Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020437 (hypercalcemia)
10,293 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical features, occurrence and reversibility of renal failure, response to treatment and survival duration in 190 multiple myeloma patients were reviewed in a retrospective study based on hospital records. Renal failure was present in 27% of the patients and constituted an important prognostic factor. Reversibility of renal failure occurred in 52% of the patients and was more frequent in those with low serum creatinine, hypercalcaemia, high erythrocyte sedimentation rate and high level of IgA. Response to chemotherapy resulted in a prolonged survival, whereas recovery of renal function did not significantly affect survival.
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PMID:[Renal insufficiency in myelomatosis. Prognostic factors and survival]. 919 25

Approximately 20% of patients with multiple myeloma are recognized by chance without significant symptoms. In order to prevent morbidity with timely therapy, reliable criteria are needed that distinguish those likely to show early or late disease progression. Multiple clinical features were assessed in 101 consecutive, asymptomatic and previously untreated patients. Patients with one or more lytic bone lesions were excluded because this feature had been found previously to be associated with early progression. Multivariate analysis indicated that only serum myeloma globulin > 30 g/l, IgA protein type, and Bence Jones protein excretion > 50 mg/d remained as significant independent variables. The presence of two or more of these features signified high-risk disease with early progression (median 17 months) whereas the absence of any adverse variable was associated with prolonged stability (median 95 months) (P < 0.01). Magnetic resonance (MR) imaging of the spine was useful only in patients with one adverse feature and an intermediate time to progression (median 39 months). An abnormal pattern (40% of patients) helped to distinguish patients with an imminent complication from those with more stable disease. Because a serious complication (fracture, hypercalcaemia) occurred in 35% of patients with early disease progression, chemotherapy seems justified for selected patients with asymptomatic disease at diagnosis. The remaining patients were at such low risk for progression (median 6 years) that they may be followed safely at long intervals without treatment.
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PMID:Prognostic features of asymptomatic multiple myeloma. 921 81

A 62-year-old woman with IgA-lambda type monoclonal gammopathy had been followed up since January 1988. In March 1991, multiple myeloma (IgA-lambda) was diagnosed on the basis of bone marrow biopsy findings and increased serum IgA levels. She was treated intermittently with melphalan and prednisolone over a perioa of about 6 years, but was eventually admitted due to renal dysfunction, hypercalcemia, increased serum IgA and the formation of subcutaneous masses. During chemotherapy she underwent emergency surgery for obturative ileus. Histological examination of the resected tissues revealed invasion of myeloma cells into the small intestine and peritoneum. Despite continued chemotherapy, the patient's soft tissue masses enlarged, and new lesions appeared in other organs. In the terminal stage, lower serum IgA levels were observed despite an increase in Bence-Jones protein levels in urine. The patient died five months after admission. An autopsy found infiltration by atypical myeloma cells in multiple organs. An immunohistochemical examination revealed and increase in lambda-light chain positive cells relative to the number of alpha-heavy chain positive cells. The terminal course was considered to be representative of aggressive phase multiple myeloma. The case was rare in that the patient's ileus was caused by invasion of myeloma cells into the small intestine.
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PMID:[A fatal case of aggressive-phase multiple myeloma with ileus and invasion into extramedullary organs]. 963 89

We report on a 61-year-old woman with coexisting early stage primary gastric plasmacytoma and sarcoidosis with hypercalcaemia. Laboratory data on admission showed hypercalcaemia, with 12.8 mg/dl, parathyroid hormone-related peptide (PTHrP) 1.2 pmol/l, C-PTHrP 69.5 pmol/l, and 1,25-dihydroxyvitamin D3 46.7 pg/ml. Neoplastic plasma cells proliferated in the propria mucosa of the stomach, showed a monoclonal immunoglobulin of cytoplasmic IgA (lambda light chain) and were positive for leucocyte common antigen and epithelial membrane antigen on paraffin section prepared from a stomach biopsy specimen. Russel bodies were present, as were crystals. Abundant sarcoid granulomas were observed in many of the regional lymph nodes around the stomach and in the dermis of a skin nodule. The patient underwent subtotal gastrectomy with administration of antimyeloma chemotherapy. We suggest that the hypercalcaemia in this patient was due to PTHrP production by neoplastic plasma cells.
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PMID:Coexisting primary early gastric plasmacytoma and sarcoidosis with hypercalcaemia. 964 49

Monoclonal gammapathies are detected because of clinical symptoms and biological tests confirm their presence. Wishing to investigate these diseases, we carried out a series of biochemical tests on 14 patients from October 1995 to July 1996: protein, cryoglobulin, electrophoresis of proteins, proteinuria of BENCE JONES, C-reactive protein, weight measuring of immunoglobulins (Ig), immunofixation of Ig, creatinine and calcium. The results we obtained confirmed the presence of 14 cases of myeloma with: -9 IgG myelomas with 6 kappa light chains and 3 lambda light chains -4 IgA myelomas with 2 kappa light chains and 2 lambda light chains -1 IgG kappa, Ig lambda biclonal gammapathy united to a cryoglobulin of class I. We observed a predominance of the IgG over the others Ig and the kappa over the alpha light chains. The proteinuria of BENCE JONES was present among 3 patients, hypercalcemia among 4 patients and hypercreatininemia in 1 patient with chronic renal failure.
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PMID:[Laboratory diagnosis of monoclonal gammopathies. Prospective study of 14 cases in Dakar, Senegal]. 977 1

Multiple myeloma and MGUS are the two most common causes of monoclonal protein in serum or urine. The usually accepted diagnostic triad for multiple myeloma consists of a significant paraprotein in the serum or urine, more than 10% to 15% plasma cells in bone marrow, and the presence of bony lesions. Patients who meet the first two criteria but have no bony lesions, cytopenias, renal failure, or hypercalcemia may have smoldering myeloma, which often can be observed for a period of time before therapy is required. MGUS is characterized by a serum IgG monoclonal protein less than 3.5 g/dL or IgA paraprotein less than 2 g/dL, with no or only a small amount of protein in urine (Bence Jones protein < 1 g/24 hr). Less than 10% plasma cells are present in bone marrow, and patients have no lytic bony lesions, anemia, hypercalcemia, or renal insufficiency. Another important criterion for MGUS is stability of the monoclonal protein over time. Nonetheless, during long-term follow-up, an associated malignant process develops in about 30% of MGUS patients. Since none of the features defining MGUS is uniformly helpful in predicting the risk for malignant disease, patients should be followed up on a regular basis indefinitely.
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PMID:The search for meaning in monoclonal protein. Is it multiple myeloma or monoclonal gammopathy of undetermined significance? 1045 45

A 52-year-old woman complained of lower back pain and gluteal pain in April 1997, and was found to have anemia, hypercalcemia and renal disorder. In September of the same year, she was diagnosed as having IgA-lambda myeloma (stage IIIA). VMMD-IFN therapy was started in November, 1997, and this resulted in improvement of the M-protein level, and relief of the pain in the lower back and gluteal region. A second course of VMMD-IFN therapy was also effective. In April 1998, however, the back pain worsened, and in July the patient suffered a fall and fractured her left femur. Upon readmission to our hospital, the level of M-protein was lower, and high fever, hypercalcemia, renal disorder, elevation of the LDH level, anemia and thrombocytopenia were observed. Bone marrow examination revealed 30% atypical large-sized CD19-, CD38+, CD56+ myeloma cells and chromosomal abnormalities. Although the symptoms were improved temporarily after a third course of VMMD therapy, disease aggravation occurred again, and extramedullary masses appeared on the head, face and pelvis. VAD therapy was performed without effect, and the patient died about 2 months after recurrence. This was a comparatively rare case of fulminant multiple myeloma occurring in the terminal stage.
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PMID:[Aggressive transformation and extramedullary tumor formation in IgA-lambda multiple myeloma]. 1102 Sep 90

Mesenteric panniculitis is a rare disease of the bowel mesentery, characterized by tumor-like infiltration by chronic inflammatory cells, fat necrosis, and fibrosis. Reported cases cited clinical presentation ranging from abdominal pain to fever of unknown origin, the majority of which were idiopathic and associated with a benign prognosis. We report the case of a 43-yr-old male who presented with malaise, weight loss, microcytic anemia, and a high erythrocyte sedimentation rate. Radiographic and histological investigations revealed typical features of mesenteric panniculitis. Initial treatment with high-dose oral prednisolone led to rapid and complete resolution of symptomatology, radiographic, and laboratory anomalies. Within 6 months, the patient presented again with anemia, renal failure, and hypercalcemia. A diagnosis of IgA kappa chain myeloma was made. Despite chemotherapy and restoration of normocalcemia, he died from refractory pulmonary edema. This is the first report of a hematological malignancy initially presenting with features of mesenteric panniculitis culminating in an aggressive course and a fatal outcome.
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PMID:Aggressive multiple myeloma presenting as mesenteric panniculitis. 1119 60

A 75-yr-old male simultaneously having lymphoplasmacytic lymphoma (LPL) and diffuse large B-cell lymphoma (DLBCL) is presented. He had manifested with lumbago, high-grade fever, and confusion. Physical examination on admission showed disorientation and severe back pain. There were neither lymphadenopathy nor hepatosplenomegaly. Routine laboratory tests showed moderate pancytopenia, hypercalcemia (serum calcium, 15.9 mg/dL), IgM lambda-type monoclonal gammopathy (IgG, 405 mg/dL; IgA, 42 mg/dL; and IgM, 2023 mg/dL), and lambda-type Bence-Jones protein in the urine (0.8 g/d). Bone marrow biopsy showed the clusters of surface lambda-positive small-sized mature-appearing lymphoplasmacytoid cells. Bone survey and computed tomographic scan showed multiple osteolytic lesions and a tumor involving the third lumbar spine (L3). An open biopsy of the L3 tumor showed diffuse proliferation of CD20- and lambda-positive large cells. We thus diagnosed the patient as simultaneously having LPL and DLBCL. Although the combination chemotherapy was at least partially effective, he died of bacteremia and organ failure after three courses of chemotherapy. To clarify the clonal relatedness between LPL and DLBCL, we analyzed the sequences of the complementarity-determining region 3 in immunoglobulin heavy-chain genes. The data showed that LPL and DLBCL in the present patient originated from two independent clones.
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PMID:Simultaneous development of lymphoplasmacytic lymphoma and diffuse large B-cell lymphoma--analyses of the clonal relatedness by sequencing CDR3 in immunoglobulin heavy chain genes. 1258 Nov 94

Patients with smoldering multiple myeloma (SMM) meet the diagnostic criteria of multiple myeloma (MM) but are asymptomatic. Between January 1978 and July 2001, 53 patients (median age 63 years) were diagnosed with SMM. The median serum M-protein and proportion of bone marrow plasma cells were 36 g/l and 27% respectively. Two subsets of SMM were identified: (i) evolving SMM (n = 22), characterized by a progressive increase in serum M-protein, a previously recognized monoclonal gammopathy of undetermined significance (MGUS) and a significant higher proportion of IgA type and (ii) non-evolving SMM (n = 26) with stable M-protein that abruptly increases when symptomatic MM develops. Thirty-four patients developed symptomatic MM. The median time to progression in the overall series was 3.2 years and the only feature associated with a shorter time to progression was the evolving versus non-evolving type (1.3 vs. 3.9 years respectively, P = 0.007). The pattern of progression consisted of anaemia, lytic bone lesions or both, without renal failure, hypercalcaemia or extramedullary plasmacytomas. Fifty-seven per cent of patients that required chemotherapy showed no or minimal response. The median survival from diagnosis and from progression was 8.2 and 3.5 years respectively.
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PMID:Smoldering multiple myeloma: natural history and recognition of an evolving type. 1461 66


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