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Biomedical subjects

W L Marsh

Publications and source records attributed to W L Marsh.

At least 91 records · Page 5Linked to original sources
PubMed1980

Bone marrow and lymph node findings in a fatal case of Kawasaki's disease.

Since the initial description in 1967, the clinical and laboratory features of Kawasaki's disease (KD) have been well documented. We studied a patient with KD who recovered from the acute phase of the disease, but who subsequently died at home from coronary arteritis. We describe this patient because of bone marrow and lymph node findings that have previously received little attention in the English-language literature.

Arteritis↗
PubMed1980

Chronic granulomatous disease and McLeod syndrome in a black child.

A 3-year-old black male child with X-linked chronic granulomatous disease and red cells of the rare McLeod phenotype is presented. The red cells showed acanthocytosis and did not react with anti-KL. Similarly the leukocytes were nonreactive with anti-Kx. The Xk and Xg linkage could not be investigated since all members of his family were Xg (a+).

Acanthocytes↗
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.

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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↗
PubMed1978

Aplastic anemia with fetallike erythropoiesis following androgen therapy.

A 43/4-yr-old black girl with acquired aplastic anemia had an increase in total hemoglobin (Hb) from 4.5 to 16.8 g/dl and fetal hemoglobin (HbF) from 0.8 g/dl (18.8%) to 9.6 g/dl (60.2%) following combined androgen-adrenal steroid therapy. Discontinuation of the drugs was followed by a decline in both HbF and total Hb. Reinstitution of the combined steroids prompted a second rise in total and fetal hemoglobin. During these responses the subject's erythrocytes exhibited an increased i antigen score and a low level of red cell carbonic anhydrase. The glycine:alanine ratio at position 136 of the gamma chains of HbF was of the fetal type (proportion of chains with glycine residues, 0.74). Hemoglobin A2 was low (0.4%). The synthesis of alpha and non-alpha chains was balanced. These results indicate that the stimulation of red cell proliferation in this subject, in response to androgen therapy, resulted in the production of cells with several characteristics of "fetal" erythrocytes.

Anemia, Aplastic↗
PubMed1977

Haematological changes associated with the McLeod phenotype of the Kell blood group system.

The McLeod phenotype is inherited as an X-linked characteristic. The red cells have weak antigenicity in the Kell blood group and lack Kx, a precursor-like substance that appears to be necessary for proper biosynthesis of Kell antigens. Kx antigen is also required for establishment of normal cell morphology. Absence of Kx antigen causes a membrane abnormality, in which the most prominent feature is acanthocytosis, and a compensated haemolytic state. The X-linked gene that determines normal Kx production is called X1k. Inheritance of a variant allele at the Xk locus is responsible for lack of Kx synthesis and the McLeod phenotype. The Xk locus is inactivated by the Lyon effect, and female carriers of the variant gene exhibit blood group mosaicism in the Kell system and have a dual red cell population of acanthocytes and discocytes.

Acanthocytes↗
PubMed1977

Anti-Lu14: a Lutheran antibody defining the product of an allele at the Lu8 blood group locus.

A 'new' Lutheran-related antibody, named anti-Lu14, reacts with approximately 2.4% of random bloods. Red cells of the rare Lu:-8 phenotype are Lu:14. The data indicate, with a high probability, that the Lu 14 antigen is a product of an allele of Lu8 and that Lu14 and Lu8 comprise a third pair of alleles at the Lutheran locus. Red cells of the original Sw (a+) propositus are Lu:14. By coincidence, he has inherited two low-incidence genes. This observation may explain the discrepancy in different families concerning a possible relationship between Swa and Lutheran. Pedigree information now suggests that Swa is not a Lutheran gene.

Alleles↗
PubMed1977

Mapping assignment of the Rh and Duffy blood group genes to chromosome 1.

Blood group genes are inherited in a straightforward manner and their products are readily detectable. Because of this they are utilized over a wide area of scientific endeavor and provide excellent markers for use in gene mapping of the human chromosomes. Both the Rh and Duffy blood group genes have now been localized to the number 1 chromosome. Studies of a patient whose red cells exhibit mosaicism for the Rh blood group indicate that loss of the end of the short arm of chromosome number 1 is associated with loss of an Rh gene complex. The Rh gene can, therefore, be assigned with some precision to this region of the chromosome.

Blood Group Antigens↗
PubMed1977

Erythrocyte morphology in genetic defects of the Rh and Kell blood group systems.

Absence of KX antigen and of normal expression of the Kell system antigens is associated with bizarre red blood cell morphology when observed by either light or scanning electron microscopy. Numerous acanthocytes and dacryocytes have been observed in the peripheral blood smear of an apparently healthy individual with McLeod-phenotype blood, in a male patient with type II chronic granulomatous disease who had a shortened 51Cr red blood cell survival time, and in a minor population of the red blood cells of his carrier mother.

Blood Group Antigens↗
PubMed1977

The Kell blood group, Kx antigen, and chronic granulomatous disease.

The Kell blood group has 18 associated red cell antigens. One, named KX, is the product of an X-linked gene and appears to be a precursor in the Kell biosynthetic pathway. Lack of KX on red cells, caused by inheritance of a variant allele at the X-linked locus, results in gross changes in Kell antigenicity, an effect called the McLeod phenotype. Such cells also show striking morphologic changes. Normal phagocytic leukocytes lack Kell antigens but have strong KX. The leukocytes of boys with X-linked chronic granulomatous disease lack KX antigen and have defective bactericidal function. The fundamental defect in chronic granulomatous disease appears to be failure to inherit the X-linked gene that determines KX synthesis. The enzymatic and functional disorders of the leukocytes, and the structural changes in the red cells, are consequences that follow.

Antibodies↗
PubMed1976

Lymph node involvement by direct extension in adenoid cystic carcinoma. Absence of classic embolic lymph node metastasis.

Thirty-four cases of adenoid cyctic carcinoma seen at the University of Virginia Hospital from 1946 to 1974 were reviewed, with special emphasis on lymph node involvement by tumor. Lymph node involvement was found in three cases of primary tumors of the submaxillary gland, and all of the affected lymph nodes were in the immediate vicinity of the primary tumor. Two lymph nodes were involved in two of the cases, and one node was involved in the third case. In all of these lymph nodes, adenoid cystic carcinoma was present in the soft tissue surrounding the node, and the tumor extended into the node. No metastatic tumors were observed in 46 lymph nodes removed incidentally at the time of local excision of the primary tumors in 10 additional cases or in 212 lymph nodes examined after unilateral radical neck dissections in six other cases. Five autopsies in this series showed no lymph node metastases. In this series of cases adenoid cystic carcinoma only invades lymph nodes in the immediate vicinity of the primary tumor. When lymph node involvement does occur, it does not result from embolic lymph node metastasis; rather, a direct invasion of the lymph node from tumor in the perinodal soft tissue occurs. Obviously, this small study does not completely exclude the possibility of embolic metastasis; however, if it does occur, it must be extremely rare.

Carcinoma, Adenoid Cystic↗