Search

Your search keyword '"R. Jaffe"' showing total 9 results
Start Over Author "R. Jaffe" Journal blood
9 results on '"R. Jaffe"'

1. G6PD Deficiency and Chronic Hemolysis: Four New Mutants—Relationships Between Clinical Syndrome and Enzyme Kinetics

Author

Laurence M. Corash, Mario C. Rattazzi, Ernst R. Jaffe, Sergio Piomelli, and George E. Van Zanen

Subjects
chemistry.chemical_classification, congenital, hereditary, and neonatal diseases and abnormalities, Heterogeneous group, Immunology, Mutant, nutritional and metabolic diseases, Cell Biology, Hematology, Biology, medicine.disease, Biochemistry, Hemolysis, Biochemical kinetics, Enzyme, chemistry, hemic and lymphatic diseases, parasitic diseases, medicine, Enzyme kinetics, Clinical syndrome
Abstract

G6PD deficiency of the common type (GdA- and GdMediterranean) results in extremely mild chronic hemolysis. In contrast, 65 males (from 47 unrelated families) have been reported with a different syndrome of severe chronic hemolysis associated with a superficially similar deficiency in the activity of G6PD. Five new such patients (from four unrelated families) are reported. Biochemical characterization of the erythrocyte G6PD from these patients indicates that these four mutant enzymes are different from each other and from previously reported variants. These new mutants have tentatively been named G6PD New York, G6PD Englewood, G6PD Rotterdam and G6PD Den Haag. The congenital nonspherocytic hemolytic anemias associated with G6PD deficiency appear to be an extremely heterogeneous group from the point of view of biochemical kinetics. The relationships between the clinical syndrome and the various biochemical enzyme characteristics are discussed in the light of the information presently available.

Published
1971
Full Text
View/download PDF

2. Experiences of the First 493 Unrelated Marrow Donors in the National Marrow Donor Program

Author

Stroncek, David F., Holland, Paul V., Bartch, Glenn, Bixby, Todd, Simmons, Roberta G., Antin, Joseph H., Anderson, Kenneth C., Ash, Robert C., Bolwell, Brian J., Hansen, John A., Heal, Joanna M., Jean Henslee-Downey, P., R. Jaffe’, Ernst, Klein, Harvey G., Lau, Peter M., Perkins, Herbert A., Popovsky, Mark A., Price, Thomas H., Rowley, Scott D., Stehling, Linda C., Weiden, Paul L., Wissel, Mary E., and McCullough, Jeffrey

Abstract

More than 410,000 people participated in the National Marrow Donor Program (NMDP) as of October 1, 1991, and more than 850 volunteers had donated marrow. While the incidence of serious morbidity as a result of bone marrow donation is rare, the incidence of lesser complications and the long-term consequences of marrow donation are not known. To determine the incidence of donor complications and measure the recovery time of volunteer, unrelated marrow donors, we analyzed the results of surveys of the first 493 persons who donated marrow through the NMDP. The marrows were collected at 42 centers. The median age of the donors was 37.9 years (range 19.1 to 55.6 years). The median volume of marrow collected was 1,050 mL (range 180 to 2,983 mL). Autologous red blood cells were transfused to 89.8% (439) of donors but only 0.6% (3) of donors received allogeneic blood. Acute complications related to the collection procedure occurred in 5.9% of donors; but a serious complication, apnea during anesthesia, occurred in only one donor. When donors were questioned approximately 2 days following discharge from their hospitalization, most donors described symptoms related to the collection; 74.8% experienced tiredness, 67.8% experienced pain at the marrow collection site, and 51.6% of the donors experienced low back pain. Donors were surveyed repeatedly until they felt that they had recovered completely. Mean recovery time was 15.8 days; however, 42 (10%) donors felt that it took them ≥30 days to recover fully. The duration of the marrow collection procedure and duration of anesthesia both positively correlated with donor pain and/or fatigue following the collection; but the duration of the collection procedure had the highest correlation with post-collection pain and fatigue. The volume of marrow collected per unit of donor weight was more weakly correlated with donor pain and/or fatigue than the anesthesia and collection times. When multivariate analysis was used to analyze the correlation between donor recovery time and these variables, only the duration of the collection was found to correlate significantly with donor recovery time (P= .001). This analysis demonstrates that marrow donation is well tolerated with few complications. To decrease further the incidence of donor discomfort and recovery time following donation, the duration of the collection procedure, and probably the duration of anesthesia, and the volume of marrow collected, should be kept to a minimum.

Published
1993
Full Text
View/download PDF

7. ABSTRACTS

Author

R. Jaffe, Ernst

Published
1965

8. In Memoriam

Author

W. Adamson, M.D., John, A. Marks, M.D., Paul, and R. Jaffé, M.D., Ernst

Published
1984

9. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease.

Author

Abla O, Jacobsen E, Picarsic J, Krenova Z, Jaffe R, Emile JF, Durham BH, Braier J, Charlotte F, Donadieu J, Cohen-Aubart F, Rodriguez-Galindo C, Allen C, Whitlock JA, Weitzman S, McClain KL, Haroche J, and Diamond EL

Subjects
Adrenal Cortex Hormones therapeutic use, Biopsy, Disease Management, Genetic Predisposition to Disease, Histiocytes metabolism, Histiocytosis, Sinus genetics, Histiocytosis, Sinus pathology, Humans, Immunotherapy, Mutation, Practice Guidelines as Topic, Prognosis, Radiotherapy, Histiocytes pathology, Histiocytosis, Sinus diagnosis, Histiocytosis, Sinus therapy
Abstract

Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by accumulation of activated histiocytes within affected tissues. RDD, which now belongs to the R group of the 2016 revised histiocytosis classification, is a widely heterogeneous entity with a range of clinical phenotypes occurring in isolation or in association with autoimmune or malignant diseases. Recent studies have found NRAS , KRAS , MAP2K1 , and ARAF mutations in lesional tissues, raising the possibility of a clonal origin in some forms of RDD. More than 1000 reports have been published in the English literature; however, there is a lack of consensus regarding approach for the clinical management of RDD. Although in most cases RDD can be observed or treated with local therapies, some patients with refractory or multifocal disease experience morbidity and mortality. Here we provide the first consensus multidisciplinary recommendations for the diagnosis and management of RDD. These recommendations were discussed at the 32nd Histiocyte Society Meeting by an international group of academic clinicians and pathologists with expertise in RDD. We include guidelines for clinical, laboratory, pathologic, and radiographic evaluation of patients with RDD together with treatment recommendations based on clinical experience and review of the literature., (© 2018 by The American Society of Hematology.)

Published
2018
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results