[Soluble transferrin receptor and erythropoietin in chronic disease anemia with or without iron deficiency]. / Receptor soluble de transferrina y eritropoyetina en la anemia de los procesos crónicos con y sin deficiencia de hierro.

The regulation of transferrin receptor (RTF) is related to intracellular iron stores and with the soluble receptor is present in plasma. It has already been demonstrated that in iron deficiency anemia (IDA), receptor expression increases when iron stores decrease. In anemia of chronic diseases (ACD) it is difficult to establish the real iron status because of the influence exerted by inflammatory or infectious diseases on iron metabolism. We studied 30 healthy normal subjects and 42 anemic patients (hemoglobin less than 120 g/L) affected with ACD divided into two groups with and without iron deficiency, in order to establish the diagnostic value of measuring the soluble transferrin receptor (sRTF). We correlated erythropoietin (EPO) (as an erythropoietic stimulating factor) with the decreased hemoglobin values observed in both groups. The results were analysed with an ANOVA statistic test of one way analysis of variance, and there were no significant differences in sRTF values between the ACD groups with or without iron deficiency. The ratio log EPO vs hemoglobin showed a remarkably significant inverse correlation in both groups. We can conclude that sRTF levels are within the normal reference values in these patients and are not related to organic iron. Consequently, sRTF cannot be considered a good parameter for making a diagnosis of iron deficiency in chronic diseases.

Receptor soluble de transferrina y eritropoyetina en la anemia de los procesos crónicos con y sin deficiencia de hierro. / [Soluble transferrin receptor and erythropoietin in chronic disease anemia with or without iron deficiency]

The regulation of transferrin receptor (RTF) is related to intracellular iron stores and with the soluble receptor is present in plasma. It has already been demonstrated that in iron deficiency anemia (IDA), receptor expression increases when iron stores decrease. In anemia of chronic diseases (ACD) it is difficult to establish the real iron status because of the influence exerted by inflammatory or infectious diseases on iron metabolism. We studied 30 healthy normal subjects and 42 anemic patients (hemoglobin less than 120 g/L) affected with ACD divided into two groups with and without iron deficiency, in order to establish the diagnostic value of measuring the soluble transferrin receptor (sRTF). We correlated erythropoietin (EPO) (as an erythropoietic stimulating factor) with the decreased hemoglobin values observed in both groups. The results were analysed with an ANOVA statistic test of one way analysis of variance, and there were no significant differences in sRTF values between the ACD groups with or without iron deficiency. The ratio log EPO vs hemoglobin showed a remarkably significant inverse correlation in both groups. We can conclude that sRTF levels are within the normal reference values in these patients and are not related to organic iron. Consequently, sRTF cannot be considered a good parameter for making a diagnosis of iron deficiency in chronic diseases.

[Immunocytochemistry techniques for the diagnosis of hematologic neoplasms]. / Aplicación de técnicas inmunocitoquímicas para el diagnóstico de neoplasias hematólogicas.

We studied 22 patients with hematological neoplasias which included: 12 patients with a diagnosis of Acute Myeloblastic Leukemia (AML) following the morphology and cytochemistry criteria established by FAB (French, American and British Committee), a Myeloblastic Leukemia secondary to MDS (Myelodysplastic Syndromes) and a biphenotypic acute leukemia where we established the relationship between the traditional peroxidase reaction with the anti-MPO by APAAP. We also carried out the nonspecific esterase reaction and determined the immunologic phenotype by FACS technology. The same procedure was used for the cellular analysis of the light chains kappa (kappa) and lambda (lambda) in 3 cases of hairy cell leukemia, one lymphoma and 4 cases of plasma cell neoplasia and reactive plasma cell disease. We conclude that immunocytochemical reactions must be used when morphology and traditional cytochemical reactions need to be confirmed in order to establish a correct diagnosis and this is specially important for B and T lymphomas. Their prognostic value is restricted and the results are useful as a complement to morphology, cytochemistry and immunological determinations.

[Partial lecithin-cholesterol acyltransferase (LCAT) deficiency syndrome]. / Síndrome de deficiencia parcial de lecitina-colesterol aciltransferasa (LCAT).

This syndrome is a pathological entity of low incidence which mainly affects high density lipoprotein (HDL) metabolism. We here show the first case reported in our country, observed in a 63-year-old woman who showed bilateral corneal opacity and eruptive xanthomas in both arms. The lipoprotein profile disclosed severe hypertriglyceridemia and normocholesterolemia, although the percentage of cholesteryl esters was low. Plasma levels of HDL-cholesterol and HDL major apolipoproteins, A-I and A-II, were markedly decreased. The patient also showed glucose intolerance and hematological alterations related to abnormal lipid composition of erythrocyte membranes. As evaluated by the exogen substrate method, LCAT activity proved to be 82% lower in the patient than in a control subject. It is noteworthy that the patient had experienced cardiac events and presented hypertension, neither of which has been commonly documented in partial LCAT deficiency syndromes.

Aplicación de técnicas inmunocitoquímicas para el diagnóstico de neoplasias hematólogicas. / [Immunocytochemistry techniques for the diagnosis of hematologic neoplasms]

We studied 22 patients with hematological neoplasias which included: 12 patients with a diagnosis of Acute Myeloblastic Leukemia (AML) following the morphology and cytochemistry criteria established by FAB (French, American and British Committee), a Myeloblastic Leukemia secondary to MDS (Myelodysplastic Syndromes) and a biphenotypic acute leukemia where we established the relationship between the traditional peroxidase reaction with the anti-MPO by APAAP. We also carried out the nonspecific esterase reaction and determined the immunologic phenotype by FACS technology. The same procedure was used for the cellular analysis of the light chains kappa (kappa) and lambda (lambda) in 3 cases of hairy cell leukemia, one lymphoma and 4 cases of plasma cell neoplasia and reactive plasma cell disease. We conclude that immunocytochemical reactions must be used when morphology and traditional cytochemical reactions need to be confirmed in order to establish a correct diagnosis and this is specially important for B and T lymphomas. Their prognostic value is restricted and the results are useful as a complement to morphology, cytochemistry and immunological determinations.

Síndrome de deficiencia parcial de lecitina-colesterol aciltransferasa (LCAT). / [Partial lecithin-cholesterol acyltransferase (LCAT) deficiency syndrome]

This syndrome is a pathological entity of low incidence which mainly affects high density lipoprotein (HDL) metabolism. We here show the first case reported in our country, observed in a 63-year-old woman who showed bilateral corneal opacity and eruptive xanthomas in both arms. The lipoprotein profile disclosed severe hypertriglyceridemia and normocholesterolemia, although the percentage of cholesteryl esters was low. Plasma levels of HDL-cholesterol and HDL major apolipoproteins, A-I and A-II, were markedly decreased. The patient also showed glucose intolerance and hematological alterations related to abnormal lipid composition of erythrocyte membranes. As evaluated by the exogen substrate method, LCAT activity proved to be 82

lower in the patient than in a control subject. It is noteworthy that the patient had experienced cardiac events and presented hypertension, neither of which has been commonly documented in partial LCAT deficiency syndromes.

Forskolin induces U937 cell line differentiation as a result of a sustained cAMP elevation.

The present study examines the effects of forskolin on U937 cell differentiation. We recently reported that dibutyryl cAMP (dbcAMP), but not cAMP-elevating agents such as histamine, promotes U937 cell differentiation. cAMP production elicited by stimulation of histamine H2 receptors showed a rapid, homologous desensitization, which might explain the dissimilar responses to histamine and dbcAMP. Forskolin induced an increase in cAMP levels in a concentration-dependent manner (EC50=30 microM) for an extended period of at least 24 h. Forskolin but not histamine (up to 100 microM), also inhibited cell growth in a dose-dependent fashion (EC50=22 microM). After 3 days of incubation, 75 microM forskolin induced U937 cell differentiation as judged by an increased rate of reduction of nitrobluetetrazolium (mean+/-S.E.M.: 21.3+/-6.6% in treated cells vs. 3.2+/-1.9% in the control group, P < 0.001) and an augmented chemotactic response to complement 5a (C5a) (33.2+/-5.9% in forskolin-treated vs. 0.34+/-0.12% in control cells, P < 0.01). Furthermore, c-Myc levels decreased following forskolin treatment, while the histamine H2 receptor agonist dimaprit had no effect. We conclude that forskolin induces U937 cell differentiation through a sustained rise in cAMP levels.

Ferritina eritrocitaria: medición y aplicación diagnóstica / Red cell ferritin: measurement and diagnostic applications

El objeto de este estudio fue determinar la ferritina eritrocitaria (FER) por enzimoinmunoensayo como una medida directa de los depósitos de hierro intracelular presente en los eritroblastos de la médula ósea. Su uso está indicado en diferencias o en sobrecargas de hierro. Se estudiaron 40 pacientes divididos en tres grupos: 1) un grupo control (n = 16) donde se obtuvieron los siguientes valores de FER: (X ñ ES) = 24 ñ 2,5 ag/cel; 2) un grupo de pacientes ferropénicos (n = 13) con resultados de FER: (X ñ ES) = 5 ñ 0,31 ag/cel y 3) pacientes ß-talasémicos: (n = 11) donde la FER fue de (X ñ ES) = 118 ñ 11,6 ag/cel. Los resultados obtenidos mediante el test de análisis de la varianza de una vía (ANOVA) mostraron una diferencia estadísticamente significativa en los dos grupos estudiados, con respecto al grupo control. La medida de FER presenta la ventaja de ser una determinación cuantitativa que puede ser realizada en sangre periférica y no se modifica en aquellos casos en los cuales la presencia de procesos inflamatorios, infecciosos o neoplásicos, falsean los resultados de la ferritina sérica (Fs), que pueden mostrar valores altos no relacionados con la cantidad de hierro de depósito. Se concluye que a pesar de su especificidad y sus ventajas diagnósticas, el valor de la FER tiene una aplicación limitada, dad en parte por la preparación de la muestra y la complejidad del método usado para medirla (AU)

Ferritina eritrocitaria: medición y aplicación diagnóstica / Red cell ferritin: measurement and diagnostic applications

El objeto de este estudio fue determinar la ferritina eritrocitaria (FER) por enzimoinmunoensayo como una medida directa de los depósitos de hierro intracelular presente en los eritroblastos de la médula ósea. Su uso está indicado en diferencias o en sobrecargas de hierro. Se estudiaron 40 pacientes divididos en tres grupos: 1) un grupo control (n = 16) donde se obtuvieron los siguientes valores de FER: (X ñ ES) = 24 ñ 2,5 ag/cel; 2) un grupo de pacientes ferropénicos (n = 13) con resultados de FER: (X ñ ES) = 5 ñ 0,31 ag/cel y 3) pacientes ß-talasémicos: (n = 11) donde la FER fue de (X ñ ES) = 118 ñ 11,6 ag/cel. Los resultados obtenidos mediante el test de análisis de la varianza de una vía (ANOVA) mostraron una diferencia estadísticamente significativa en los dos grupos estudiados, con respecto al grupo control. La medida de FER presenta la ventaja de ser una determinación cuantitativa que puede ser realizada en sangre periférica y no se modifica en aquellos casos en los cuales la presencia de procesos inflamatorios, infecciosos o neoplásicos, falsean los resultados de la ferritina sérica (Fs), que pueden mostrar valores altos no relacionados con la cantidad de hierro de depósito. Se concluye que a pesar de su especificidad y sus ventajas diagnósticas, el valor de la FER tiene una aplicación limitada, dad en parte por la preparación de la muestra y la complejidad del método usado para medirla

[Vitamin E as protective agent against hemolysis in leprosy patients under dapsone treatment]. / Vitamina E como agente protector de hemólisis en pacientes de lepra tratados con dapsona.

Dapsone (4,4'diaminodiphenyl-sulphone) commonly used in the treatment of patients who suffer from leprosy, is a strongly oxidative drug, producing damage to the red cell membrane. This study investigated whether Vitamin E would have a protective effect on the red cell membrane from oxidant damage caused by Dapsone in patients with leprosy. We have studied 16 patients for 4 months, divided into two groups. Group 1 (n = 7) dapsone (DDS): 100 mg/day; Group 2 (n = 9) dapsone: 100 mg/day in addition with Vitamin E: 800 U/day. We did not include patients with low levels of Glucose-6-Phosphate Dehydrogenase (G-6-PD) because of their sensibility to this drug. At the beginning of the treatment we determined the level of G-6-PD. All patients showed a normocytic normochromic anemia with a decrease in Haptoglobine levels (below 5 mg/dl). Statistical analyses showed that reticulocyte counts did not present significant differences between groups all through evolution. As for methemoglobin (Hi) we observed in Group 1 an increase between the first and the fourth month, which was not seen in group 2. Statistical analyses of the results suggest that oral Vitamin E confers partial protective effect and does not correct the hemolysis parameters produced by Dapsone treatment except for Hi levels which were more sensitive to the oxidant damage.