Principales modificaciones asociadas a la esplenectomía / Main modifications associated with splenectomy

Introducción. El bazo es un órgano linfoide implicado en el reconocimiento antigénico, la depuración de patógenos y la remoción de eritrocitos envejecidos o con inclusiones citoplasmáticas. La esplenectomía es una técnica utilizada tanto para el diagnóstico (linfomas), el tratamiento (trombocitopenia inmune, anemia hemolítica adquirida) y la curación (microesferocitosis hereditaria) de diversas enfermedades. Métodos. Describir los principales cambios hematológicos y complicaciones asociadas al procedimiento de esplenectomía. Discusión. Los cambios posteriores a la esplenectomía pueden ser inmediatos, como la aparición de cuerpos de Howell-Jolly, la trombocitosis y la presencia de leucocitosis durante las primeras dos semanas. Otras complicaciones tempranas incluyen la presencia de trombosis, en especial en pacientes con factores de riesgo secundarios (edad, sedentarismo, manejo hospitalario, obesidad) o un estado hipercoagulable (diabetes, cáncer, trombofilia primaria), siendo tanto el flujo de la vena porta como el volumen esplénico los principales factores de riesgo para su aparición. Las complicaciones tardías incluyen la alteración en la respuesta inmune, aumentando el riesgo de infecciones por bacterias encapsuladas, en conjunto con una reducción en los niveles de IgM secundario a la ausencia de linfocitos B a nivel de bazo. Debido al riesgo de infecciones, principalmente por Streptococcus pneumoniae, la esplenectomía parcial se ha considerado una opción. Conclusión. Una adecuada valoración de la indicación de esplenectomía y la identificación precoz de complicaciones posoperatorias son fundamentales para reducir la mortalidad asociada a la esplenectomía

Introduction. The spleen is a lymphoid organ involved in antigen recognition, pathogen clearance, and removal of aged erythrocytes or those with cytoplasmic inclusions. Splenectomy is a technique used for diagnosis (lymphomas), treatment (immune thrombocytopenia, acquired hemolytic anemia), and cure (hereditary microspherocytosis) of various diseases. Methods. To describe the main hematological changes and complications associated with the splenectomy procedure. Discussion. Changes after splenectomy can be considered immediate: the appearance of Howell-Jolly bodies, thrombocytosis, and leukocytosis during the first two weeks. Other complications include the presence of thrombosis, especially in patients with risk factors (age, sedentary lifestyle, long hospital stay, obesity) or a hypercoagulable state (diabetes, cancer, primary thrombophilia), with both portal vein flow and splenic volume being the main risk factors for its appearance. Late complications include altered immune response, increased risk of infections by encapsulated bacteria, and a reduction in IgM levels secondary to the absence of B lymphocytes in the spleen; due to the risk of diseases mainly by Streptococcus pneumoniae, partial splenectomy has been considered an option. Conclusion. An adequate assessment of the indication for splenectomy and the early identification of complications are essential to reduce the mortality associated with splenectomy

Evaluation of Thalassaemia Screening Tests in the Antenatal and Non-Antenatal Populations in Singapore

INTRODUCTION@#Haemoglobinopathy testing is performed for carrier screening and evaluation of microcytic anaemia. We evaluated the effectiveness of thalassaemia screening tests at our institution and suggest ways of improving the testing algorithm.@*MATERIALS AND METHODS@#A total of 10,084 non-antenatal and 11,364 antenatal samples with alkaline gel electrophoresis (AGE), capillary electrophoresis (CE), haemoglobin H (HbH) inclusion test, mean corpuscular haemoglobin (MCH) and mean corpuscular volume (MCV) were retrospectively reviewed. A subgroup of 187 samples with genetic testing was correlated with HbH inclusions and MCH/ MCV. The effect of iron deficiency on percentage hemoglobin A2 (HbA2) was studied.@*RESULTS@#HbH inclusion test showed low sensitivity of 21.43% for α-thalassaemia mutations but higher sensitivity of 78.95% for deletion. By receiver operating characteristic (ROC) analysis, MCH ≤28 pg or MCV ≤80 fl for non-antenatal samples and MCH ≤27 pg or MCV ≤81 fl for antenatal samples had >98% sensitivity for HbH inclusions. Above these thresholds, the probability that HbH inclusions would be absent was 99%). MCH ≥28 pg had 100% sensitivity (95% CI 95.63%-100%) for α-thalassaemia mutations and 97.68% calculated NPV in the antenatal population. Detection of haemoglobin variants by CE correlated highly with AGE (99.89% sensitivity, 100% specificity). Severe iron deficiency reduced HbA2 in hemoglobin ( <0.001) and α-thalassaemia ( = 0.0035), but not in β-thalassaemia.@*CONCLUSION@#MCH/MCV thresholds have adequate sensitivity for α-thalassaemia in the antenatal population, and genotyping plays an important role as HbH inclusion test shows low sensitivity. CE without AGE, may be used as initial screening for haemoglobin variants. Our study provides contemporary data to guide thalassaemia screening algorithms in Singapore.

Preliminary uterine artery ligation versus pericervical mechanical tourniquent during abdominal myomectomy

Interventions that reduce blood loss during myomectomy are many. The effectiveness and the adverse effects of each are still under investigation. To compare preliminary uterine artery ligation versus pericervical mechanical tourniquet during abdominal myomectomy. Randomized prospective study. Two hundreds and six patients with symptomatic myomas in premenopausal and/or childbearing period attending the gynecology department, Mansoura University Hospital. Clinical examination, urine examination, blood count, liver function tests, kidney function tests and plasma glucose were carried out. Abdominal and transvaginal ultrasound were performed. The studied cases were randomly allocated into two groups; group I; 104 patients underwent preliminary uterine artery ligation and group II; 102 women underwent pericervical mechanical tourniquet during abdominal myomectomy. The operative time and operative blood loss were significantly lower in the uterine artery ligation group [P < 0.001]. Post operative reduction of haemoglobin concentrations and haematocrite values were significantly lower in uterine artery ligation group [P 0.015 for each]. Operative complications [conversion to hysterectomy, urinary tract injury, gastrointestinal were significantly lower in the uterine artery ligation group versus the touriquet [p < 0.001]. Post operative beven the need for blood transfuion and post operative analgesia were significantly highen in touriquet group [p < 0.001]

Diphosphoglycerate and phenotypic severity of sickle cell disease

Sickle cell disease is a hereditary blood disorder characterized by chronic manifestations of progressive organ failure. To control a disease of this type, it is important to study the factors which affect the course of the disease. Diphosphoglycerate [2, 3 DPG] is the most common metabolite generated by the glycolysis. It is a potent modifier for hemoglobin function. To clarify the role of 2, 3 DPG in sickling, its concentration was measured in 15 normal healthy individuals and in 12 sickle cell patients at steady state and during crisis. Hematological indices and partial oxygen tension [PO[2]] were also determined for sicklers and controls. Sickle cell patients during crisis were found to have a significantly higher levels of 2, 3 DPG [9.17 +/- 5.36 mmol/L] [p<0.05], and significantly low PO[2] [8.41 +/- 1.82 Kpa, p< 0.01] than controls [8.54 +/- 0.72 mmol/L for 2, 3 DPG and 12.44 +/- 0.7 Kpa for PO[4]]. The increase in 2, 3 DPG concentration during crisis may be due to its increased production via glycolysis, which in turn increased as a result of decreased oxygen tension during crisis. The increased 2, 3 DPG concentration increases and stabilizes the deoxygenated HbS leading to its precipitation, sickling, and hemolysis of red blood cells. In conclusion, the factors which can increase 2, 3 DPG or decrease PO[4] may lead to the sickling crisis and increase the phenotypic severity of the disease

Esquistossomose hepato-esplênica em crianças: corpúsculo de Howell-Jolly após esplenectomia e esplenose / Hepatosplenic schistosomiais mansoni in children: Howell-Jolly bodies after splenectomy and splenosis

A esquistossomose mansônica hepato-esplênica com varizes esofágicas é infrequente em crianças, entretanto, determina morbidade atingindo a produtividade desses futuros adultos. Há evidências de que a esplenose pós-esplenectomia, por trauma, mantém, de forma parcial, as funçSes imunológica e hemocaterética esplênicas. Estudos semelhantes, entretanto, nÝo foram realizados em esquistossomóticos submetidos a esplenectomia. Foram analisados, para avaliaçÝo da funçÝo de esplenose, 15 pacientes, de 7 a 18 anos, com esquistossomose hepato-esplênica submetidos a esplenectomia, ligadura de veia gástrica esquerda e auto-implante esplênico (Grupo A). A esplenose foi comprovada por cintilografia ou por Dopplerfluxometria. Também foram analisados cinco pacientes similares antes da cirurgia (grupo B) e três crianças, nÝo esquistossomóticas submetidas a esplenectomia sem auto-implante esplênico (grupo C). Os esfregaços de sangue periférico foram examinados para pesquisa de corpúsculos de Howell-Jolly, que indicam ausência ou insuficiência de funçÝo hemocaterética esplênica. Dos pacientes do grupo A, um (7por cento) apresentou corpúsculosde Howell-Jolly. Os demais nÝo apresentaram estes corpúsculos (93por cento). Nenhum paciente do grupo B os apresentou, enquanto que todos os esfregaços dos pacientes do Grupo C apresentaram estes corpúsculos. Os dados confirmam o auto-implante esplênico como método edicaz na manutençÝo da funçÝo hemocaterética esplênica

Hypofunción esplénica espontánea en lupus eritematoso sistémico y sindrome de Sjögren primario / Spontaneous splenic hypofunction in systemic lupus erythematosus and primary Sjogren syndrome

El objetivo del presente trabajo fue evaluar la existencia de hipoesplenismo funcional en enfermedades autoinmunes. La hipofunción esplénica se determinó por la presencia de cuerpos de Howell-Jolly en eritrocitos de la sangre y posterior centellografía esplénica con radiocoloides marcados con 99Tc, en pacientes en los que se habían descartado otras posibles causas de cuerpo de Howell-Jolly. Se estudiaron extendidos de sangre periférica de 174 pacientes con enfermedades autoinmunes y de 32 otras enfermedades de patogenia inmunológica y 63 indivíduos normales. Se encontró evidencia de hipoesplenia funcional en 4 de 79 pacientes con Lupus Eritematoso Sistémico (LES) y en 2 de 18 casos de Síndrome de Sjorgren Primario (SSP). En uno de los pacientes de LES con hipoesplenia funcional y leve esplenomegalia, se demostró ausencia de cuerpos Howell-Jolly en un estudio realizado 11 meses después de la primera evaluación. En el grupo control, un paciente con leucemia linfática crónica (LLC) y esplenomegalia tenía evidencia de hipoesplenismo. Los resultados obtenidos revelan: 1) Las enfermedades autoinmunes que con mayor frecuencia presentan hipoesplenia funcional son el LES y SSP; 2) La hipoesplenia funcional en enfermedades autoinmunes puede ser transitoria. 3) Esplenomegalia e imagen centelleográfica aumentada del bazo no descartan hipoesplenia

Cuerpos de Howell-Jolly en sangre periférica de falcémicos / Howell-Jolly bodies in peripheral blood of pacients with sickle cell disease

Se investiga la presencia de cuerpos de Howell Jolli como expresión de hipoesplenismo en niños falcémicos sin sombra esplénica radiográfica, encontrándose una correlación de un 100%