Hemotological and Coagulatory Alteration in Splenectomized Versus Non-Splenectomized Transfusion Dependant Β-Thalassemic Major Patients

AL-Shamari, Haider; M. Majeed Al Sharifi, Liqaa; A. Hussain, Farah

	Hemotological and Coagulatory Alteration in Splenectomized Versus Non-Splenectomized Transfusion Dependant Β-Thalassemic Major Patients
Medical Journal of Babylon
Article 1, Volume 12, Issue 2, 2015, Pages 551-560
Authors
Haider AL-Shamari; Liqaa M. Majeed Al sharifi; Farah A. Hussain
Abstract
β-Thalassemia is an inherited hemolytic disorder caused by a partial or complete deficiency of β- globin chain synthesis. Profound hemostatic changes have been observed in patients with β-thalassemia major with history of thromboembolic or haemorrahgic manifestation. The aim of this study was to assess the coagulation parameters; PT, PTT, factor V, factor VIII level in β-thalassemia major and to compare the effect of splenectomy on each and to evaluate the patients for increased risk of thrombosis and/ or bleeding. Sixty patients with transfusion dependent β-thalassemia major ;thirty of them splenectomized (16 females and 14 males), and thirty non-splenectomized (13 females and 17 males) aged between (3-38) years at different stages of the disease were included in this study from Babil hospital for maternity and pediatrics from the first of April 2012 to the 15th of July 2012. Seven milliliters of venous blood were collected from each patients and control persons included in this study by clear venipuncture under aseptic conditions from antecubital fossa with easy withdrawal of blood , subdivided as follows: 11.8ml in o.2 ml trisodium citrate with gentle mixing for coagulation testing include PT,PTT, factors V and VIII. 22ml in EDTA tube for routine haematological screening tests include: PCV,WBC, platelet count. Normal pooled plasma for factor assay obtained from 20 normal healthy persons ,plasma was mixed and divided into aliquot each containing about 1ml stored at -40C0 for 3-4 weeks as a control for factor assay study. The mean age was 18.2 years in splenectomized patients and 8.9years in non splenectomized patients. The PCV level was low in both groups 26.8,24.6 in splenectomized and non splenectomized patients respectively with statistical significant difference between them (p value <0.05). WBC and platelets count were both within normal limit ,however there was a statistical difference in platelets count between splenectomized and non splenectomized groups with mean value were 259.6 and 218.7 x109 /L respectively, p value <0.05. WBC count mean were 8.2 and 7.0x109 /L in splenectomized and non splenectomized patients respectively with no statistical significant difference between them p value >0.05. These patients (splenectomized and non splenectomized) have normal mean PT,PTT, which were 14.4 sec, 33 sec for splenectomized and 14.1 sec,33.2 sec for non splenectomized patients respectively with no statistical significant difference between them p value >0.05. Factors V,VIII level were 103%,127.8% for splenectomized patients and 100.3, 128.9% for non splenectomized patients respectively with no statistical significant difference between them p value>0.05 ,but there was statistical significant difference between the control group and the total thalassemic patients (P value <0.05),however splenectomized patients had more episodes of bleeding and thrombotic manifestations than non splenectomized patients which might be due to causes unrelated to coagulation factors V and VIII level. Splenectomy could affect PCV but had no effect on coagulation factors V and VIII.-Bleeding manifestations were more frequent in splenectomized patients with normal factor V,VIII and platelets count.-Thalassemics in Babylon thalassemic center were under transfused.
Keywords
beta thalassemia major; PTT; factor V; factor VIII; Splenectomy

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