سيكل سل ايران و دستورالعمل اجرايي برنامه پيشگيري از موارد جديد بتاتالاسمي ماژور

IRAN SICKLE CELL AND THALASSEMIA SCREENING PROGRAMME: A PILOT STUDY ON 50 SICKLE CELL CASES

Reviewing methods used in the present thalassemia-screening programme in Khozestan province seems to be necessary. This screening is primarily depends on MCV, MCH and A2 measurements obtained from the routine CBC blood tests, which we suspect to be neither specific nor selective for detection of thalassemia traits. Only when these levels are below the standard value haemoglobin electrophoresis is performed. In order to test this hypothesis, we carried out a pilot, prospective and randomized study of one-year duration (Sept 1999 to Sept 2000) on the 50 cases of sickle cell case among the referred patients both to Shafa hospital, Ahwaz, and in our private practice. Data from first screening line showed MCV>80, MCH>27 in more than 94% and A2 value of less than 3% in 100% of cases. These normal results showed present public health thalassemia-screening programme was not sensitive enough to detect the obscured sickle cell trait, and confirm our suspicions. This results in issuing of formality pass-card of marriage of young couples. We therefore recommended an obligatory haemoglobin electrophoresis test to be added to the routine first line tests in the public health thalassemia-screening programme in Khozestan province, especially in the South-Western region.