Esplenectomía parcial en pacientes con drepanocitosis / Partial splenectomy in sickle cell disease

INTRODUCCIÓN: La esplenectomía total en la drepanocitosis se relaciona con riesgo de infecciones sobreagudas y con aumento de la incidencia de otros eventos, lo que no se ha comunicado en pacientes con esplenectomía parcial. En este estudio se caracterizó a los pacientes con drepanocitosis y esplenectomía parcial, y se comparó el comportamiento clínico y de laboratorio con los pacientes no esplenectomizados. MATERIAL Y MÉTODOS: Se estudió a 54 pacientes con drepanocitosis sometidos a esplenectomía parcial durante la edad pediátrica, desde 1986 hasta el año 2011, en el Instituto de Hematología e Inmunología. Se compararon con 54 pacientes no esplenectomizados seleccionados por muestreo aleatorio con características similares. RESULTADOS: La esplenectomía parcial se realizó a una edad media de 4,1 años, con una frecuencia mayor en la anemia drepanocítica (70,4%) y su causa más común fue la crisis de secuestro esplénico recurrente. Las complicaciones posoperatorias más frecuentes fueron: fiebre de origen desconocido (14,8%) y síndrome torácico agudo (11,1%). Después de la esplenectomía, aumentaron significativamente los leucocitos, neutrófilos y plaquetas; estos 2 últimos parámetros se mantuvieron elevados de manera significativa cuando se compararon con los pacientes no esplenectomizados. No hubo diferencias en la incidencia de los eventos clínicos, excepto el secuestro hepático, que fue más frecuente en los esplenectomizados. CONCLUSIÓN: La esplenectomía parcial constituyó un proceder seguro en los pacientes con drepanocitosis. No hubo diferencias en el cuadro clínico entre los niños esplenectomizados y los no esplenectomizados, salvo la mayor frecuencia de crisis de secuestro hepático en los primeros

INTRODUCTION: Total splenectomy in sickle cell disease is related to a high risk of fulminant sepsis and increased incidence of other events, which have not been reported in patients with partial splenectomy. In this study we examined the patients with sickle cell disease and partial splenectomy and compared the clinical and laboratory results with non-splenectomized patients. MATERIAL AND METHODS: We studied 54 patients with sickle cell disease who underwent partial splenectomy in childhood from 1986 until 2011 at the Institute of Hematology and Immunology. They were compared with 54 non-splenectomized patients selected by random sampling with similar characteristics. RESULTS: Partial splenectomy was performed at a mean age of 4.1 years, with a higher frequency in homozygous hemoglobin S (70.4%), and the most common cause was recurrent splenic sequestration crisis. The most common postoperative complications were fever of unknown origin (14.8%) and acute chest syndrome (11.1%). After splenectomy there was a significant increase in leukocytes, neutrophils, and platelets, the latter two parameters remained significantly elevated when compared with non-splenectomized patients. There was no difference in the incidence of clinical events, except hepatic sequestration, which was more common in splenectomized patients. CONCLUSION: Partial splenectomy was a safe procedure in patients with sickle cell disease. There were no differences in the clinical picture in children splenectomized and non-splenectomized except the greater frequency of hepatic sequestration crisis in the first group

[Partial splenectomy in sickle cell disease]. / Esplenectomía parcial en pacientes con drepanocitosis.

INTRODUCTION: Total splenectomy in sickle cell disease is related to a high risk of fulminant sepsis and increased incidence of other events, which have not been reported in patients with partial splenectomy. In this study we examined the patients with sickle cell disease and partial splenectomy and compared the clinical and laboratory results with non-splenectomized patients. MATERIAL AND METHODS: We studied 54 patients with sickle cell disease who underwent partial splenectomy in childhood from 1986 until 2011 at the Institute of Hematology and Immunology. They were compared with 54 non-splenectomized patients selected by random sampling with similar characteristics. RESULTS: Partial splenectomy was performed at a mean age of 4.1 years, with a higher frequency in homozygous hemoglobin S (70.4%), and the most common cause was recurrent splenic sequestration crisis. The most common postoperative complications were fever of unknown origin (14.8%) and acute chest syndrome (11.1%). After splenectomy there was a significant increase in leukocytes, neutrophils, and platelets, the latter two parameters remained significantly elevated when compared with non-splenectomized patients. There was no difference in the incidence of clinical events, except hepatic sequestration, which was more common in splenectomized patients. CONCLUSION: Partial splenectomy was a safe procedure in patients with sickle cell disease. There were no differences in the clinical picture in children splenectomized and non-splenectomized except the greater frequency of hepatic sequestration crisis in the first group.

Contaje absoluto de linfocitos como factor pronóstico en la leucemia aguda linfoblástica del niño / Absolute lymphocyte count as a prognostic factor in children with acute lymphoblastic leukemia

Introducción: Estudios recientes sugieren el contaje absoluto de linfocitos (CAL) como un nuevo indicador pronóstico en enfermedades malignas, de forma que aquellos pacientes que posean CAL superiores en determinados momentos del tratamiento, tendrán mayores posibilidades de supervivencia. En particular se ha visto la influencia de las células T y las células naturales asesinas en la inmunidad de pacientes con cáncer. Materiales y métodos: Se realizó un estudio retrospectivo en pacientes pediátricos con leucemia aguda linfoblástica tratados en el Instituto de Hematología e Inmunología entre los años1995 y 2008 (105 pacientes), con el objetivo de evaluar la influencia del conteo absoluto de linfocitos como factor pronóstico en la sobrevida libre de enfermedad (SLE) y la sobrevida global (SG) a los 5 años. Resultados: Evolucionaron desfavorablemente 24,8% y la mediana del CAL determinado los días 15 y 28 de tratamiento de estos pacientes fue de 1.000 células/ l. Los pacientes con CAL el día 15 (CAL-15) y 28 (CAL-28) <1.000 y ≥ 1.000/ l, mostraron una SLE de 51% vs 83% y 55%vs 82% (p = 0,02 y p = 0,04), respectivamente. Igualmente la SG para aquellos con CAL-15 yCAL-28 ≥1.000/ l fue 89% y 86% contra un 59% y 66% para valores <1.000 (p = 0,001 y p = 0,01),respectivamente. Al realizar el análisis multivariado junto a otros factores de riesgo como la edad, el estudio molecular, la respuesta al tratamiento y el contaje inicial de leucocitos, el CAL-15 mostró significación estadística tanto para la SLE (p = 0,006) como para la SG (p = 0,001). Conclusiones: El CAL fue un predictor significativo de supervivencia y recaída. Además tuvo un comportamiento independiente como factor pronóstico (AU)

Introduction: Recent studies have suggested that the absolute lymphocyte count (ALC) may bea prognostic indicator in malignant diseases, in that those patients who have higher ALC at certain times during treatment may have a better chance of survival. The influence of T cellsand natural killer cells in the immune system of the patient with cancer as a response to cancercells is particularly noted. Materials and Method: We prospectively assessed the prognostic value of absolute lymphocytic count (ALC) in 105 pediatric patients with acute lymphoblastic leukemia (ALL), treated in the Cuban Immunology and Hematology Institute from 1995 to 2008. ALC was studied at days 15 (ALC-15) and 28 (ALC-28) of treatment. Results: In our patients, 1000 cells/uL was the median ALC value for patients who relapsed ordied. Using 1000/uL we found that ALL patients with an ALC-15 <1000 cells/ l had a 5-year relapse free survival (RFS) of 51%. In contrast, an ALC-15 >1000 cells/uL showed an excellent prognosis, with a 5-year RFS of 83% (p=0.02). Similarly in our study, an ALC-28 <1000 cells/ lpredicted a 5-year overall survival (OS) of 66%, where as an ALC-28 >1000 cells/ l predicted excellent outcome, with a 5-year OS of 86% (p=0.01). Importantly, ALC is also a strong predictorin multivariate analysis with known prognostic factors. ALC is a simple, statistically powerful measurement for patients with de novo ALL. Conclusions: The results, when combined with previous studies, demonstrate that ALC is a powerful new prognostic factor for a range of malignancies (AU)

[Absolute lymphocyte count as a prognostic factor in children with acute lymphoblastic leukemia]. / Contaje absoluto de linfocitos como factor pronóstico en la leucemia aguda linfoblástica del niño.

INTRODUCTION: Recent studies have suggested that the absolute lymphocyte count (ALC) may be a prognostic indicator in malignant diseases, in that those patients who have higher ALC at certain times during treatment may have a better chance of survival. The influence of T cells and natural killer cells in the immune system of the patient with cancer as a response to cancer cells is particularly noted. MATERIALS AND METHOD: We prospectively assessed the prognostic value of absolute lymphocytic count (ALC) in 105 pediatric patients with acute lymphoblastic leukemia (ALL), treated in the Cuban Immunology and Hematology Institute from 1995 to 2008. ALC was studied at days 15 (ALC-15) and 28 (ALC-28) of treatment. RESULTS: In our patients, 1000 cells/uL was the median ALC value for patients who relapsed or died. Using 1000/uL we found that ALL patients with an ALC-15 <1000 cells/µl had a 5-year relapse free survival (RFS) of 51%. In contrast, an ALC-15 >1000 cells/uL showed an excellent prognosis, with a 5-year RFS of 83% (p=0.02). Similarly in our study, an ALC-28 <1000 cells/µl predicted a 5-year overall survival (OS) of 66%, whereas an ALC-28 >1000 cells/µl predicted excellent outcome, with a 5-year OS of 86% (p=0.01). Importantly, ALC is also a strong predictor in multivariate analysis with known prognostic factors. ALC is a simple, statistically powerful measurement for patients with de novo ALL. CONCLUSIONS: The results, when combined with previous studies, demonstrate that ALC is a powerful new prognostic factor for a range of malignancies.