Demographic Data about Glanzmann's Thrombasthenia and Bernard Soulier in Wassit , Iraq

Abstract

Objective: Thrombasthenia is rare inherited bleeding disorder .Clinical presentation differs and the aim of this  study is to determine the demographic Data for Glanzmann's thrombasthenia ,How it differs from Bernard soulier, the clinical presentation of patients with Glanzmann’s thrombasthenia (GT) and Bernard and to correlate their clinical presentation and laboratory findings.

Methods: A retrospective analysis of 32 patient having Bleeding disorder ( Thrombasthenia -un specified,Glanzmann’s and Bernard ) registered in The hematology center \ Al Karama hospital \ Al Kut city, Wassit state, Iraq. The data were collected from records from June 2003 to April 2016.The Demographic data of patient's age at time of diagnosis, sex, ABO blood group ,Chief complaint ,investigation values family history and the consequent state of patients were collected to be  analyzed .

Results:From total of 32 patients , 65.6% from them were males, 21 males and 11 females. In this study The patients were  from three provinces ( most patients were from Wassit 25, Dhi-qaar 4 and Missan 3.We found that from total of 32 patients, the number of patient above 5 years of age were 20 (62.2%), where the patients below 5 years were 12 (37.5%).A positive family history were found in 21 (65.6%), negative family history were found in 11 (34.4%).In this study, most of cases were Bernard soulier (43.8%) with 14 patients , while Glanzmann's (34.4%) in 11 patients, and 7 patients with thrombasthenia (21.9%). As for blood groups, most cases were unknown (37.5%) in 12 pts ,the second most common were A+ in 8 pts (25%) , B+ group and O+ were equal in 5 pts (15.6%) each, the least common were O- in 2 pts (6.2%).Most cases presented as bleeding in 24 pts (75%) , known case in 5 pts (15.6%) and pallor in 2 pts (6.2%), accidental during surgical operations was the least common with only 1 patient (3.1%).Most cases were positive consequent state -21 patients with 65.6% percentage, negative consequent were 9 pts (28.1%) and unknown in 2 pts (6.2%) .

Conclusion: Bleeding disorders such as Glanzmann’s and Bernard are not that rare in Iraq. There is also female:male ratio that is higher than other researches. Most of the patients were above the age of 5 ,while in other researches the diseases are most likely diagnosed in infancy.

Key word: bleeding, platelet function, thrombasthenia