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Biomedical subjects

P R Greipp

Publications and source records attributed to P R Greipp.

At least 163 records · Page 9Linked to original sources
PubMed1983

Hyposplenism in primary systemic amyloidosis.

Twenty-two of 91 (24%) patients with primary systemic amyloidosis were functionally hyposplenic. This group, identified by blood smear findings, had a different survival distribution when compared with a normosplenic group (p less than 0.0001). The peripheral blood smear was more sensitive than the splenic scan for a diagnosis of hyposplenism. Amyloidosis should be included in the differential diagnosis of acquired hyposplenism in the adult.

Amyloidosis↗
PubMed1983

Amyloidosis (AL). Clinical and laboratory features in 229 cases.

At the Mayo Clinic from 1970 to 1980, 229 patients with primary systemic amyloidosis (AL) were examined. Nephrotic syndrome, congestive heart failure, orthostatic hypotension, carpal tunnel syndrome, and peripheral neuropathy were often associated features. Electrophoresis of the serum revealed a spike in only 40%, but immunoelectrophoresis disclosed a monoclonal protein in 68%. Eighty-nine percent of patients in whom it was sought had a monoclonal protein in the serum or urine. The diagnosis of amyloidosis was made before death in 96% of the patients. The preferred sites for biopsy were the bone marrow, rectum, kidney, carpal ligament, liver, small intestine, skin, and sural nerve. The median survival of the 229 patients was 12 months. The median survival of the 77 patients with congestive heart failure was 6 months after the onset of symptoms. Congestive heart failure or arrhythmias accounted for death in 40%. Treatment for amyloidosis is unsatisfactory but includes melphalan, prednisone, colchicine, and dimethyl sulfoxide.

Adult↗
PubMed1983

Clinical, morphological, and cell kinetic differences among multiple myeloma, monoclonal gammopathy of undetermined significance, and smoldering multiple myeloma.

We reviewed the clinical and morphological findings in 43 cases of monoclonal gammopathy of undetermined significance (MGUS), 9 of smoldering multiple myeloma (SMM), and 23 of overt multiple myeloma (MM). In all cases, the patients' physicians had requested a bone marrow examination because of the possibility of MM. In all 75 cases, 3H-thymidine labeling indices were performed. The plasma cell labeling index correctly classified 62 of the 75 cases (83%). A linear discriminant function combining the labeling index and percentage of plasma cells improved the accuracy to 92% (69/75), or to 95% (71/75) if patients in whom MM developed within 6 mo were considered to have MM. The labeling index was most critical for the differential diagnosis of MM from SMM (p less than 0.001). Serum or urine M-protein level, percentage of plasma cells or lymphocytes in the bone marrow, and plasma cell grade, asynchrony, and nucleolar size failed to discriminate the group with SMM from the group with MM. In patients with MGUS or SMM, a plasma cell labeling index greater than 0.4% warned of impending MM. The plasma cell labeling index is a reliable diagnostic test when applied in cases of monoclonal gammopathy, especially when differentiation from MM is difficult using standard clinical criteria.

Aged↗
PubMed1982

"Idiopathic" Bence Jones proteinuria: long-term follow-up in seven patients.

We studied seven patients with idiopathic Bence Jones proteinuria (excretion greater than 1.0 g per 24 hours) who did not have acquired Fanconi's syndrome or an intact monoclonal immunoglobulin in the serum. None had evidence of overt multiple myeloma, of its variants, of systemic amyloidosis, or of malignant lymphoproliferative diseases when the proteinuria was recognized. In three of the seven patients, symptomatic multiple myeloma (associated with systemic amyloidosis in one) developed 8.8 to 21.1 years later. A fourth patient has asymptomatic myeloma and has not been treated. A fifth patient had an evolving myeloma at nine years but died of an unrelated cause. The remaining two patients still have apparently benign Bence Jones proteinuria after 7.7 and 12 years. Plasma-cell labeling indexes were low in all cases tested. This experience shows that although idiopathic Bence Jones proteinuria may remain stable for years, multiple myeloma or amyloidosis often develops, and consequently these patients must be kept under observation indefinitely.

Adult↗
PubMed1982

Recurrent incapacitating mucosal ulcerations. A prodrome of the hypereosinophilic syndrome.

The hypereosinophilic syndrome is a multisystem disease characterized by infiltration of eosinophils in bone marrow, heart, and other organs. Cutaneous involvement occurs, but usually it appears late in the disease and is of less importance than the cardiac and other organ involvement. We describe two patients with the hypereosinophilic syndrome (HES) in whom the initial manifestations of the disease were recurrent, severe mucosal ulcers involving the mouth and genitalia. In both patients, the mucosal involvement antedated the cardiac manifestations of the disease, and in both, the disease was refractory to treatment.

Adult↗
PubMed1981

Factor-X deficiency in amyloidosis: a critical review.

Thirty cases of amyloidosis with factor-X deficiency, including six of our own, were reviewed. Modest deficiency of factor X was often associated with severe bleeding. In many of the cases, clinical bleeding could not be accounted for by deficiency of factor X alone. Other hemostatic defects were found in these patients and probably contributed to the bleeding. Modes of treatment, including the empiric use of corticosteroids and splenectomy, were discussed in light of current knowledge of pathogenesis of this unusual blood clotting disorder. This involves the interaction of amyloid fibrils and blood clotting factors.

Adrenal Cortex Hormones↗
PubMed1978

3. The laboratory investigation of monoclonal gammopathies.

The monoclonal gammopathies are a group of disorders characterized by proliferation of a single clone of plasma cells that produce a homogeneous, monoclonal (M) protein. The structure of immunoglobulins, relationship of normal (polyclonal) immunoglobulins to myeloma and macroglobulin (monoclonal) immunoglobulins, pattern of monoclonal immunoglobulin overproduction, and laboratory methods for the recognition and study of monoclonal proteins in the serum and urine are reviewed. Interpretation of these laboratory tests is emphasized. The demonstration of a monoclonal protein in the serum or urine of a patient suggests multiple myeloma or one of its variants (solitary plasmacytoma of bone, extramedullary plasmacytoma, or plasma cell leukemia); Waldenström's macroglobulinemia or, occasionally, lymphoma; heavy-chain diseases (gamma, alpha, and mu); primary amyloidosis; and monoclonal gammopathies of undertermined significance. The electrophoretic and immunoelectrophoretic patterns found in the monoclonal gammopathies are discussed. Periodic electrophoresis of the serum and urine is essential in the follow-up and management of patients with a monoclonal gammopathy.

Amyloidosis↗
PubMed1978

Primary systemic amyloidosis: comparison of melphalan and prednisone versus placebo.

Satisfactory treatment for primary amyloidosis does not exist. Because the amyloid fibrils consist of a portion of a monoclonal light chain, it appears reasonable to treat amyloidosis with alkylating agents that are effective against the plasma cells that synthesize monoclonal light chains. Fifty-five patients with primary systemic amyloidosis were randomized (double blind) to melphalan-prednisone or placebo. In comparison with the placebo group, patients given melphalan-prednisone were able to continue on treatment for a longer time and to receive larger doses before the code was broken. Among this group, the nephrotic syndrome disappeared in two patients and urinary excretion of protein was reduced by more than 50% in eight others. Of 13 patients who received melphalan-prednisone for more than 12 mo, 6 improved, 3 were stable, and 4 had progression of disease. Survival did not differ significantly between the groups.

Alkaline Phosphatase↗
PubMed1978

Idiopathic pulmonary hemosiderosis: two cases in a family.

Two patients, a mother and her son, had idiopathic pulmonary hemosiderosis. In the son, the onset was acute and he died of massive hemoptysis. The mother's disorder developed less acutely and the condition improved after temporary respiratory support and massive corticosteroid therapy. This is the first reported instance of idiopathic pulmonary hemosiderosis occurring in more than one member of the same family. The diagnosis of idiopathic pulmonary hemosiderosis depends on compatible clinical and pathologic data and on exclusion of secondary causes of intrapulmonary hemorrhage.

Adult↗
PubMed1978

Cardiobacterium hominis endocarditis. Four cases with clinical and laboratory observations.

The clinical and microbiologic features of Cardiobacterium hominis endocarditis in four patients seen at the Mayo Clinic from 1971 through 1976 are described. All four were men ranging in age from 39 to 60 years. The precipitating factor in three was a dental procedure, and the illness was a prolonged, chronic one, with symptoms having been present 10 to 18 months before diagnosis. The other patient had a late prosthetic valve endocarditis and had had symptoms for only 3 months. Three patients were cured; the fourth died after 32 days of adequate therapy and what was considered a bacteriologic cure. Because of the pronounced fastidiousness of these bacteria, in vitro susceptibility tests could be done in only two of the four; the minimum inhibitory concentration for penicillin G was 0.07 microgram/ml in both. The therapeutic regimens were penicillin G plus streptomycin in the first case, predominantly penicillin G alone in the second, penicillin G for 2 weeks in the third, and ampicillin for 4 weeks in the fourth (prosthetic valve case) in addition to valve replacement. Clinical and laboratory experiences in the total reported cases lead us to believe that 3 weeks of therapy with penicillin G or ampicillin alone is adequate therapy for C. hominis endocarditis of natural valves.

Adult↗
PubMed1976

Platelet to leukocyte adherence phenomena associated with thrombocytopenia.

Two patients developed thrombocytopenia associated with an in vitro platelet to leukocyte adherence phenomenon (PLAP). In one patient, whole serum, intact plasma, and the IgG fraction of the serum could induce the PLAP of normal platelets in normal whole blood or buffy coat preparations. Passage of serum over a Sepharose column coupled with antihuman IgG resulted in the loss of all the PLAP activity. Activity could not be recovered from the whole serum or the IgG fraction of serum of the second patient; however, the PLAP was transferred to her newborn infant. In both patients, an unusual pulmonary illness accompanied the PLAP and thrombocytopenia.

Adult↗