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Search Results: 1 - 10 of 253 matches for " Porfirio; Svarch Guerchicoff "
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Frecuencia de la leucemia promielocítica en Cuba Frequency of promyelocytic leukemia in Cuba
María Teresa Milanés Roldán,Porfirio Hernández Ramírez,Eva Svarch Guerchicoff,Gisela Martínez Antu?a
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2001,
Abstract: Se realizó el análisis de 1 112 pacientes con LA (leucemias agudas) de todo el país (714 adultos y 398 ni os menores de 15 a os de edad), 637 (554 adultos y 83 ni os) presentaron LMA (leucemia mieloide aguda), y se diagnosticaron 98 casos (72 adultos y 26 ni os) con LPM (leucemia promielocítica aguda). La LPM representó el 8,9 % de todos los casos de LA en adultos y el 6,5 % de todos los casos de LA en ni os. Su frecuencia en relación con el total de casos de LMA sin límite de edad representó el 15,3 %. Sin embargo, cuando esta frecuencia se analizó por grupos de edades se apreció que en los ni os era de 31,3 % mientras que en los adultos fue solo del 13 %. En nuestro estudio se pudo comprobar que de forma similar a lo que ocurre en ciertas regiones geográficas y grupos poblacionales, existe un incremento de la frecuencia de LPM en ni os en relación con los datos de la literatura previa. Nuestros resultados destacan la necesidad de considerar por separado este tipo de leucemia en ni os y adultos, pues su análisis en un solo grupo podría ocultar variaciones en su frecuencia 1 112 patients with AL (acute leukemias) from all over the country (714 adults and 398 children under 15) were analyzed. 637 (554 adults and 83 children) had AML (acute myeloid leukemia) and 98 cases (72 adults and 26 children) were diagnosed APL (acute promyelocytic leukemia). The APL accounted for 8.9 % of all the cases of AL in adults and 6.5 % of all the cases of AL in children. Its frequency in relation to the total of cases of AML with no age limit represented 15.3 %. However, when this frequency was analyzed by age group, it was observed that it was 31.3 % in children, whereas in adults it was just 13 %. In our study, it was possible to prove that similarly to what happens in certain geographic regions and population groups, there is an increase of the frequency of APL in children compared with the data found in the previous literature. Our results stress the need to consider by separate this type of leukemia in children and adults, since its analysis in only one group may occult variations in its frequency
Frecuencia de la leucemia promielocítica en Cuba
Milanés Roldán,María Teresa; Hernández Ramírez,Porfirio; Svarch Guerchicoff,Eva; Martínez Antu?a,Gisela; Ballester Santovenia,José M;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2001,
Abstract: 1 112 patients with al (acute leukemias) from all over the country (714 adults and 398 children under 15) were analyzed. 637 (554 adults and 83 children) had aml (acute myeloid leukemia) and 98 cases (72 adults and 26 children) were diagnosed apl (acute promyelocytic leukemia). the apl accounted for 8.9 % of all the cases of al in adults and 6.5 % of all the cases of al in children. its frequency in relation to the total of cases of aml with no age limit represented 15.3 %. however, when this frequency was analyzed by age group, it was observed that it was 31.3 % in children, whereas in adults it was just 13 %. in our study, it was possible to prove that similarly to what happens in certain geographic regions and population groups, there is an increase of the frequency of apl in children compared with the data found in the previous literature. our results stress the need to consider by separate this type of leukemia in children and adults, since its analysis in only one group may occult variations in its frequency
La drepanocitosis en Cuba La drepanocitosis en Cuba
Eva Svarch,Porfirio Hernández Ramírez,José M Ballester Santovenia
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2004,
Abstract:
El accidente vascular encefálico en ni?os con drepanocitosis
Svarch,Eva;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: stroke is one of the more severe complications of drepanocytemia. it appears mainly in the first decade of life, with a trend to recurrence, and with cognitive and neurologic permanents. its optimal prevention and treatment are not accurately established, but nowadays, the more used strategies with better results are the red-blood cell transfusions and the hydroxyurea. transcranial doppler ultrasound (tcus) is an usefulness and safe method to predict stroke in many patients.
Fisiopatología de la drepanocitosis
Svarch,Eva;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: hemoglobin s (hb) is the consequence of a point mutation in of b globin chain 6 position resulting in glutamic acid substitution by valine. hb s polymerizes in conditions of oxygen low tension deforming the erythrocyte, the latter with the hb polymerized, has a shorten survival occluding the microcirculation, causing a chronic hemolytic anemia, and painful vasoconstrictive crises noting down to large extent the clinical picture of disease. vascular occlusion is a complex process due mainly to transit time extension of erythrocytes through microcirculation and/or decrease of delayed time of polymerization of hbs. nowadays, occlusion in the microcirculation is considered as an injury way of reperfusion in which oxidative stress of coagulation participates in the process. hemolytic anemia contributes to vascular occlusion because of the reticulocytes, with more adhesive molecules in its surface than the mature ones, to determines its greater adhesion to endothelium, and because of the plasma free hb to consume nitric oxide (no). no acts in different ways, where the most important is the deviation of normal balance between vasodilatation and vasoconstriction. as consequence from these facts we describe a sub-phenotype characterized by pulmonary hypertension, stroke, priapism, and malleolar ulcer. there are genetic factors influencing in clinical picture severity, e.g. 158 c-t mutation in globin gen g, and a-thalassemia. the first one increase the hbf, and the second one decrease the chcm, the number of thick cells, and the hemolysis identity. however, relation between a-thalassemia and severity of disease is not clear.
Programa cubano de atención integral al paciente con drepanocitosis Cuban program of comprehensive medical care to patients with sickle cell disease
Eva Svarch
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2011,
Abstract:
Fisiopatología de la drepanocitosis Physiopathologic features of drepanocytemia
Eva Svarch
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: La hemoglobina (Hb) S es la consecuencia de una mutación puntual en la posición 6 de la cadena de la globina que resulta en la sustitución del ácido glutámico por la valina. La HbS polimeriza en condiciones de baja tensión de oxígeno y deforma al hematíe. El hematíe con Hb polimerizada tiene una sobrevida acortada y ocluye la microcirculación, lo que da lugar a la anemia hemolítica crónica y a las crisis vasooclusivas dolorosas que marcan en gran medida el cuadro clínico de la enfermedad. La oclusión vascular es un proceso complejo debido fundamentalmente a la prolongación del tiempo de tránsito de los hematíes a través de la microcirculación y/o a la disminución del tiempo de demora de polimerización de la HbS. En la actualidad se considera a la oclusión en la microcirculación como una forma de injuria de reperfusión en la que el estrés oxidativo y la inflamación llevan al da o crónico de los órganos. Los leucocitos y factores de la coagulación participan en el proceso. La anemia hemolítica contribuye a la oclusión vascular porque el aumento de reticulocitos, con más moléculas de adhesión en su superficie que las células maduras, determina su mayor adhesión al endotelio y porque la Hb libre en plasma consume óxido nítrico. El óxido nítrico actúa en diferentes formas, una de las más importantes es que desvía el balance normal entre vasodilatación-vasoconstricción hacia la vasoconstricción. Como consecuencia de estos hechos se describe un subfenotipo caracterizado por hipertensión pulmonar, accidente vascular encefálico, priapismo y úlcera maleolar. Existen factores genéticos que influyen en la severidad del cuadro clínico como la mutación 158 C-T en el gen de la globina g y la a talasemia. La primera aumenta la HbF y la segunda disminuye la CHCM, el número de células densas y la intensidad de la hemólisis. Sin embargo, la relación entre a talasemia y la severidad de la enfermedad no es clara. Hemoglobin S (Hb) is the consequence of a point mutation in of b globin chain 6 position resulting in glutamic acid substitution by valine. HB S polymerizes in conditions of oxygen low tension deforming the erythrocyte, the latter with the HB polymerized, has a shorten survival occluding the microcirculation, causing a chronic hemolytic anemia, and painful vasoconstrictive crises noting down to large extent the clinical picture of disease. Vascular occlusion is a complex process due mainly to transit time extension of erythrocytes through microcirculation and/or decrease of delayed time of polymerization of HbS. Nowadays, occlusion in the microcirculation is consi
El accidente vascular encefálico en ni os con drepanocitosis Stroke present in children presenting drepanocytemia
Eva Svarch
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: El accidente vascular encefálico (AVE) es una de las complicaciones más graves de la drepanocitosis. Se presenta, sobre todo, en la primera década de la vida, tiene tendencia a recurrir y puede dejar secuelas neurológicas y cognoscitivas permanentes. Su prevención y tratamiento óptimos no se han establecido con precisión, pero en el momento actual, los que más se utilizan con buenos resultados son las transfusiones de glóbulos rojos y la hidroxiurea. El ultrasonido doppler transcraneal (UDTC) es un método útil y no invasivo para predecir el AVE en un número grande de pacientes. Stroke is one of the more severe complications of drepanocytemia. It appears mainly in the first decade of life, with a trend to recurrence, and with cognitive and neurologic permanents. Its optimal prevention and treatment are not accurately established, but nowadays, the more used strategies with better results are the red-blood cell transfusions and the hydroxyurea. Transcranial Doppler ultrasound (TCUS) is an usefulness and safe method to predict stroke in many patients.
Relevancia biológica y clínica del inmunofenotipaje celular en la leucemia linfoide aguda del ni?o
Marsán Suárez,Vianed; Cos Padrón,Yanelkys; Sánchez Segura,Miriam; Socarrás Ferrer,Bertha B.; Macías Abraham,Consuelo; del Valle Pérez,Lázaro O.; Nú?ez Quintana,Aramís; González Otero,Alejandro; Svarch Guerchicoff,Eva; Lam Díaz,Rosa M.;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2008,
Abstract: the biological and clinical characteristics of 238 children with acute lymphocytic leukemia (all) were studied for 13 years. the cellular immunophenotyping of samples from the bone marrow was performed by an immunocytochemical ultramicromethod. of the total of studied all, 81.4 % were phenotype b and 18.5 % phenotype t. 48.4 % of the children with b-all were 2-5 years old, whereas 65.9 % with t-all were 6 or over. no statistically significant differences were found when sex and colour of the skin were analyzed in relation to the cellular leukemic phenotype. on diagnosing the disease, 59.3 % of the patients with b-all showed figures of leukocytes in peripheral blood < 20x109/l, whereas in 61.4 % with t-all, the figures were higher than 50x109/l. it was observed a greater incidence of organomegaly, mediastinal adenopathies, hemorrhagic manifestations and initial infiltration of the central nervous system in patients with t-all compared with those suffering b-all. the differences were highly significant. these results proved that the leukemic phenotype in all in children could be considered as a positive or negative prognostic factor of the disease.
Heterogeneidad biológica y clínica de la leucemia linfoide aguda pediátrica de fenotipo T
Marsán Suárez,Vianed; Cos Padrón,Yanelkys; Fuentes de Armas,Lillian Teresa; Socarrás Ferrer,Bertha Beatriz; Sánchez Segura,Miriam; Nú?ez Quintana,Aramís; Svarch Guerchicoff,Eva; Lam Díaz,Rosa; del Valle Pére,Lázaro O; Macías Abraham,Consuelo;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2005,
Abstract: the biological and clinical characteristics of 31 children with phenotype t acute lymphoid leukemia (t-all) were studied in a period of 14 years. the cellular immunophenotyping was performed by the immunocytochemical (umiciq) and alkaline phosphatase anti-alkaline phosphatase (apaap) methods. it was observed a higher incidence (38.5 %) in the age group 2-5. the white male children were the most affected. 61.3 % of the patients showed leukocytes < 20x109/l at the onset of the disease. the mean figure of hemoglobin was 8.8x109/l. 71 % presented lymphadenopathies and splenomegalia. mediastinal mass, hepatomegalia and hemorrhages were found in 32.2 %, 83.9 % and 16.1 %, respectively. 25.8 % had infiltration of the central nervous system. the immunological classification revealed a predominance of the late t-all variety (45.2 %) over the early (29 %) and the cortical (25.8 %). 2 (6,4 %) mi+ t-all were diagnosed. these results proved that the t-all of the child is a clinical and biologically heterogeneous disease
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