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Biomedical subjects

W L Marsh

Publications and source records attributed to W L Marsh.

At least 55 records · Page 3Linked to original sources
PubMed1989

Effect of phosphatidylserine on the shape of McLeod red cell acanthocytes.

The rare McLeod blood group phenotype is characterized by weak Kell antigens, lack of the common Kx antigen, and acanthocytic morphology. Previous studies that did not detect membrane or cytoskeletal protein abnormalities suggested a lipid disturbance. In normal red cells, dimyristoyl phosphatidylserine (DMPS) is transported across the membrane by an enzymatic process and accumulates in the inner leaflet of the membrane bilayer causing discocyte to stomatocyte shape changes. Scanning electron microscopy of McLeod red cells shows a mixture comprised of 15% discocytes, 51% with irregular surfaces, and 34% acanthocytes. On incubation with various concentrations of DMPS at 37 degrees C for periods up to two hours, McLeod red cells transported DMPS across the membrane and caused irregularly shaped and acanthocytic McLeod red cells to attain normal discocyte shape and later to become stomatocytes. Chlorpromazine, which at 0 degrees C preferentially partitions into the inner monolayer of the membrane, had a similar effect on the shape of McLeod red cells. This suggests that in McLeod cells acanthocytosis is due to a lack of lipid in the inner leaflet of the membrane bilayer but that the imbalance is not caused by defective transport of phosphatidylserine across the membrane.

Acanthocytes↗
PubMed1988

Hematologic evaluation of employees with leukopenia. Naval Weapons Center, China Lake, California.

Evaluation of 86 employees with a history of leukopenia at the Naval Weapons Center (NWC), China Lake, California, was done by exposure questionnaires, medical histories, physical examinations, peripheral blood smear, and bone marrow evaluations, including morphologic examination, stem cell culture, and cytogenetics. Forty-eight subjects were found to be leukopenic at the time of the study, and two subjects were found to have hairy cell leukemia. All subjects had positive exposure histories and were healthy at the time of the study. Review of peripheral smears identified the patients with marrow abnormalities. Bone marrow cultures revealed several patients with possible marrow suppression. Chromosome studies were not diagnostic. Five-year follow-up health questionnaires revealed no significant health problems; the two workers with hairy cell leukemia are alive and fully functional. Leukopenia in itself does not appear to be a risk factor for poor health, and it is unknown whether or not it may be a useful screening tool to identify workers at risk in toxic environments. Careful evaluation of blood cell counts and peripheral smears should be sufficient to identify people with potential marrow abnormalities.

Adult↗
PubMed1988

Biochemical studies on McLeod phenotype red cells and isolation of Kx antigen.

Red cells of the McLeod blood group phenotype have weak Kell antigens, lack Kx antigen and have acanthocytic morphology. We have immunoprecipitated Kell antigens from McLeod red cells and show that they are markers on the same 93 kDa membrane protein that carries Kell antigens on normal red cells. However, as determined by Western immunoblotting, McLeod red cells have a marked deficiency of this protein. We have also studied the near-neighbour relationship of McLeod and common Kell red-cell membrane proteins by cross-linking intrinsic sulphydryl groups by oxidation, catalysed with orthophenanthroline and copper, or by cross-linking amino groups with dimethyl-3,3'-dithiobispropionimidate. Results were analysed by diagonal mapping in two-dimensional gels. No abnormalities of membrane protein inter-relationship were detected in McLeod red cells. We have isolated Kx antigen from K0 red cells by immunoprecipitation with human alloimmune anti-Kx serum, isolation of immune complexes from detergent-solubilized cell membranes with protein A-Sepharose and analysis of the eluted immune complex by SDS-PAGE under reducing conditions. Kx antigen is a marker on a red-cell membrane protein of approximately 37 kDa. Ko (Knull) red cells have about twice the amount of Kx antigen as do red cells of common Kell type. McLeod red cells have no detectable Kx antigen by serological tests or by immunoprecipitation.

Antigens, Bacterial↗
PubMed1988

Blood grouping with the Olympus PK7100 testing system.

The Olympus PK7100, an automated microtitre blood typing machine, identifies, aliquots, and dilutes samples, adds reagents, photometrically detects agglutination, and records reaction results, with a throughput of 240 samples/h. A total of 20,147 donors was tested in parallel with the Groupamatic 360. Of these, 207 could not be ABO typed after two runs. Three samples typed as B by the Olympus were found to be A2B. Seventy-seven could not be typed for D after two runs. Of these, 55 were Rh positive and 22 negative. The Olympus identified 37 of 48 Du positive samples as Rh positive, while it typed three as Rh negative, and eight as 'uncertain'. None of these samples was identified as Rh positive by the Groupmatic 360. The Olympus PK7100 is accurate, reliable, easy to operate, and capable of high throughput with minimal operator intervention.

ABO Blood-Group System↗
PubMed1988

Sinus histiocytosis with massive lymphadenopathy. Occurrence in identical twins with retroperitoneal disease.

Sinus histiocytosis with massive lymphadenopathy (SHML) was originally defined as a relatively specific benign pseudolymphomatous disorder. Although the etiology remains unknown, the spectrum of SHML has been expanded to include predominance of extranodal disease in some patients, clinically significant immunologic abnormalities in 10% of patients, and fatal outcome in 7% of patients. We report the rare occurrence of SHML in identical twins; to our knowledge, SHML in identical twins has been reported only once previously. The two patients described are also unusual because of the predominance of retroperitoneal disease with minimal peripheral adenopathy. After a seven-year clinical course, one twin died of extensive retroperitoneal disease, liver failure, bleeding diathesis, and seizure disorder. The other twin is alive after a six-year course of progressive retroperitoneal disease.

Adolescent↗
PubMed1988

Localization of the McLeod locus (XK) within Xp21 by deletion analysis.

The McLeod phenotype is an X-linked, recessive disorder in which the red blood cells demonstrate acanthocytic morphology and weakened antigenicity in the Kell blood group system. The phenotype is associated with a reduction of in vivo red cell survival, but the permanent hemolytic state is usually compensated by erythropoietic hyperplasia. The McLeod phenotype is accompanied by either a subclinical myopathy and elevated creatine kinase (CK) or X-linked chronic granulomatous disease (CGD). Seven males with the McLeod red-blood-cell phenotype and associated myopathy but not CGD, one male with the McLeod phenotype associated with CGD, and two males known to possess large deletions of the Duchenne muscular dystrophy (DMD) locus were studied. DNA isolated from each patient was screened for the presence or absence of various cloned sequences located in the Xp21 region of the human X chromosome. Two of the seven males who have only the McLeod phenotype and are cousins exhibit deletions for four Xp21 cloned fragments but are not deleted for any portion of either the CGD or the DMD loci. Comparison of the cloned segments absent from these two McLeod cousins with those absent from the two DMD boys and the CGD/McLeod patient leads to the submapping of various cloned DNA segments within the Xp21 region. The results place the locus for the McLeod phenotype within a 500-kb interval distal from the CGD locus toward the DMD locus.

Adult↗
PubMed1987

Presacral myelolipoma.

Extramedullary tumors of hematopoietic tissue can be divided into two categories: those associated with a bone marrow disorder, myelolipomas which have no apparent relation to a bone marrow disorder. The etiology of myelolipoma is unknown and the vast majority are adrenal in location. Extramedullary myelolipomas are usually presacral in location and none of the approximately ten reported cases has been diagnosed before surgery. Although radiographic and ultrasound features of myelolipomas are relatively characteristic, they are not specific and biopsy is required for diagnosis. Microscopic diagnosis requires demonstration of benign lipomatous tissue admixed with cellular hematopoietic tissue resembling normal bone marrow. This study reports a patient with presacral myelolipoma and an unusual finding of urinary retention and azotemia associated with compression of urinary bladder neck by the myelolipoma.

Female↗
PubMed1987

Acquired loss of red cell Kell antigens.

A 19-year-old patient with a long history of idiopathic thrombocytopenic purpura developed a potent antibody against a high-incidence antigen in the Kell blood group system. The direct antiglobulin test on his red cells was negative. His cells exhibited profound depression of Kell blood group antigens, but antigens of other blood groups were normal. Transfusion of incompatible blood was well tolerated and differential agglutination tests, using selected Rh antisera, showed in vivo survival of the transfused red cells for more than 8 weeks. However, the transfused red cells also showed acquired loss of Kell antigens. Five months after the initial findings, Kell-related antibody disappeared and Kell antigens reappeared on his red cells. The patient's serum stored from the initial investigation now reacted with his freshly collected red cells. These data suggest that an environmental agent in the patient's plasma was responsible for the temporary loss of Kell antigens from red cells in his circulation.

Adult↗
PubMed1987

Evaluation of red cell volume distribution width (RDW).

The purpose of this study was to evaluate red cell distribution width (RDW) in the differential diagnosis of patients with well defined iron deficiency (ID), anemia of chronic disease (ACD), and thalassemia trait. Mean RDW in 100 "normals" was 13.1%. Elevated RDW (greater than 15.0%) was observed in 90% of ID (N = 39), 32% of ACD(N = 79), 66% of beta-thalassemia trait (N = 100), 53% of alpha-thalassemia trait men (N = 127), 34% of alpha-thalassemia trait women (N = 116), 3% of sickle cell trait (N = 101), and 88% of sickling disorders (N = 32). The Bessman classification by mean corpuscular volume and RDW was of limited value in separating ID from thalassemia trait but was improved by adopting 16.0% as the upper limit of normal for RDW. Although many attempts have been made to discriminate between ID and thalassemia trait on the basis of red cell values from a cell counter, none (including RDW) is sufficiently specific or sensitive to obviate the need for confirmation of the diagnosis by standard laboratory methods.

Adult↗
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↗
PubMed1986

Osteoarticular and pulmonary manifestations of acute leukemia. Case report and review of the literature.

Acute leukemia with bone or joint symptoms of sufficient magnitude to obscure and delay the correct diagnosis has been reported but is rarely mentioned in the recent literature, particularly in adults. The authors report an adult with rapidly fatal acute leukemia and a clinical course dominated by recurrent osteoarticular symptoms; osteoarticular symptoms preceded the diagnosis of leukemia by 3 months and delayed diagnosis and treatment. The correct diagnosis requires awareness of the osteoarticular manifestations of acute leukemia and examination of blood or bone marrow smear by an experienced observer; a biopsy specimen of bone or joint lesions or examination of cytocentrifuged synovial fluid may aid in diagnosis. The immediate cause of death in our patient was respiratory failure with prominent leukemic interstitial pulmonary infiltrates at autopsy; clinically significant leukemic pulmonary infiltrates have only rarely been reported. The authors review the literature on pulmonary and osteoarticular manifestations caused by acute leukemic infiltrates.

Acute Disease↗
PubMed1986

Castleman's disease in children.

A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castleman's disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms were consistent with those seen in the plasma-cell type of this disease. The boy presented with acute appendicitis and a left hilar mass was noted on his chest radiograph. His asymptomatic presentation was typical of the hyaline-vascular form. Both patients are well without evidence of recurrence four years following resection. Castleman's disease is a benign disorder of lymph nodes that occurs rarely in children. Since the original report in 1954 we could find only 18 cases in the 16-year and younger age group and our patient with the symptomatic form represents the youngest patient reported in the English literature. The management requires surgical resection of the enlarged nodes both for diagnosis and therapy since the enlarged nodes can mimic malignant tumors of the lymphoid system. No recurrences have been reported in pediatric patients.

Child↗
PubMed1986

Acquired loss of red-cell Wj antigen in a patient with Hodgkin's disease.

A patient with Hodgkin's disease became temporarily Wj-negative with alloanti-Wj in his serum. Four human autoantibodies, and 1 of 2 murine monoclonal antibodies, with serological characteristics of anti-Wj were nonreactive with his red cells, confirming that they have anti-Wj specificity. Six siblings of the patient are all Wj-positive. The patient was also temporarily Anton-negative, and cross-testing between Wj and Anton red cells and antisera showed mutual compatibility, indicating that the antigens are the same. The patient and 3 of his 6 siblings are also of the rare Lu: - 13 phenotype, providing the first evidence that this is an inherited characteristic.

Adult↗
PubMed1986

Coccidioidomycosis of the female genital tract.

Female genital tract involvement is a rare manifestation of disseminated coccidioidomycosis; to our knowledge, only ten patients have previously been described in the English literature. We describe a patient who seems to be unique in that she developed female genital tract coccidioidomycosis and coccidioidal peritonitis after chemotherapy for Hodgkin's disease. Coccidioidomycosis of the female genital tract is usually manifest as granulomatous endometritis and/or granulomatous tubo-ovarian disease with peritonitis. The diagnosis of coccidioidomycosis was unsuspected clinically in all 11 reported cases (including our patient); initial diagnosis was made by biopsy or culture in all 11 patients. In eight of the reported cases of female genital tract coccidioidomycosis (including our patient), clinical improvement occurred after treatment with surgery or antifungal chemotherapy; three patients died of disseminated coccidioidomycosis.

Adult↗
PubMed1985

Severe congenital neutropenia with unique features of dysgranulopoiesis.

Congenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3-year-old girl with neutropenia (less than 500/mm3) and recurrent severe infections in whom repeated marrow evaluations revealed large (30-50 microns) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly, immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony-forming cells, and normal colony-stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B12, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously described cases.

Agranulocytosis↗