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PREVALENCE AND CAUSES OF CLOTTING TIMES PROLONGATION IN PATIENTS WITH TRANSFUSION DEPENDENT BETA THALASSEMIA
Author(s):
Immacolata Tartaglione
,
Immacolata Tartaglione
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
martina caiazza
,
martina caiazza
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
anna bonadies
,
anna bonadies
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
domenico roberti
,
domenico roberti
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
maddalena casale
,
maddalena casale
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
saverio scianguetta
,
saverio scianguetta
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
francesca rossi
,
francesca rossi
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
silverio perrotta
silverio perrotta
Affiliations:
Dipartimento della Donna, del Bambino e della Chirurgia generale e specialistica,Università della Campania 'Luigi Vanvitelli',Naples,Italy
(Abstract release date: 05/18/17) EHA Library. Tartaglione I. 05/18/17; 182914; PB2201
Dr. Immacolata Tartaglione
Dr. Immacolata Tartaglione
Contributions
PDF Abstract
Abstract

Abstract: PB2201

Type: Publication Only

Background

Thalassemia is traditionally known to be a thrombophilic, rather than hemorrhagic, disorder. In spite of this, prolongation of clotting times are often reported. Understanding if there is a real risk of bleeding, and what this risk can be associated to, is crucial, especially in relation to the frequent referral to surgery (e.g. for splenectomy, cholecistecomy). Hepatopathy due to iron overload or HCV infection has been addressed as a main cause of this finding, even though disorders in the clotting profile are often reported also in patients with no alterations of hepatic function. The impairment of factors XI and XII often reported has been hypothesized to be secondary to intravascular haemolysis or multiple transfusions ( Caocci et al, Acta Haematol 1978, Mcfadyen et al, Ann Hematol 2014), but no data are available to confirm this supposition.

Aims

To determine the prevalence of clotting disorders in a group of Transfusion dependent Thalassemia (TDT) patients and to assess the correlation with hepatopathy, degree of the hemolysis, transfusion frequency, erythroblastosis, iron chelation.

Methods

TDT patients followed at our center for whom clotting tests were available were included. From chart review data were collected regarding clotting times, demographics, disease history, comorbidities and concomitant medications, iron chelation therapies, iron overload ( serum ferritin, LIC, cardiac T2*), liver function tests, hemolysis parameters, hemocromocitometric values. Patients on anticoagulation therapy were excluded.

Results
56 TDT patients (female 55,35%) were enrolled in our study, mean age 26,02±13,38 years, 17 of them were pediatric. In 20/56 patients (35,71%) prolongation of clotting time was found: this included both prolonged INR ( 23,21%) and prolonged aPTT ratio (25%); 7 patients (12,5%) had both prolonged INR and aPTT. Subgroup with clotting disorder (group A) was compared to subgroup with clotting times within normal ranges (group B) using T-Test. No differences were found in terms of sex, age, genotype, transfusion interval, hemolysis degree, comorbidities, HCV infection included, iron overload, liver function, erythroblastosis and platelets levels, nor in history of thrombotic complications. No patients had history of hemorrhagic disease. Pretransfusion Hb was lower in patients with prolonged clotting times (p=0,045); none of the patients in Group A was splenectomized ( p=0,042).

Conclusion

In our population clotting disorders were not correlated with hepatic disease, nor hemolysis or transfusions. The mild correlation with lower Hb values and with the lacking splenectomy could be consistent with the known effect of low Ht on lab procedures for clotting tests. In relation to this observation in patients with altered coagulation tests the repetition of clotting test after blood transfusion could be advisable to overcome the low Hb effect.

Session topic: 26. Thalassemias

Keyword(s): Thalassemia, Coagulation

Abstract: PB2201

Type: Publication Only

Background

Thalassemia is traditionally known to be a thrombophilic, rather than hemorrhagic, disorder. In spite of this, prolongation of clotting times are often reported. Understanding if there is a real risk of bleeding, and what this risk can be associated to, is crucial, especially in relation to the frequent referral to surgery (e.g. for splenectomy, cholecistecomy). Hepatopathy due to iron overload or HCV infection has been addressed as a main cause of this finding, even though disorders in the clotting profile are often reported also in patients with no alterations of hepatic function. The impairment of factors XI and XII often reported has been hypothesized to be secondary to intravascular haemolysis or multiple transfusions ( Caocci et al, Acta Haematol 1978, Mcfadyen et al, Ann Hematol 2014), but no data are available to confirm this supposition.

Aims

To determine the prevalence of clotting disorders in a group of Transfusion dependent Thalassemia (TDT) patients and to assess the correlation with hepatopathy, degree of the hemolysis, transfusion frequency, erythroblastosis, iron chelation.

Methods

TDT patients followed at our center for whom clotting tests were available were included. From chart review data were collected regarding clotting times, demographics, disease history, comorbidities and concomitant medications, iron chelation therapies, iron overload ( serum ferritin, LIC, cardiac T2*), liver function tests, hemolysis parameters, hemocromocitometric values. Patients on anticoagulation therapy were excluded.

Results
56 TDT patients (female 55,35%) were enrolled in our study, mean age 26,02±13,38 years, 17 of them were pediatric. In 20/56 patients (35,71%) prolongation of clotting time was found: this included both prolonged INR ( 23,21%) and prolonged aPTT ratio (25%); 7 patients (12,5%) had both prolonged INR and aPTT. Subgroup with clotting disorder (group A) was compared to subgroup with clotting times within normal ranges (group B) using T-Test. No differences were found in terms of sex, age, genotype, transfusion interval, hemolysis degree, comorbidities, HCV infection included, iron overload, liver function, erythroblastosis and platelets levels, nor in history of thrombotic complications. No patients had history of hemorrhagic disease. Pretransfusion Hb was lower in patients with prolonged clotting times (p=0,045); none of the patients in Group A was splenectomized ( p=0,042).

Conclusion

In our population clotting disorders were not correlated with hepatic disease, nor hemolysis or transfusions. The mild correlation with lower Hb values and with the lacking splenectomy could be consistent with the known effect of low Ht on lab procedures for clotting tests. In relation to this observation in patients with altered coagulation tests the repetition of clotting test after blood transfusion could be advisable to overcome the low Hb effect.

Session topic: 26. Thalassemias

Keyword(s): Thalassemia, Coagulation

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