RESUMEN
We report increased numbers of hematogones in a 7-year-old girl with pancytopenia due to Shwachman-Diamond syndrome. Her hematogones expressed the T-cell marker CD5 as well as CD19, CD10, and CD20, and terminal deoxynucleotidyl transferase and HLA-DR. These findings suggest that hematogones are precursors of both CD5-positive B cells and CD5-negative B cells. Thus CD5-positive B cells in bone marrow may be derived from bone marrow stem cells, and not from the residual fetal B cells of yolk sac/liver origin. The finding of CD5 expression on hematogones also raises the possibility that neoplastic B cells of chronic lymphocytic leukemia, which characteristically co-express CD5 and CD19, may be derived from CD5-positive B-cell precursors in bone marrow and not from mature B cells in lymph nodes.
Asunto(s)
Linfocitos B/patología , Antígenos CD5/biosíntesis , Insuficiencia Pancreática Exocrina/patología , Células Madre Hematopoyéticas/patología , Linfocitos B/metabolismo , Células de la Médula Ósea/metabolismo , Células de la Médula Ósea/patología , Niño , ADN Nucleotidilexotransferasa/biosíntesis , Insuficiencia Pancreática Exocrina/diagnóstico por imagen , Insuficiencia Pancreática Exocrina/metabolismo , Femenino , Citometría de Flujo , Trastornos del Crecimiento , Antígenos HLA-DR/biosíntesis , Células Madre Hematopoyéticas/metabolismo , Humanos , Técnicas para Inmunoenzimas , Inmunofenotipificación , Hibridación Fluorescente in Situ , Leucemia Linfocítica Crónica de Células B/etiología , Leucemia Linfocítica Crónica de Células B/metabolismo , Leucemia Linfocítica Crónica de Células B/patología , Páncreas/diagnóstico por imagen , Páncreas/patología , Síndrome , Tomografía Computarizada por Rayos XRESUMEN
BB/Wor rats develop autoimmune diabetes mellitus with many features in common with human insulin-dependent diabetes mellitus. Since retinoids are known to have effects on insulin secretion and immune function, these studies were designed to investigate the effects of retinoid deficiency on diabetes in BB/Wor rats and to identify a role for retinoid status in the pathogenesis of autoimmune diabetes mellitus. Litters of diabetes-prone (DP) and diabetes-resistant (DR) BB/Wor rats were divided at weaning and fed a diet either (1) devoid of retinoids and leading to clinical deficiency at approximately 60 days of age (A-def diet)-following 10 days of clinical deficiency, rats on the A-def diet were changed to a diet containing 2 microg/g retinoic (A-def/RA diet); (2) containing 2 microg/g retinoic acid but deficient in retinol (RA diet); or (3) replete in retinol with 4 microg/g retinyl palmitate (RP diet). Rats receiving RP or RA diets were pair-fed to rats on the A-def/RA diet. Diabetes by 120 days of age was greatly reduced (P < .01) in DP rats that received the A-def/RA diet (four of 27) or RA diet (four of 29) versus the RP diet (13 of 31). Insulitis progressed with age in nondiabetic DP rats receiving the RP diet (P < .02) or RA diet (P < .05), but not the A-def/RA diet (P > .22). Insulin secretion was measured in perfused pancreas of nondiabetic rats after age 120 days and correlated negatively with insulitis (P < .05). DP rats receiving the RP diet had reduced insulin secretion as compared with other DP and DR rats (P < .05). In DR rats, retinoid status had no effects on insulitis through 120 days of age or on insulin secretion after 120 days of age. In conclusion, retinol deficiency reduces diabetes and insulitis in DP BB/Wor rats, and retinoic acid can at least partly substitute for retinol in the development of insulitis.
Asunto(s)
Diabetes Mellitus Tipo 1/etiología , Insulina/metabolismo , Islotes Pancreáticos/metabolismo , Deficiencia de Vitamina A/complicaciones , Animales , Dieta , Femenino , Inflamación , Secreción de Insulina , Islotes Pancreáticos/patología , Masculino , Ratas , Ratas Endogámicas BBRESUMEN
We report the first case of benign aldosteronoma of an ordinary size with calcifications. We review the clinical, clinical imaging, histopathological, and laboratory features of aldosterone-producing adrenal adenoma versus carcinoma. We conclude that no single feature is diagnostic, and the full range of data must be considered. Calcifications may not necessarily be a distinguishing point.