloidosis AL type can be found.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[The kidney in multiple myeloma. The physiopathological and clinical aspects]. 818 90

Renal insufficiency, which is present initially in almost half of patients with multiple myeloma, usually results from myeloma kidney or hypercalcemia. Neither the class of light chain nor the isoelectric point plays an important role in kidney failure. Acute renal failure must be treated with appropriate fluids and with electrolytes and hemodialysis if necessary. Plasma exchange may be helpful, but has not been proven as such. The presence of a nephrotic syndrome and a monoclonal kappa or lambda light chain in the urine almost always indicates primary amyloidosis (AL) or light-chain deposition disease. Amyloid fibrils must be distinguished from the fibrils of immunotactoid glomerulopathy.
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PMID:Monoclonal proteins and renal disease. 819 2

MGUS is characterized by a serum M-protein concentration of less than 30 milligrams (3 g/dl), fewer than 10% plasma cells in the bone marrow, no or only small amounts of M-protein (Bence Jones protein) in the urine, the absence of lytic lesions, anaemia, hypercalcaemia and renal insufficiency, and most importantly, stability of the M-protein and failure of the development of additional abnormalities. Electrophoresis on agarose, followed by immunoelectrophoresis or immunofixation for the identification of the type of M-protein, is recommended. In 1994, 971 patients at the Mayo Clinic were found with a serum M-protein. The most frequent diagnosis was MGUS, which occurred in 52% of patients. MGUS is found in approximately 3% of people older than 70 years and in at least 1% of those aged over 50. The incidence of monoclonal gammopathies increases with advancing age and is higher in African-Americans than in Caucasians. Two hundred and forty-one patients from the Mayo Clinic with a monoclonal gammopathy but no evidence of MM, macroglobulinaemia, amyloidosis, lymphoma or related disorders were followed for 24-38 years. In 62 patients (26%), multiple myeloma, macroglobulinaemia, amyloidosis or a malignant lymphoproliferative disorder developed (the actuarial rate of development of serious disease at 10 years was 16%; at 20 years, 33%; and at 25 years, 40%). Thirty patients (12%) were alive and had a stable M-protein value. In 23 patients (10%), the serum M-protein level increased to 30 milligrams (3 g/dl) or more, but they did not require therapy for myeloma or related disorders. Fifty-two per cent of patients (126) died of unrelated diseases without the development of a malignant plasma cell lymphoproliferative disorder. The actual rate of development of serious disease was the same for those with IgG, IgA and IgM M-proteins. Differentiation of MGUS from myeloma or macroglobulinaemia is difficult. The M-protein value must be measured periodically and clinical evaluation carried out to determine whether or not serious disease has developed.
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PMID:Monoclonal gammopathy of undetermined significance (MGUS). 884 71

The clinical spectrum of MM is variable. Infiltration of bone and bone marrow by malignant plasma cells results in severe osteopenia, lytic lesions, pathological fractures and anaemia. Occasionally, significant numbers of plasma cells circulate in the bloodstream. Hypercalcaemia and Bence Jones proteinuria are the main reasons for renal impairment, but amyloidosis and monoclonal immunoglobulin deposition should also be considered. Neurological impairment is most often due to spinal cord pressure by an extradural plasma cell tumour. In some patients, symptoms and signs of peripheral neuropathy may be present. Amyloidosis complicates the course of a minority of patients with MM and further impairs the performance of affected patients. Circulating monoclonal protein may increase serum viscosity, impair the function of platelets and coagulation factors, and behave as a cryoglobulin. The levels of uninvolved immunoglobulins are usually decreased, rendering patients susceptible to various bacterial infections. One or more of these complications provides a clue for the diagnosis, forms the basis for defining prognosis and must be managed expeditiously and concurrently, with the institution of specific treatment for the myeloma.
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PMID:Complications and supportive therapy of multiple myeloma. 884 76

Paraneoplastic manifestations are present in up to 20% of patients with renal cell carcinoma (RCC). There is convincing evidence that RCC tumor cells elaborate proteins that serve as mediators of endocrine (eg, ectopic production of parathyroid hormone-related protein or erythropoietin) as well as nonendocrine paraneoplastic syndromes. A paraneoplastic syndrome may be the initial clinical presentation of RCC in a significant number of patients, and recognition of these syndromes may facilitate early diagnosis. Most paraneoplastic syndromes associated with RCC remit after resection of the primary RCC or treatment of metastatic sites. The natural history of metastatic RCC is extremely variable. A significant proportion of patients may survive several years with slowly progressing metastatic disease. In these patients, the accurate diagnosis and management of paraneoplastic syndromes may be important in palliative management. Except for hypercalcemia, conventional medical therapies are seldom helpful. Other paraneoplastic manifestations of RCC include cachexia, fever, hepatic dysfunction, anemia, and amyloidosis, although our understanding of the underlying pathophysiology remains incomplete.
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PMID:Paraneoplastic manifestations of renal cell carcinoma. 894 20

MGUS is characterized by the presence of a serum M-protein less than 3 g/dL; fewer than 10% plasma cells in the bone marrow; no, or only small amounts of, M-protein in the urine; absence of lytic lesions, anemia, hypercalcemia, and renal insufficiency; and, most importantly, stability of the M-protein and failure of development of other abnormalities, MGUS is found in approximately 3% of persons older than 70 years and in 1% of those 50 years or older. During long-term follow-up, approximately one fourth of patients develop multiple myeloma (MM), amyloidosis, macroglobulinemia, or a similar malignant lymphoproliferative disorder. Actuarial rate of development of serious disease was 16% at 10 years, 33% at 20 years, and 40% at 25 years in our experience. The interval from recognition of the M-protein to the diagnosis of MM ranged from 2 to 29 years (median, 10 years). The size of the M-protein, hemoglobin value, percentage of bone marrow plasma cells, amount of light-chain excretion, presence of hypercalcemia or renal insufficiency, and presence of lytic bone lesions are often helpful in differentiating MGUS from MM and macroglobulinemia. The plasma cell labeling index and the presence of circulating plasma cells in the peripheral blood are indicators of active disease; however, there are no findings at the diagnosis of MGUS that reliably distinguish patients who will remain stable from those in whom a malignant condition will develop. Thus, a physician must perform serial measurements of the M-protein in the serum and periodic evaluation of the pertinent clinical and laboratory features to determine whether MM, macroglobulinemia, systemic amyloidosis, or related disorders have developed. Solitary plasmacytoma is characterized by the presence of a tumor consisting of monoclonal plasma cells identical to those in MM. In addition, skeletal roentgenograms must show no lytic lesions, a bone marrow aspirate must contain no evidence of MM, and immunoelectrophoresis or immunofixation of the serum and concentrated urine should show no M-protein. Exceptions to the presence of an M-protein occur, but therapy of the solitary lesion often results in disappearance of the M-protein. Tumoricidal irradiation (4000 to 5000 cGy) for approximately 4 weeks is the treatment of choice. Overt MM occurs in approximately 50% of patients with solitary plasmacytoma. Progression occurs in most patients within 3 years. The three patterns of failure are (1) development of MM, (2) local recurrence, and (3) development of new bone lesions in the absence of MM.
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PMID:Monoclonal gammopathy of undetermined significance and solitary plasmacytoma. Implications for progression to overt multiple myeloma. 908 Dec 5


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