Evaluación clínica y hematológica de pacientes con drepanocitosis esplenectomizados en el Instituto de Hematología e Inmunología de Cuba: 30 años de experiencia / Clinical and hematological evaluation of patients with splenectomized drepanocytosis at the Hematology and Immunology Institute of Cuba: 30 years of experience

Introducción: Desde 1986 se ha introducido en nuestro país la esplenectomía parcial, que logra prevenir la recurrencia de la crisis de secuestro esplénico y disminuir la incidencia de sepsis sobreaguda postesplenectomía. Objetivo: Comparar desde el punto de vista clínico y de laboratorio los pacientes con esplenectomía total y parcial. Pacientes y métodos: Se estudiaron todos los pacientes con drepanocitosis, seguidos en el Instituto de Hematología e Inmunología, que se hubieran sometido a esplenectomía durante la edad pediátrica. Resultados: Se incluyeron 39 pacientes en cada método de esplenectomía; la mayoría eran varones (60,3%) y predominaba la anemia drepanocítica (69,2%). La edad de aparición de la esplenectomía fue menor en la de tipo parcial (4,7 años; p= 0,009) que en la total (6,7 años), y el tiempo de seguimiento fue de 12,5 años. El grupo de esplenectomía total presentó un mayor aumento de hemoglobina, leucocitos y plaquetas (p= 0,039), así como valores elevados de lactato deshidrogenasa (p= 0,015), hemoglobina plasmática (p= 0,001) y velocidad de regurgitación tricuspídea (p= 0,038). La crisis vasooclusiva dolorosa fue más frecuente tras la esplenectomía total (75,8 ± 14,3 frente a 39,8 ± 10,1; p < 0,001), al igual que las úlceras maleolares (p= 0,04). La crisis hepática y la mortalidad fueron también más frecuentes en la esplenectomía total aunque sin significación estadística (p= 007 y p= 0,305, respectivamente). Conclusiones: La esplenectomía parcial presenta menos complicaciones a largo plazo que la total (AU)

Introduction: Since 1986 has been introduced in our country partial splenectomy, which prevent the recurrence of splenic sequestration crisis and possible reduce the number of overhelming septicemia. Objective: Compare clinical and laboratory aspects in patients with total and partial splenectomy. Patients and methods: All patients with sickle cell disease were studied, followed at the Instituto de Hematología e Inmunología, who have had a splenectomy in childhood. Results: 39 patients were included in each method of splenectomy, where a predominance of males (60.3%) and sickle cell anemia (69.2%). Splenectomy age was lower in the partial, 4.7 years than in total splenectomy, 6.7 years (0.009). Follow-up time was 12.5 years. Total splenectomy group had greater increase in hemoglobin, leucocytes and platelets (p= 0.039), elevated LDH levels (p= 0.015), plasma hemoglobin (p= 0.001) and tricuspid regurgitation velocity (p= 0.038). Vaso-occlusive painful crises was more frequent after total splenectomy (75.8 ± 14.3 vs. 39.8 ± 10.1; p <0.001), as leg ulcer (p= 0.04). Hepatic crisis (p <0.07) and mortality were higher in individuals with complete splenectomy (p= 0.305) but withouth significative statistics. Conclusions: Partial splenectomy has fewer long-term complications that total (AU)

[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.

Perforating ocular wounds in occupational accidents.

We review 77 patients with perforating ocular wounds due to occupational accidents seen in our hospital over a period of 5 years. Industrial accidents were the most frequent cause (61.04%), followed by agricultural (25.97%) and services trade (12.98%). Seventy-five patients were males (97.40%), with a mean age of 32.88 years. The cornea was statistically the most frequent location (p less than 0.001). The nasal quadrants were more frequently affected than the temporal ones (p less than 0.001) and the upper quadrants were also more affected than the lower ones, especially in the cornea (p less than 0.001). Fifty-two percent had intraocular foreign bodies; industrial accidents were responsible of 65% of these injuries. They were significantly more frequent in summer than in autumn (p less than 0.001) and spring (p less than 0.01). We discuss the prognosis of these injuries and the circumstances that may influence in their occurrence, stressing the importance of prevention programs.

Orbital phycomycosis.

3 cases with rhinoorbital phycomycosis and a 4th case, in which it was considered retrospectively, are presented in hematological patients. 2 of the 4 patients survived. In the first 3 cases, which appeared in a period of 2 months in the Hematology Unit, an epidemiological study was made, showing that the cause of the infections was contamination of the air-conditioning systems of this unit by numerous Phycomycetes and Mucor pusillus.

Intraocular hemorrhage in sudden increased intracranial pressure (Terson syndrome).

We examined 19 cases with SAH, 4 of which presented intraocular hemorrhages (retinal, subhyaloid and in vitreous). The mortality rate was 50% when the intraocular hemorrhages were present compared to 20% when they were absent.