Brief report: atrial systolic failure in primary amyloidosis.
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Amyloidosis↗
Biomedical subjects
G G Cornwell
Publications and source records attributed to G G Cornwell.
Seminal vesicle amyloid: the first example of exocrine cell origin of an amyloid fibril precursor.
Amyloid fibrils have been extracted from seminal vesicles, and a dominant 14 kD amyloid fibril protein has been identified. An antiserum to this protein reacted both with amyloid and with epithelial cells in some normal seminal vesicles. These reactions were blocked with seminal vesicle secretion and seminal vesicle amyloid fibril protein, but not by degraded amyloid fibrils or fibril protein from other types of amyloid. It is concluded that seminal vesicle amyloid is derived from secretory proteins of the seminal vesicles. As such, it is the first amyloid described which appears to be the product of an exocrine secretory cell.
Aged↗
Senile aortic amyloid. Evidence for two distinct forms of localized deposits.
Aortic tissues obtained at autopsy were examined from 84 patients (age, 18-96 years). Amyloid deposits were present in the media in 61 of 63 (97%) of the patients above the age of 50. In addition, intimal amyloid deposits were present in 35% of this group. Intimal amyloid differed from medial amyloid both in its morphologic characteristics and its association with atherosclerosis. An antiserum raised to a low molecular weight protein extracted from amyloid fibrils of the aortic media reacted specifically with medial amyloid but did not react with intimal deposits. Neither type of amyloid reacted with anti-ATTR (Senile systemic amyloid), anti-AANF (isolated atrial amyloid), or antisera to other known forms of amyloid. These findings are consistent with the presence of two separate forms of localized amyloid in the aging aorta.
Adolescent↗
Myocardial fibrosis in aging germ-free and conventional Lobund-Wistar rats: the protective effect of diet restriction.
The influences of diet restriction and germ-free environment on aging were studied in 127 male Lobund-Wistar rats ranging in age from 7 to 48 months. There was an age-related increase in collagen deposition of the heart, particularly involving the left ventricle. The degree of fibrosis was significantly less extensive in middle and old age rats (18 mo, 30 mo, 32+ mo) maintained on a restricted diet. No clear-cut influence of germ-free environment was apparent. No amyloid was detected in 175 tissue sections examined from hearts, lungs, and livers.
Aging↗
Macromolecular properties of glycosaminoglycans in primary AL amyloid fibril extracts of lymphoid tissue origin.
We have previously demonstrated the presence of glycosaminoglycans (GAGs) in water extracts of secondary AA amyloid fibrils. In the present study we isolated significant quantities of GAGs from fibril extracts of immunoglobulin light chain (AL) type derived from the spleens from two patients afflicted with primary amyloidosis. Employing ion-exchange chromatography and gel filtration subsequent to various specific chemical and enzymatic treatments, different types of high molecular weight GAGs were found in both preparations, but not in the corresponding normal splenic extracts. The amyloid-associated GAGs of the extracts derived from one patient consisted of 60% dermatan sulphate and 40% heparan sulphate whereas those obtained from the second spleen were 25% dermatan sulphate and 75% heparan sulphate. The heparan sulphate fraction occurred in the form of proteoglycans, whereas the dermatan sulphate apparently occurred as free GAG chains, resembling the data recently obtained from AA amyloid fibril extracts.
Amyloid↗
Autologous bone marrow transplantation for acute myeloid leukemia following in vitro treatment with neuraminidase and monoclonal antibodies.
Although monoclonal antibodies (MoAbs) to CD15, especially PM-81, react with leukemic blasts from the majority of patients with acute myeloid leukemia (AML), a small subset of patients have cells that are CD15 negative or dim. We determined previously that neuraminidase will increase the reactivity of PM-81 with AML blasts, as well as blasts from many patients with acute lymphoblastic leukemia (ALL). In this report, we describe the laboratory results and clinical course of the first patient with AML whose harvested bone marrow was treated with neuraminidase prior to MoAbs and complement treatment. Neuraminidase increased the percentage of the patient's leukemia cells that reacted with PM-81 from 18% to 90% and more than doubled the percentage of AML blasts that were lysed by PM-81 and complement. The patient suffered no acute toxicity, engrafted rapidly, and was transfusion independent by day 21 post-ABMT. This report demonstrates the probable safety and efficacy of pretreatment of bone marrow with neuraminidase, and increases the number of patients with AML or ALL who may benefit from ABMT using marrow purging with MoAb to CD15.
Acute Disease↗
Chromosome changes in a patient achieving complete remission in the acute phase of chronic myelogenous leukemia.
A patient with chronic myelogenous leukemia (CML) had a Philadelphia chromosome (Ph') [t(9q+;22q-)] in all cells and trisomy C in 13% of cells (later determined to be trisomy 8) at the time of diagnosis. After 21 months of intermittent treatment with busulfan, the acute phase developed with the appearance of an additional abnormality (trisomy ? 19). During a complete remission and reconversion to the chronic phase, trisomies 8 and ? 19 DISAPPEARed, although the Ph' remained. Following a period of marked thrombocytosis, a second relapse occurred with the reappearance of both marker chromosomes.
Adult↗
Combination chemotherapy and radiotherapy for acute lymphocytic leukemia in adults: results of CALGB protocol 7113.
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Adult↗
Hand-mirror cell leukemia associated with mental retardation: immunologic, chromosome, and morphological studies.
Cycochemical, morphological, immunologic, and cytogenetic studies were carried out on hand-mirror cells (HMC) from a mentally retarded patient with a constitutional chromosome abnormality, 46,XX,r(21), and acute lymphoblastic leukemia. Scanning electron and differential interference contrast microscopy showed microspikes on the uropodia, but little evidence of cellular motility, despite formation and disappearance of individual uropodia in cell suspensions. The cells rosetted with sheep erythrocytes, suggesting T-cell origin. Cells derived from the bone marrow (80% HMC) showed a high degree of polyploidy (60%) and a bimodal chromosome number of 49 (49,XX,+10,-21, +3 rings) and 94 (6 no. 10, 3 no. 18, 2 no. 21 chromosomes, 3 ring chromosomes, plus 4 copies of each other chromosome).
Bone Marrow↗
Hypersensitivity reactions to iv melphalan during treatment of multiple myeloma: Cancer and Leukemia Group B experience.
Ten patients developed allergic reactions to iv melphalan (L-PAM) during therapy for multiple myeloma. The incidence of such reactions was 2.4% among 425 patients receiving iv L-PAM with or without other drugs and 3.9% among 255 patients receiving iv L-PAM alone. Only one such reaction was demonstrated in 294 patients who initially received oral L-PAM. The median day of first reaction to iv L-PAM was Day 222 (range, Days 44-909) and the median total dose prior to a reaction was 185 mg (range, 51-250 mg). Of five patients who subsequently received oral L-PAM, four developed a reaction similar to that experienced with the iv drug.
Administration, Oral↗
Chromosome studies in myelomatosis.
Cytogenetic studies of patients with multiple myeloma and plasma cell leukemia have shown that chromosome abnormalities occur in the bone marrow and/or the PHA-stimulated blood of at least half the patients. The abnormalities include numerical and structural aberrations and are highly variable. Hypodiploid modes occur fairly frequently. Addition of material to the long arm of the No. 14 chromosome (14q+ marker) occurs in about 17% of the small series of patients that have been studied with banding so far.
Bone Marrow↗
Multivariate approach to predictive diagnosis of bone-marrow iron stores.
The authors constructed a computer-assisted multivariate data analysis system that finds the subset of a group of laboratory tests (i.e., experimental variables) best able to discriminate between previously identified subpopulations. A Bayesian decision-analysis algorithm was devised for predictive diagnosis by the use of sample means and standard deviations of those identified tests. This approach was used on a set of nine hematologic laboratory tests to find a subset capable of efficiently predicting the presence or absence of bone-marrow iron stores. This identified subset proved to have a diagnostic efficiency of 0.90. The success of this system suggests the feasibility of such an approach in finding a "best" subset of loosely related variables.
Anemia, Hypochromic↗
Senile cardiac amyloid: demonstration of a unique fibril protein in tissue sections.
Antisera were raised against degrading amyloid fibrils isolated from the heart of a patient with senile cardiac amyloidosis (SCA), and from a medullary carcinoma of the thyroid (MCT). The antisera were absorbed and used in indirect immunofluorescence to identify an amyloid fibril protein (ASCA) in heart tissue from patients with senile cardiac amyloidosis and to identify the amyloid fibril protein (AMCT) found in association with medullary carcinomas of the thyroid. Absorbed anti-ASCA antiserum did not react with normal tissue such as heart, liver, spleen, and striated muscle, or with amyloid tissue known to contain amyloid fibril proteins AA, AlambdaI, AlambdaIV, AlambdaV, AMCT or with pancreatic tissue containing islet amyloid deposits. The reactions with senile amyloid he,rt tissue could be blocked completely by degraded amyloid fibrils extracted from senile amyloid heart tissue or by amyloid fibril protein ASCA isolated from such fibrils. The anti-AMCT antiserum showed a similar specific reaction restricted to amyloid associated with MCT. In addition, antisera specific for amyloid fibril proteins AA, AlambdaI, AlambdaIV, and AlambdaV failed to react with senile cardiac amyloid, pancreatic islet amyloid, or medullary thyroid amyloid.
Aged↗
Identification and characterization of different amyloid fibril proteins in tissue sections.
Antisera specific for 4 different classes of amyloid fibril proteins, amyloid protein AA and the immunoglobulin light-chain amyloid proteins AlambdaI, AlambdaIV, and AlambdaV, were used to identify these proteins directly in tissue sections from 25 patients with amyloidosis. The specificity of these reactions was established by blocking experiments with purified amyloid fibril proteins and Bence Jones proteins of known variable subgroups. Protein AA was detected in 17 patients, including all 13 with secondary amyloidosis, 2 with primary amyloidosis and 2 with Waldenström's macroglobulinemia. Immunoglobulin light-chain proteins AlambdaI, AlambdaIV, and AlambdaV were in 3, 1, and 2 patients, respectively, all of whom had primary or myeloma/macroglobulinemia-associated amyloidosis. Antiserum specific for the amyloid-related serum protein SAA reacted with the same tissues as anti-AA and had the same pattern of staining in tissue sections.
Amyloid↗
Ssytemic effects of intravenous polyriboinosinic-polyribocytidylic acid in man.
To investigate the systemic, clinical and laboratory effects of iv polyriboinosinic-polyribocytidylic acid (poly I-poly C), 32 doses of poly I-poly C were administered to 22 patients. Doses between 1 and 10 mg/kg induced the formation of serum interferon (IF) and fever. Whereas a direct relationship was seen between the poly I-poly C dose and fever, serum IF levels were not significantly changed by increasing the dose of poly I-poly C over a log range from 1 to 10 mg/kg. Transient abnormalities were noted in liver function tests in 4 of 13 patients who received greater than 6.0 mg/kg. Other laboratory changes were confined to an increase in the absolute granulocyte count that paralleled fever development and abnormalities in coagulation parameters of 1 patient. In vitro lymphocyte DNA synthesis in response to mitogens was transiently impaired at times corresponding to serum IF appearance. These studies have established dose levels of poly I-poly C that can be safely administered to man with minimal toxicity and result in IF induction.
Adult↗
The use of low-dose prednisone and melphalan in the treatment of poor-risk patients with multiple myeloma.
A study was designed to evaluate the effectiveness of prednisone therapy in poor-risk patients with multiple myeloma. Patients were treated with melphalan alone or in combination with prednisone at doses of either 0.6 mg/kg or 0.3 mg/kg. The group of patients receiving melphalan and prednisone 0.6 mg/kg had significantly improved responses in hemoglobin, lowering of the M-protein concentration, and reduction of azotemia. Significant benefits attributable to prednisone were not demonstrated in the group receiving 0.3 mg/kg. Good responses have been shown to produce improved survival. The combination of melphalan and prednisone is effective in producing good responses, but the dose of steroids is important. A dose of prednisone of 0.6 mg/kg in gradually decreasing doses has been found to be effective in production of good responses, especially in the uremic patient.
Dose-Response Relationship, Drug↗
Letter: Autoimmune thrombocytopenia purpura and Hashimoto's thyroiditis.
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Adult↗