بررسي ارتباط بين شدت علايم باليني و داده هاي آزمايشگاهي در بيماران سيكل سل

A Study of Correlation between Clinical Severity with Laboratory Data in Sickle Cell Disease

Objectives: Sickle cell disease is an inherited red cell disorder characterized by diverse clinical courses. Some individual patients follow a more severe course that begins shortly after birth, whereas others are infrequently ill. The purpose of this work was to study the correlation between clinical severity with laboratory data in sickle cell disease patients in the hope to find data that can be used to predict the severity of clinical episode of sickle cell disease and therefore preclude the application of unnecessary interventions which arc only needed for severe episodes. Methods: In this study, during one year period, 78 patients suffering from sickle cell disease (48 HbSS, 21 SC, 9 33 thalassaemia) who attended the Haematology Clinic at the Department of Clinical Haematology of Manchester Royal Infirmary in England were studied. Red cell indices and haemoglobin analysis were examined when the patients were in their steady state. Red cell indices were determined using Syismax NE 8000, and haemoglobin variants (HbA2, HbC, llbS, HbA and HbF) were identified by fully automated Bio Rad HPLC, The patients were categorized as severe mild and moderate according to their clinical states. Correlation between clinical severity and laboratory data was assessed using multivariate statistical methods. Results: The results, as expected, showed a significant correlation, at p <0.05, in the whole group of phenotype with the total Hb, HbS, HbC. There was a positive correlation (r 0.32, p < 0.05) between clinical severity and HbS level in all patient groups and negative correlation (r = - 0.41, p < 0.05) between clinical severity and HbF level in the SS but not in the SC or S-J3thal patients. Conclusions: The high level of HbF, found in all patient groups, therefore could not be the underlying reason for the amelioration of the clinical severity and there must be other factors such as genetic, nutrition and immunity that might affect the clinical status of sickle cell patients.