Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Several trials have shown the activity of thalidomide (THAL) in relapsed multiple myeloma (MM) patients failing PBSCT or conventional chemotherapy. PBSCT is considered standard treatment for most patients requiring therapy for MM; however, patients with VAD-resistant disease may not be able to receive PBSCT due to rapidly advancing disease. We report four cases of VAD-refractory MM salvaged with THAL + VAD followed by PBSCT. All patients underwent stem cell mobilization with cyclophosphamide (Cy) (4.5 g/m(2)) and GMCSF. Melphalan (140-200 mg/m(2)) was given as conditioning. All patients engrafted within 12-16 days after PBSCT. Day +100 evaluation showed the following: very good partial response (n = 1) and complete response (n = 3). After a median follow-up to 153 days, two patients continue to take THAL with no signs of disease progression. One patient developed CHF and was taken off THAL while another patient has died of progressive disease while on THAL (MTD 50 mg). In conclusion, VAD-refractory patients were salvaged with the addition of THAL to VAD. They were subsequently able to undergo autologous PBSCT for MM, which will likely improve their overall survival. This suggests that THAL and other related immunomodulatory drugs may prove useful for initial MM therapy in combination with standard chemotherapy followed by PBSCT.
 ...
PMID:Thalidomide as salvage therapy for VAD-refractory multiple myeloma prior to autologous PBSCT. 1285 10

The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 patients with POEMS syndrome. Minimal criteria were a sensorimotor peripheral neuropathy and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from neuropathy associated with monoclonal gammopathy of undetermined significance, additional criteria were included: a bone lesion, Castleman disease, organomegaly (or lymphadenopathy), endocrinopathy, edema (peripheral edema, ascites, or effusions), and skin changes. The median age at presentation was 51 years; 63% were men. Median survival was 165 months. With the exception of fingernail clubbing (P =.03) and extravascular volume overload (P =.04), no presenting feature, including the number of presenting features, was predictive of survival. Response to therapy (P <.001) was predictive of survival. Pulmonary hypertension, renal failure, thrombotic events, and congestive heart failure were observed and appear to be part of the syndrome. In 18 patients (18%), new disease manifestations developed over time. More than 50% of patients had a response to radiation, and 22% to 50% had responses to prednisone and a combination of melphalan and prednisone, respectively. We conclude that the median survival of patients with POEMS syndrome is 165 months, independent of the number of syndrome features, bone lesions, or plasma cells at diagnosis. Additional features of the syndrome often develop, but the complications of classic multiple myeloma rarely develop.
 ...
PMID:POEMS syndrome: definitions and long-term outcome. 1245

AL amyloidosis is a rare disorder characterised by tissue deposition of a fibrillary proteinaceous material, formed from monoclonal immunoglobulin light (or exceptionally heavy) chains. Although it may complicate multiple myeloma or B-cell lymphomas, AL amyloidosis is often associated with a low burden of clonal plasma cells ("primitive" AL amyloidosis). The mechanisms involved in the formation of AL amyloid deposits remain unclear, but are probably related to structural peculiarities of monoclonal immunoglobulin light chains. AL amyloidosis is usually a systemic disease, often revealed by renal involvement, the most common complication of the disease. The longterm prognosis of AL amyloidosis is poor, mainly related to amyloid restrictive cardiomyopathy leading to congestive heart failure. Oral melphalan and prednisone is considered the standard treatment for AL amyloidosis, but with limited increase in the median survival. High-dose intra-venous melphalan with autologous stem cell transplantation is an effective treatment, aimed at eliminating the clonaly expanded plasma cells, which has been shown to induce complete hematologic remissions and to prolong survival. However, the tolerability of such treatment is low, limiting its use to selected patients. The development of new drugs, able to interfere with amyloid fibril deposition, may provide a new therapeutic approach.
 ...
PMID:[Immunoglobulin light chain amyloidosis: recent molecular, clinical and therapeutic approach]. 1529 Nov 38

A 53-year-old man was admitted to Keio University Hospital because of serious dyspnea and edema of the lower extremities. Eighteen months previously, the patient had complained of chest discomfort, and was then admitted for the first time to our hospital for evaluation of chest pain. Electrocardiography showed poor R wave progression in leads Vl through V4, and diffuse nonspecific ST-segment and T wave abnormalities with low voltage. However, no definitive diagnosis could be made at this initial admission and a calcium-channel blocker was prescribed. Despite this treatment, the patient was readmitted with worsening dyspnea and lower extremity edema. The diagnosis of heart failure and nephritic syndrome was made at the second admission. In addition, immunoelectrophoresis showed a monoclonal IgD (lambda) M protein and increased plasma cells in the bone marrow, suggesting a diagnosis of multiple myeloma. The patient was thus given dexamethasone (20 mg per day for 4 days) intravenously, but his symptoms did not improve. Two weeks later, the patient deteriorated further with congestive heart failure and renal failure, and subsequently died of cardiac arrest with ventricular fibrillation. On autopsy, IgD (lambda)-positive plasma cell proliferation was found in the bone marrow, confirming the diagnosis of multiple myeloma. In addition, amyloid deposition was detected in various organs including the heart, kidneys, esophagus, duodenum, ileum, colon, tongue, and lungs. In particular, the weight of the heart was 650 g demonstrating a hypertrophic septum and amyloid deposition in the myocardium and even coronary arteries. In summary, the final diagnosis was IgD (lambda) multiple myeloma associated with systemic amyloidosis.
 ...
PMID:IgD myeloma with systemic amyloidosis with chest discomfort as an initial symptom. 1547 32

Cerebral infarction is an uncommon complication in multiple myeloma with hyperviscosity. Serum hyperviscosity may cause a variety of clinical manifestations including bleeding from mucosal membranes, congestive heart failure, retinopathy, and various neurologic deficits. These manifestations have been attributed to the presence of large quantities of asymmetrical molecules of high molecular weight in the serum. We recently experienced a case of multiple myeloma with acute cerebral infarction, which caused by hyperviscosity, as an initial manifestation in IgG multiple myeloma, and reviewed the relevant literature of myeloma presenting with the stroke. A 68-yr-old woman abruptly developed hypesthesia and monoplegia in the left leg. The stroke confirmed by the brain MRI and MR angiography, which revealed acute infarction at the right anterior cerebral artery territory. On admission, routine blood tests showed a slight decrease in hemoglobin and a marked increase in erythrocyte sedimentation rate. Peripheral blood smear, serum protein electrophoresis, serum visocity, and bone marrow aspiration showed that she had IgG multiple myeloma with hyperviscosity. She was treated by chemotherapy with cyclophosphamide and discharged with the improved clinical condition.
 ...
PMID:Cerebral infarction in IgG multiple myeloma with hyperviscosity. 1610 Apr 71

An increasing number of diagnostic imaging procedures requires the use of intravenous radiographic contrast agents, which has led to a parallel increase in the incidence of contrast-induced nephropathy. Risk factors for development of contrast-induced nephropathy include pre-existing renal dysfunction (especially diabetic nephropathy and multiple myeloma-associated nephropathy), dehydration, congestive heart failure and use of concurrent nephrotoxic medication (including aminoglycosides and amphotericin B). Because contrast-induced nephropathy accounts for a significant increase in hospital-acquired renal failure, several strategies to prevent contrast-induced nephropathy are currently advocated, including use of alternative imaging techniques (for which contrast media are not needed), use of (the lowest possible amount of) iso-osmolar or low-osmolar contrast agents (instead of high-osmolar contrast agents), hyperhydration and forced diuresis. Administration of N-acetylcysteine, theophylline, or fenoldopam, sodium bicarbonate infusion, and periprocedural haemofiltration/haemodialysis have been investigated as preventive measures in recent years. This review addresses the literature on these newer strategies. Since only one (nonrandomized) study has been performed in intensive care unit patients, at present it is difficult to draw firm conclusions about preventive measures for contrast-induced nephropathy in the critically ill. Further studies are needed to determine the true role of these preventive measures in this group of patients who are at risk for contrast-induced nephropathy. Based on the available evidence, we advise administration of N-acetylcysteine, preferentially orally, or theophylline intravenously, next to hydration with bicarbonate solutions.
 ...
PMID:Bench-to-bedside review: preventive measures for contrast-induced nephropathy in critically ill patients. 1613 85

Amyloidosis is a systemic disease in which clinical manifestations are caused by the replacement of normal tissue with insoluble amyloid fibrils. Cardiac involvement causes a restrictive cardiomyopathy and is associated with poor functional outcomes. Cardiac magnetic resonance imaging and measurement of B-type natriuretic peptide are particularly helpful in distinguishing restrictive cardiomyopathy from constrictive pericarditis, but a tissue biopsy is required to make the diagnosis of amyloidosis. Although standard treatment options for congestive heart failure may provide symptomatic relief in cardiac amyloidosis, prognosis remains dismal. Judicious diuretic use remains the mainstay of therapy, but achieving optimal fluid balance is difficult because patients are usually "preload dependent." Angiotensin-converting enzyme inhibitors in low doses are often helpful but may lead to orthostatic hypotension, particularly in patients who also have involvement of the autonomic nervous system. beta Blockers may be useful if given relatively early in the disease process, but should be used with caution in patients with advanced disease because they may exacerbate symptoms. Therapy aimed at the underlying disease process in primary systemic amyloidosis is based on treatment regimens used in multiple myeloma, such as melphalan and prednisone. These offer limited benefit when cardiac involvement is significant, but newer treatments, including the novel anthracycline 4'-iodo-4'-deoxydoxorubicin, potentially combined with autologous stem cell transplantation, offer some hope for the future.
 ...
PMID:Current therapeutic strategies in cardiac amyloidosis. 1628 71

Primary (AL) amyloidosis is the most common form of systemic amyloidosis. The morbidity arises from extracellular deposition of immunoglobulin light chain (LC) fibrils in major organs, such as the kidneys, heart, and bowel. Organ dysfunction contributes to a high mortality and poor prognosis, with a median survival time of 1-2 years from diagnosis. Here, we present a 46-year-old man with an exceptional clinical course of an LC multiple myeloma with generalized amyloidosis, causing renal insufficiency, congestive heart failure, and complete intestinal necrosis. We have summarized recent knowledge on AL amyloidosis, its association with monoclonal gammopathies, clinical presentations, diagnostic tools, and treatment strategies. Our comprehensive overview of this rare and often fatal disease aims to increase the awareness of AL amyloidosis. This may facilitate earlier diagnosis, and thus allow initiation of prompt and specific therapies, which are indispensable in order to improve disease prognosis.
 ...
PMID:Primary (AL) amyloidosis in plasma cell disorders. 1688 Feb 41

A case of plasma cell leukemia showing mostly pleomorphic plasma cells in the form of convoluted and multilobated nuclei with some having bilobed nuclei and internuclear bridges is being reported for its rarity of occurrence. Patient presented with congestive cardiac failure and features of nephropathy. There were no lytic lesions in the bone. Serum electrophoresis did not show any M-band while urine electrophoresis demonstrated Bence Jones protein confirming a light chain only type of myeloma. Patient is in remission 5 months after diagnosis. The significance of recognising such pleomorphic plasma cells is discussed.
 ...
PMID:Plasma cell leukemia with pleomorphic plasma cells--a case report. 1700 15

The differential diagnosis for hypophosphatemia is long, and involves complex, overlapping physiological systems. Practitioners are often guilty, however, of simply supplementing phosphate without fully investigating the etiology of the problem. The purpose of this case presentation is to illustrate a case of spurious hypophosphatemia that initially led to unnecessary phosphate replacement in a woman with undiagnosed multiple myeloma. An 85-year-old African American woman was admitted to the hospital for congestive heart failure exacerbation. The patient was incidentally found to be profoundly hypophosphatemic and was also diagnosed with multiple myeloma at this hospitalization. Normal phosphorus levels were difficult to maintain despite aggressive replacement. A serum sample initially reported to have an abnormally low phosphorus concentration on the Beckman CX7 analyzer was reanalyzed with the Kodak Ektachem 700 system, revealing the phosphorus concentration to be towards the higher limit of the normal range on the same sample. We conclude that clinicians should proceed with caution before aggressively treating abnormal phosphorus levels in patients with known paraproteinemia. Conversely, unexplained phosphorus abnormalities should bring disorders associated with paraproteinemia, such as multiple myeloma, into the differential diagnosis. Knowledge of how various phosphorus assays are affected by paraproteins is essential to guiding diagnosis and treatment. We also review mechanisms of reported interference with common assays.
 ...
PMID:Hypophosphatemia associated with paraproteinemia: a case report and review of the literature. 1823 74


<< Previous 1 2 3 4 5 6 7 8 Next >>