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Biomedical subjects

W L Marsh

Publications and source records attributed to W L Marsh.

At least 19 recordsLinked to original sources
PubMed1991

Molecular cloning and primary structure of Kell blood group protein.

The Kell blood group is a major antigenic system in human erythrocytes. Kell antigens reside on a 93-kDa membrane glycoprotein that is surface-exposed and associated with the underlying cytoskeleton. We isolated tryptic peptides and, based on the amino acid sequence of one of the peptides and by using the PCR, prepared a specific oligonucleotide to screen a lambda gt10 human bone-marrow cDNA library. Four clones were isolated, one containing cDNA with an open reading frame for an 83-kDa protein. All known Kell amino acid sequences were present in the deduced sequence; moreover, rabbit antibody to a 30-amino acid peptide, prepared from this sequence, reacted on an immunoblot with authentic Kell protein. The Kell cDNA sequence predicts a 732-amino acid protein. Hydropathy analysis indicates a single membrane-spanning region, suggesting that Kell protein is oriented with 47 of its N-terminal amino acids in the cell cytoplasm, and a 665-amino acid segment, which contains six possible N-glycosylation sites, is located extracellularly. Computer-based search showed that Kell has structural and sequence homology to a family of zinc metalloglycoproteins with neutral endopeptidase activity.

Amino Acid Sequence↗
PubMed1990

Nonpigmented papillary carcinoma in a black thyroid gland.

A case of papillary thyroid carcinoma occurring in a 27-year-old man with black thyroid syndrome is reported. The tumor, in contrast to the remaining thyroid, was not pigmented. The significance and potential utility of the differential pigmentation are discussed.

Adult↗
PubMed1990

Poorly differentiated ('insular') thyroid carcinoma. Report of two cases and review of the literature.

The clinical, histopathologic, immunohistochemical, and electron microscopic findings of two cases of poorly differentiated ("insular") thyroid carcinoma are reported and compared with the 25 cases previously described in the literature. These 27 cases occurred in eight men and 19 women, aged from 34 to 77 years. All but 2 presented with a thyroid mass. Eleven (41%) of the 27 patients experienced local recurrences following surgery, 17 (63%) had development of cervical lymph node metastases, 5 (19%) had development of mediastinal lymph node metastases, 11 (41%) had pulmonary metastases, and 9 (33%) had bone metastases, and 9 (33%) had bone metastases. At least 15 patients (56%) are known to have died of their disease, usually within 8 years of diagnosis. Pathologically, the tumors are distinctive and grow as solid islands (insulae) of small cells separated by artifactually created clefts. In some instances, small follicles are also noted within the insulae. All tumors were positive on immunostaining for thyroglobulin. In view of the propensity for local recurrences and lymphatic and hematogenous dissemination, a total thyroidectomy and neck dissection would seem advisable. Additionally, adjuvant external beam irradiation, systemic chemotherapy, and/or radioactive iodine therapy should also be considered.

Adenocarcinoma↗
PubMed1990

Abnormal membrane physical properties of red cells in McLeod syndrome.

McLeod red cells (RBCs) lack Kx antigens and have weak expression of the Kell antigens. Individuals who carry the McLeod phenotype have acanthocytic RBCs and a compensated hemolytic state. To elucidate the role of the protein on which the Kx antigens reside in maintaining membrane deformability, the rheologic properties of McLeod RBCs were determined by ektacytometry. RBCs were obtained from normal individuals and from four patients with McLeod syndrome. Osmotic gradient deformability profiles of McLeod RBCs showed decreased whole cell deformability. Resealed ghosts from McLeod RBCs also showed decreased deformability, partly because of the decreased cell surface area and partly because of an intrinsic membrane stiffness in this syndrome. For the measurement of membrane mechanical stability, resealed ghosts were subjected to constant high shear stress in the ektacytomer, and deformability was recorded continuously as the deformable ghosts fragmented into rigid spherical vesicles. Membranes from McLeod RBCs showed a noticeable increase in mechanical stability. Acquired causes of acanthocytosis, such as liver disease, did not cause the rheologic abnormalities observed in McLeod cells. Other abnormalities noted in McLeod RBCs were decreased RBC potassium content and an increased number of dense RBCs, as determined by centrifugation on a discontinuous density gradient. The data indicate that McLeod RBCs are rigid and have decreased surface area and that their membranes are intrinsically rigid with increased mechanical stability. These abnormalities may account for the reduced RBC survival observed in McLeod syndrome. The protein that carries the Kx surface antigen seems to be required for the maintenance of the normal physical function of RBC skeletal proteins.

Adult↗
PubMed1990

Hairy cell leukemia involving an inguinal hernial sac.

A 37-year-old man with hairy cell leukemia was found incidentally to have a right inguinal hernia. Microscopic examination of the resected herniorrhaphy specimen disclosed dense transmural infiltration by hairy cells. Electron-microscopic and immunoperoxidase studies confirmed the presence of surface cytoplasmic projections and B cell phenotype, respectively, of the infiltrating cells. This case is, to our knowledge, the first reported instance of hairy cell leukemia involving a hernial sac and demonstrates the capacity of hairy cells to infiltrate unexpected soft-tissue sites.

Adult↗
PubMed1990

Diffuse angiomatosis of the extremities presenting as a sarcoma.

Diffuse angiomatosis of the extremities is a rare condition characterized by extensive or multifocal benign mass-forming vascular lesions involving multiple tissue planes; histologically, adipose and fibrous tissue are admixed with vascular elements that vary from capillary proliferations to large vessels that are often structurally abnormal. Although diffuse angiomatosis most likely represents a hamartomatous form of arteriovenous malformation, it behaves clinically as a benign but slowly progressive and unresectable neoplasm. We describe a case of diffuse angiomatosis of the lower extremity that presented clinically as a soft-tissue sarcoma.

Angiomatosis↗
PubMed1986

Kell blood group antigens are part of a 93,000-dalton red cell membrane protein.

Monospecific Kell blood group antibodies, of either human alloimmune or mouse monoclonal origin, react with a single surface-exposed protein of 93,000 daltons. Chymotryptic peptide maps of the 93,000-dalton protein isolated by antibodies of two different specificities (anti-K7 or anti-K14) indicate that Kell epitopes reside on the same protein. Kell protein is similar in size to band 3 protein but differs markedly in its tryptic and chymotryptic peptide maps, indicating that they are different proteins. In addition, sheep antibody to human band 3 does not react with Kell protein. Rabbit antibody to Kell protein reacts, by Western immunoblotting, with membrane proteins from Kell antigen positive red blood cells but not from those of a Ko (Kell null) cell. In intact red cells only a small portion of the Kell protein is available to lactoperoxidase-catalyzed iodination. Under nonreducing conditions Kell antigen is isolated not only as a 93,000-dalton protein but also as larger protein complexes ranging in size from above 200,000 to 115,000 daltons. Treatment of red cells with iodoacetamide, prior to isolation of Kell protein, reduces the amount of the very large complexes, but Kell protein occurs both as 115,000- and 93,000-dalton proteins.

Animals↗
PubMed1979

Adenoid cystic carcinoma: biologic behavior in 38 patients.

This study presents the clinical and pathologic data on 38 patients with adenoid cystic carcinoma. Most of the tumors arose from the major salivary glands or the mucus glands of the upper respiratory tract and oral cavity. The typical biological behavior of these tumors was slow but aggressive local growth despite all therapy, followed by eventual death from local disease and/or metastases. Twenty-eight of the patients have died, 22 from adenoic cystic carcinoma, 3 from postoperative complications, and 3 from intercurrent disease. Of the 10 living patients, 4 have active disease, 2 have no evidence of disease 14 years after diagnosis, and 4 have no evidence of disease but follow-up is less than 5 years. We compared various microscopic features of the tumors, including histologic grading, with prognosis but did not demonstrate a correlation in this small series.

Adult↗
PubMed1979

Autoimmune hemolytic anemia and the Kell blood groups.

Approximately one in 250 people with autoimmunity involving their red cells have IgG autoantibodies with specificity in the Kell blood groups. Red cells of these individuals have an acquired temporary weakening of their Kell antigens. Some of the patients also have allo-anti-K in their serum. This report presents a case in which an IgG autoantibody may define a new high-incidence red cell antigen related to the Kell blood groups. The patient's Kell blood group antigens are depressed, and his serum contains allo-anti-K. It is postulated that reduced red cell Kell antigenicity is caused by enzymatic degradation, possibly of bacterial origin, and that the acquired loss of Kell antigens, the Kell-specific autoimmune state, and the serum all0-anti-K, are all related aspects of one phenomenon.

Adult↗
PubMed1979

Hereditary acanthocytosis associated with the McLeod phenotype of the Kell blood group system.

Some boys with X-linked chronic granulomatous disease (CGD) have red cells of the rare McLeod phenotype in the Kell blood group system. Only one example of this phenotype has previously been described in a non-CGD subject. We have studied a 10-year-old boy and a maternal brother who do not have CGD and whose red cells are of the McLeod type . The boy presented as a haematological problem with red-cell abnormalities. These were acanthocytosis, anisocytosis and 'tailing' in the osmotic fragility curve, changes now known to occur with the McLeod phenotype. Subsequent studies revealed his rare blood group. A family study has established that an uncle also has acanthocytic red cells and the McLeod phenotype. In addition the boy's sister, mother and maternal grandmother all show red-cell mosaicism with double populations of McLeod acanthocytes and normal red cells of common Kell type. The gene that determines inheritance of the McLeod phenotype is X-linked and the mosaicism present in female carriers is believed to result from X chromosome inactivation by the Lyon effect. The study provides further evidence that the McLeod phenotype arises by inheritance of a variant X-linked modifying gene and not through inheritance of a variant gene at the Kell autosomal locus. It also represents the first occasion that a person of rare blood group has been recognized because of an associated anomaly in red cell morphology.

Acanthocytes↗
PubMed1979

Auto-immune hemolytic anemia caused by anti-K13.

A case of auto-immune hemolytic anemia caused by auto anti-K13 is described. Direct antiglobulin tests using monospecific reagents showed that IgG and the C3 component of complement were present on the patient's red cells. Eluted auto-antibody did not react with Ko or with K:--13 cells. Kell blood group antigenicity of the patient's red cells did not appear to be reduced.

Anemia, Hemolytic, Autoimmune↗
PubMed1979

Anti-KL.

Explore the source record for details and available documents.

Blood Group Antigens↗
PubMed1978

Staging laparotomy in Hodgkin's disease.

Staging laparotomy was performed at the University of Virginia Medical Center on 111 patients with Hodgkin's disease. The operation included multiple liver and lymph node biopsies and, excepting three patients, splenectomy. The histopathology was reviewed and the 111 patients were classified as follows: nodular sclerosis, 74; mixed cellularity, 28; lymphocyte predominance, 7; and undetermined, 2. There were no deaths. Wound, pulmonary or urinary tract complications occurred in 11 patients. One case of postoperative thrombophlebitis occurred and in another case small bowel obstruction developed, and resolved without reoperation. The pathologic stage (PS) following laparotomy was unchanged from the clinical stage (CS) in 64%, reduced in 20%, and advanced in 16%. The therapy, however, was altered in 38% of the patients. Lymphangiography in 103 patients was interpreted as showing lymph node involvement in 38, equivocal involvement in 11, and no involvement in 54. Among the 92 examinations reported as either positive or negative, 77% were confirmed histopathologically, 21% were falsely positive, and 2% were falsely negative. The spleen was positive for Hodgkin's disease in 39% of cases, and in these patients with positive spleens there was no reason to suspect intra-abdominal involvement preoperatively in 21%.

Abdominal Neoplasms↗