A study of some physiological changes in patients with inherited thrombosthenia

Abstract EN

Aim : this study was designated to investigate the changes of some hematological and biochemical parameters in patients affected with hereditary thrombosthenia. Method : the study included 30 patients (males and females) with hereditary thrombosthenia who admitted to the hereditary bleeding disorder center at Babylon maternity and pediatrics teaching hospital in Hilla city and at the hereditary bleeding disorder center in AL-Zahraa maternity and pediatrics teaching hospital in AL-Najaf city.

Their age was ranged 3- ≤ 18 years (mean age 11 years ± SE 5 years).

The patients were classified into 2 groups according to the age which are group I (3- ≤ 10 years) and group II (10- ≤ 18 years) and each age group was subdivided into males and females subgroups.

Thirty apparently healthy subjects (15 males and 15 females) of matched age and gender were also included in this study. Results : concerning the hematological parameters, it was found that hemoglobin (Hb), packed corpuscular volume(PCV), means corpuscular volume (MCV), means corpuscular hemoglobin (MCH), and means corpuscular hemoglobin concentration (MCHC) show significant decrease in most groups of hereditary thrombosthenia in comparison with the control groups.

Regarding the biochemical parameters, it was found that serum iron and serum ferritin are significantly decreased and serum total iron binding capacity (TIBC) are significantly increased in most groups of hereditary thrombosthenia compared to the control groups. Conclusion : in view of the changes summarized, the increase or decrease in some hematological and biochemical parameters may be attributed to recurrent blood loss which is the most important manifestation of the diseases.