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Effects of Factor XIII Deficiency on Thromboelastography. Thromboelastography with Calcium and Streptokinase Addition is more Sensitive than Solubility Tests

View Article: PubMed Central - PubMed

ABSTRACT

Background: Homozygous or double heterozygous factor XIII (FXIII) deficiency is characterized by soft tissue hematomas, intracranial and delayed spontaneous bleeding. Alterations of thromboelastography (TEG) parameters in these patients have been reported. The aim of the study was to show results of TEG, TEG Lysis (Lys 60) induced by subthreshold concentrations of streptokinase (SK), and to compare them to the clot solubility studies results in samples of a 1-year-old girl with homozygous or double heterozygous FXIII deficiency.

Case: A year one girl with a history of bleeding from the umbilical cord. During her first year of life, several hematomas appeared in soft upper limb tissue after punctures for vaccination and a gluteal hematoma. One additional sample of a heterozygous patient and three samples of acquired FXIII deficiency were also evaluated.

Materials and methods: Clotting tests, von Willebrand factor (vWF) antigen and activity, plasma FXIII-A subunit (pFXIII-A) were measured by an immunoturbidimetric assay in a photo-optical coagulometer. Solubility tests were performed with Ca2+-5 M urea and thrombin-2% acetic acid. Basal and post-FXIII concentrate infusion samples were studied. TEG was performed with CaCl2 or CaCl2 + SK (3.2 U/mL) in a Thromboelastograph.

Results: Prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time, fibrinogen, factor VIIIc, vWF, and platelet aggregation were normal. Antigenic pFXIII-A subunit was < 2%. TEG, evaluated at diagnosis and post FXIII concentrate infusion (pFXIII-A= 37%), presented a normal reaction time (R), 8 min, prolonged k (14 and 11min respectively), a low Maximum-Amplitude (MA) ( 39 and 52 mm respectively), and Clot Lysis (Lys60) slightly increased (23 and 30% respectively). In the sample at diagnosis, clot solubility was abnormal, 50 and 45 min with Ca-Urea and thrombin-acetic acid, respectively, but normal (>16 hours) 1-day post-FXIII infusion. Analysis of FXIII deficient and normal plasma mixtures (< 2–102% of pFXIII-A), showed that Ca-urea solubility was abnormal at pFXIII-A < 9%, thrombin-acetic acid at pFXIII-A<18%, but TEG MA and elasticity at 23% and Lys60 with SK at pFXIII-A< 40%.

Conclusions: TEG parameters MA and elasticity, and Lys 60 in TEG either with Ca2+ or Ca2+ and SK are more sensitive to low levels of pFXIII than solubility tests. The increased Lys60 induced by a subthreshold concentration of SK could probably reflect the clot characteristics “in vivo” in many patients with pFXIII levels between 5–40% and could be potentially considered as screening test.

No MeSH data available.


Whole blood TEGs of a normal control and patient’s samples at different time points: at diagnosis, 24 hours post the first replacement therapy and 21 days after the last replacement, 12 months from replacement therapy start.
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f1-mjhid-8-1-e2016037: Whole blood TEGs of a normal control and patient’s samples at different time points: at diagnosis, 24 hours post the first replacement therapy and 21 days after the last replacement, 12 months from replacement therapy start.

Mentions: Results of coagulation tests obtained from patient’s plasma and platelet function results are shown in Table 1. TEG of de patient’s whole blood at diagnosis was pathologic, with prolonged k, diminished α angle, MA and ɛ, and increased Lys 60. To test the lysis of the clot, the analysis of TEGs from normal, patient at diagnosis, at day one post-FXIII concentrate infusion and day 21 after one year of replacement therapy, were performed with or without SK(subthreshold concentration). Both basal and one-day post infusion TEG showed completely lysis with SK addition but not normal donor’s one (Figure 1). Parameters of TEGs from patient whole blood pre and day-1 post-FXIII infusion, with and without SK addition, as well as 21 days post infusion are shown in Table 2. As it can be seen, low pFXIII-A levels have not significant influence in R but affect k, MA, ɛ and Lys 60, except in 21 days post infusion sample after a one year period of prophylactic treatment, in which only Lys 60 was increased.


Effects of Factor XIII Deficiency on Thromboelastography. Thromboelastography with Calcium and Streptokinase Addition is more Sensitive than Solubility Tests
Whole blood TEGs of a normal control and patient’s samples at different time points: at diagnosis, 24 hours post the first replacement therapy and 21 days after the last replacement, 12 months from replacement therapy start.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5016016&req=5

f1-mjhid-8-1-e2016037: Whole blood TEGs of a normal control and patient’s samples at different time points: at diagnosis, 24 hours post the first replacement therapy and 21 days after the last replacement, 12 months from replacement therapy start.
Mentions: Results of coagulation tests obtained from patient’s plasma and platelet function results are shown in Table 1. TEG of de patient’s whole blood at diagnosis was pathologic, with prolonged k, diminished α angle, MA and ɛ, and increased Lys 60. To test the lysis of the clot, the analysis of TEGs from normal, patient at diagnosis, at day one post-FXIII concentrate infusion and day 21 after one year of replacement therapy, were performed with or without SK(subthreshold concentration). Both basal and one-day post infusion TEG showed completely lysis with SK addition but not normal donor’s one (Figure 1). Parameters of TEGs from patient whole blood pre and day-1 post-FXIII infusion, with and without SK addition, as well as 21 days post infusion are shown in Table 2. As it can be seen, low pFXIII-A levels have not significant influence in R but affect k, MA, ɛ and Lys 60, except in 21 days post infusion sample after a one year period of prophylactic treatment, in which only Lys 60 was increased.

View Article: PubMed Central - PubMed

ABSTRACT

Background: Homozygous or double heterozygous factor XIII (FXIII) deficiency is characterized by soft tissue hematomas, intracranial and delayed spontaneous bleeding. Alterations of thromboelastography (TEG) parameters in these patients have been reported. The aim of the study was to show results of TEG, TEG Lysis (Lys 60) induced by subthreshold concentrations of streptokinase (SK), and to compare them to the clot solubility studies results in samples of a 1-year-old girl with homozygous or double heterozygous FXIII deficiency.

Case: A year one girl with a history of bleeding from the umbilical cord. During her first year of life, several hematomas appeared in soft upper limb tissue after punctures for vaccination and a gluteal hematoma. One additional sample of a heterozygous patient and three samples of acquired FXIII deficiency were also evaluated.

Materials and methods: Clotting tests, von Willebrand factor (vWF) antigen and activity, plasma FXIII-A subunit (pFXIII-A) were measured by an immunoturbidimetric assay in a photo-optical coagulometer. Solubility tests were performed with Ca2+-5 M urea and thrombin-2% acetic acid. Basal and post-FXIII concentrate infusion samples were studied. TEG was performed with CaCl2 or CaCl2 + SK (3.2 U/mL) in a Thromboelastograph.

Results: Prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time, fibrinogen, factor VIIIc, vWF, and platelet aggregation were normal. Antigenic pFXIII-A subunit was < 2%. TEG, evaluated at diagnosis and post FXIII concentrate infusion (pFXIII-A= 37%), presented a normal reaction time (R), 8 min, prolonged k (14 and 11min respectively), a low Maximum-Amplitude (MA) ( 39 and 52 mm respectively), and Clot Lysis (Lys60) slightly increased (23 and 30% respectively). In the sample at diagnosis, clot solubility was abnormal, 50 and 45 min with Ca-Urea and thrombin-acetic acid, respectively, but normal (>16 hours) 1-day post-FXIII infusion. Analysis of FXIII deficient and normal plasma mixtures (< 2–102% of pFXIII-A), showed that Ca-urea solubility was abnormal at pFXIII-A < 9%, thrombin-acetic acid at pFXIII-A<18%, but TEG MA and elasticity at 23% and Lys60 with SK at pFXIII-A< 40%.

Conclusions: TEG parameters MA and elasticity, and Lys 60 in TEG either with Ca2+ or Ca2+ and SK are more sensitive to low levels of pFXIII than solubility tests. The increased Lys60 induced by a subthreshold concentration of SK could probably reflect the clot characteristics “in vivo” in many patients with pFXIII levels between 5–40% and could be potentially considered as screening test.

No MeSH data available.