ABSTRACT
Acute promyelocytic leukemia (APL) is characterized by the presence of rearrangements involving the retinoic acid receptor alpha (RARalpha) gene and a variable incidence in different populations. The hybrid gene PML-RARalpha, present in 98% of cases, encodes a fusion protein essential to the pathogenesis of the disease. Depending of the PML's gene breakpoint in chromosome 15, the transcript subtypes bcr1, bcr2 and bcr3 may be formed. The correlation between these transcript subtypes and clinical parameters is still controversial. The objective of this study was to determine the frequencies of the PML-RARalpha transcripts and subtypes in a series of 32 APL patients from Northeast Brazil and to evaluate the association of these subtypes to different parameters. The method used was RT-PCR. The frequency of our APL cases is approximately 28% of the acute leukemias. The results showed the presence of PML-RARalpha isoform in all patients and a higher frequency of the bcr1/2 subtype. No significant statistical association was found between molecular subtypes and age, sex, French-American-British (FAB) classification, leukocyte and platelet count, hemoglobin level or coagulation tests. In conclusion, these data suggest similar molecular and biological features for our APL patients at diagnosis in comparison with those reported in current scientific literature.
Subject(s)
Leukemia, Promyelocytic, Acute/genetics , Neoplasm Proteins/genetics , Oncogene Proteins, Fusion/genetics , Adolescent , Adult , Brazil , Child , Female , Hemoglobins/metabolism , Humans , Leukocyte Count , Male , Middle Aged , Neoplasm Proteins/blood , Oncogene Proteins, Fusion/blood , Platelet Count , Protein Isoforms/blood , Protein Isoforms/genetics , Proto-Oncogene Proteins c-bcr/geneticsABSTRACT
The multidrug resistance P-glycoprotein is a transmembrane efflux pump expressed by lymphocytes and is involved in their cytolytic activity. In the present study, we investigated the age-related changes of P-glycoprotein function in normal peripheral blood lymphocytes. Blood samples from 90 normal volunteers (age range, 0 to 86 years) were analyzed. P-glycoprotein function was assessed by the flow cytometric rhodamine 123 assay. P-glycoprotein function was highest in cord blood and progressively declined with age in peripheral blood T CD4+ and CD8+ cells. In contrast, P-glycoprotein function did not vary with age in CD19+ B or CD16+CD56+ natural killer cells. These data suggest that the decline in P-glycoprotein function in T CD4+ and CD8+ lymphocytes as a function of age may contribute to the decrease in T cell cytolytic activity with aging.
Subject(s)
ATP Binding Cassette Transporter, Subfamily B, Member 1/physiology , Drug Resistance, Multiple/physiology , T-Lymphocytes/physiology , Adolescent , Adult , Age Factors , Aged , Aged, 80 and over , Aging/physiology , Child , Child, Preschool , Flow Cytometry , Fluorescent Dyes , Humans , Infant , Infant, Newborn , Killer Cells, Natural/physiology , Middle Aged , Rhodamine 123 , Stem Cells/physiologyABSTRACT
The multidrug resistance P-glycoprotein is a transmembrane efflux pump expressed by lymphocytes and is involved in their cytolytic activity. In the present study, we investigated the age-related changes of P-glycoprotein function in normal peripheral blood lymphocytes. Blood samples from 90 normal volunteers (age range, 0 to 86 years) were analyzed. P-glycoprotein function was assessed by the flow cytometric rhodamine 123 assay. P-glycoprotein function was highest in cord blood and progressively declined with age in peripheral blood T CD4+ and CD8+ cells. In contrast, P-glycoprotein function did not vary with age in CD19+ B or CD16+CD56+ natural killer cells. These data suggest that the decline in P-glycoprotein function in T CD4+ and CD8+ lymphocytes as a function of age may contribute to the decrease in T cell cytolytic activity with aging.
Subject(s)
Adolescent , Infant, Newborn , Infant , Child, Preschool , Child , Adult , Middle Aged , Humans , Drug Resistance, Multiple , ATP Binding Cassette Transporter, Subfamily B, Member 1 , T-Lymphocytes , Age Factors , Aged, 80 and over , Flow Cytometry , Fluorescent Dyes , Rhodamine 123Subject(s)
ATP Binding Cassette Transporter, Subfamily B, Member 1/metabolism , Aging/metabolism , Fluorescent Dyes/metabolism , Hematopoietic Stem Cells/metabolism , Rhodamine 123/metabolism , Adolescent , Adult , Aged , Aged, 80 and over , Biological Transport/drug effects , Bone Marrow/metabolism , Child , Hematopoietic Stem Cells/drug effects , Humans , Middle Aged , Reference Values , Verapamil/pharmacologyABSTRACT
BACKGROUND: Idiopathic juxtafoveolar telangiectasis (IJT) is a rare retinal vasculopathy of adulthood that may cause central loss of visual acuity. The purpose of this paper is to evaluate the type and distribution of fundus changes in patients with this disorder. METHODS: Medical records of 19 patients with IJT using the Gass & Blodi classification were studied. Eight patients in group 1A (42%) and 11 in group 2A (58%) were analyzed. By projecting color photographs we divided the macula into four quadrants and studied the fundus changes in each one. RESULTS: All patients in group 1A presented unilateral disease with easily seen telangiectasis, lipid deposits and cystic macular edema. The mean age of this group was 55.8 years. Five (63%) patients were male and 3 (37%) were female. All patients in group 2A, 2 males (18%) and 9 females (82%), had bilateral disease evidenced by fluorescein angiography. The mean age of this group was 54.5 years. Fundoscopic findings included: intraretinal pigment plaques, grayish coloring of the macular retina, superficial crystalline deposits, right-angle venules, subretinal and intraretinal neovascularization. CONCLUSIONS: All patients in this survey were adults. In group 1A, men were affected 2 times more often than women, and the main affected macular quadrant was the inferior temporal, followed by the inferior nasal. In group 2A, women were affected 4 times more often than men and all eyes had inferior and superior temporal macular quadrant involvement.