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Biomedical subjects

R A Kyle

Publications and source records attributed to R A Kyle.

At least 109 records · Page 6Linked to original sources
PubMed1999

Guidelines for clinical and laboratory evaluation patients with monoclonal gammopathies.

This guideline provides the recommendations of an expert panel for the clinical and laboratory evaluation of patients suspected of having a clinical condition that produces a monoclonal protein in serum or urine. The recommendations describe the clinical conditions in which a monoclonal protein should be sought, the optimal sequence of testing to diagnose and monitor these patients, and the most effective laboratory procedures.

Evaluation Studies as Topic↗
PubMed1999

Sequence of testing for monoclonal gammopathies.

The first test for recognition of monoclonal gammopathies should be serum protein electrophoresis with high-resolution agarose gel. Serum protein electrophoresis should be performed whenever multiple myeloma, Waldenström's macroglobulinemia, primary amyloidosis, or a related disorder is suspected. Immunofixation is critical for the differentiation of a monoclonal from a polyclonal increase in immunoglobulins. Quantitation of immunoglobulins should be performed with a rate nephelometer. The viscosity of serum should be measured if the patient has signs or symptoms of hyperviscosity syndrome. A 24-hour urine specimen should be obtained for determination of the total amount of protein excreted each day. Immunofixation of the urine should be performed on every patient who has an M-protein level greater than 1.5 g/dL (15 g/L) in the serum or in whom multiple myeloma, Waldenström's macroglobulinemia, primary amyloidosis, or a related disorder is suspected.

Electrophoresis↗
PubMed1999

High-dose therapy in multiple myeloma and primary amyloidosis: an overview.

Autologous stem cell transplantation is a reasonable approach for patients younger than 70 years and should be discussed with each patient before instituting alkylating agent therapy. Ideally, it should be done in a clinical trial. Although most patients relapse, it does provide a modest prolongation of survival. The major needs are an improved preparative regimen before transplantation and the removal of myeloma cells and, more importantly, their precursors from the peripheral blood. Conventional allogeneic transplantation is associated with too high a mortality rate at present and cannot be recommended. Efforts must be directed toward reducing transplant-related mortality by T-cell depletion or other means. The preparative regimen must be improved, because most patients relapse after transplantation. The use of dendritic cells and vaccines is an important area of research. The role of autologous transplantation in primary amyloidosis (AL) is indeterminate at present. Longer follow-up evaluation of patients who have undergone transplantation is needed. Patient selection is a critical aspect. Stem-cell transplantation should be performed in a clinical trial for primary AL.

Amyloidosis↗
PubMed1999

Clinical aspects of multiple myeloma and related disorders including amyloidosis.

Multiple myeloma is characterized by the presence of bone pain, weakness, and fatigue. Ninety-eight percent of patients have an M-protein in the serum or urine at the time of diagnosis. Skeletal roentgenograms are abnormal in nearly 80%. Renal insufficiency (creatinine > or = 2 mg/dL) is present in one-fourth. The major causes of renal insufficiency are "myeloma kidney" and hypercalcemia. The diagnosis of multiple myeloma depends upon the presence of more than 10% plasma cells or a plasmacytoma plus an M-protein in the serum or urine or lytic bone lesions. Multiple myeloma must be differentiated from monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. The plasma cell labeling index and the presence of circulating plasma cells in the peripheral blood are helpful in the differential diagnosis. Plasma cell leukemia, osteosclerotic myeloma (POEMS syndrome), and plasmacytomas are discussed. The heavy-chain diseases consist of alpha, gamma, and mu heavy-chain disease. The fibrils of primary amyloidosis consist of kappa or lambda monoclonal light chains. Weakness, fatigue, and weight loss are the most frequent symptoms. Macroglossia occurs in 10%. An M-protein is found in the serum or urine in 90%. The presence of nephrotic syndrome, renal insufficiency, congestive heart failure, orthostatic hypotension, or sensorimotor peripheral neuropathy, and an M-protein in the serum or urine suggest the possibility of primary amyloidosis. The diagnosis depends upon the demonstration of amyloid in tissues. The subcutaneous fat aspirate is positive in 80% while the bone marrow is positive in 55%. If these tissues are negative, one should obtain tissue from an involved organ.

Amyloidosis↗
PubMed1999

Maintenance therapy and supportive care for patients with multiple myeloma.

Although multiple myeloma remains incurable, several drug therapies are valuable in the management of patients suffering from this condition. Interferon-alpha2b and prednisone are two agents that have been shown to modestly prolong disease-free survival when each is used as monotherapy and when used in combination. Bone resorption and anemia are among several complications that adversely affect health and quality of life in patients with multiple myeloma. Bone resorption leads to skeletal complications, such as pathologic fractures, pain, spinal cord compression, and hypercalcemia. Bisphosphonates are specific inhibitors of osteoclastic activity that can be used to treat bone resorption. Intravenous pamidronate is effective in preventing skeletal complications and also may exert a direct inhibitory effect on myeloma cells. Exogenous epoetin alfa is helpful in treating more than half of patients with anemia during the plateau state of multiple myeloma. Overall, the management of patients with multiple myeloma is complex and should focus on the treatment of the disease process and the associated complications.

Antineoplastic Agents↗
PubMed1998

Primary systemic amyloidosis with delayed progression to multiple myeloma.

BACKGROUND: Primary systemic amyloidosis (AL) and multiple myeloma both are clonal plasma cell proliferative disorders. Although 10-15% of patients with myeloma have coexisting primary amyloidosis, it is unusual for patients with primary amyloidosis to progress to myeloma at a later date. The authors describe a case series of six patients in whom such progression occurred. METHODS: A computerized search was done of the medical records of all patients seen at the Mayo Clinic between January 1, 1960 and December 31, 1994 with a diagnosis of AL. Of 1596 patients with AL, 6 patients (age range, 60-74 years; median age, 68 years) with biopsy-proven AL were reviewed in whom delayed (at least 6 months after the diagnosis of AL) progression to multiple myeloma occurred. RESULTS: At the time of the diagnosis of AL, none of the six patients had evidence of multiple myeloma. The dominant manifestation of AL was peripheral neuropathy in three patients and cutaneous AL, renal AL, and amyloid arthropathy in one patient each. The diagnosis of multiple myeloma was made 10-81 months after the diagnosis of AL, based on the demonstration of multiple osteolytic lesions (4 patients) or marked bone marrow infiltration (> or = 50%) by plasma cells (5 patients). Two patients had received chemotherapy (melphalan and prednisone) for AL. Five patients received chemotherapy (four patients) or high dose methylprednisolone (one patient) after the diagnosis of multiple myeloma. Five patients died, and the median actuarial survival after the diagnosis of multiple myeloma was 20 months. Multiple myeloma was the cause of death in four patients; one patient died of systemic amyloidosis. In 2 patients death occurred within 3 months. CONCLUSIONS: AL occasionally progresses to overt multiple myeloma. These cases usually occur in patients without significant cardiac or hepatic AL who live long enough to develop multiple myeloma.

Aged↗