FUNCTIONAL PLATELET ACTIVITY ANALYSIS IN CHILDREN WITH BLEEDING.
(Abstract release date: 05/19/16)
EHA Library. Zharkov P. 06/09/16; 132550; E1001
Disclosure(s): none
Dr. Pavel Zharkov
Contributions
Contributions
Abstract
Abstract: E1001
Type: Eposter Presentation
Background
Diagnosis in a bleeding child without thrombocytopenia or clotting factor deficiency is always challenging. In these cases using a platelet flow cytometry (PFC) method might be helpful because it can detect several glycoprotein deficiencies on platelet surface and confirm the diagnosis of platelet dysfunction. Here we present a case series of patients with bleeding due different platelet disorders, established by a modification of PFC method – functional platelet activity analysis (FPAA).
Aims
to asses the role of a modification of platelet flow cytometry (PFC) method – functional platelet activity analysis (FPAA) in children with bleeding.
Methods
32 patients (1-17 yo) with mild to severe bleeding without von Willebrand disease, factor XIII deficiency, or other coagulopathy were included. Whole blood platelets were studied by FC either in native or activated with collagen-related peptide (0.18 µg/ml) and thrombin receptor activating peptide (12.5 µM). Fluorescencently labeled antibodies against CD42b, CD61 and PAC-1, phosphatidylserine (PS) and CD62p were used; dense granule (DG) release was studied using loading with mepacrine.
Results
After FPAA from 32 children 2 pts were excluded because of normal results and 1 because of preanalytical issues. From those 29, 4 pts were diagnosed with CD61 deficiency, and 25 – with different granule defects. Isolated deficiency or mobilization defect of dense and alpha granules was found in 9 and 4 children, respectively. Combined storage pool deficiency/mobilization defect (CSPD/MD) was diagnosed in 8, while we found 1 combination of CSPD/MD and PAC-1 deficiency, and 3 combinations of CSPD/MD and PS deficiency.
Conclusion
Different platelet disorders could be found in up to 93,5% of patients with bleeding without clotting factor deficiency. Most of the alterations include storage alpha/dense granule pool deficiency/mobilization defect or CSPD/MD.
Session topic: E-poster
Type: Eposter Presentation
Background
Diagnosis in a bleeding child without thrombocytopenia or clotting factor deficiency is always challenging. In these cases using a platelet flow cytometry (PFC) method might be helpful because it can detect several glycoprotein deficiencies on platelet surface and confirm the diagnosis of platelet dysfunction. Here we present a case series of patients with bleeding due different platelet disorders, established by a modification of PFC method – functional platelet activity analysis (FPAA).
Aims
to asses the role of a modification of platelet flow cytometry (PFC) method – functional platelet activity analysis (FPAA) in children with bleeding.
Methods
32 patients (1-17 yo) with mild to severe bleeding without von Willebrand disease, factor XIII deficiency, or other coagulopathy were included. Whole blood platelets were studied by FC either in native or activated with collagen-related peptide (0.18 µg/ml) and thrombin receptor activating peptide (12.5 µM). Fluorescencently labeled antibodies against CD42b, CD61 and PAC-1, phosphatidylserine (PS) and CD62p were used; dense granule (DG) release was studied using loading with mepacrine.
Results
After FPAA from 32 children 2 pts were excluded because of normal results and 1 because of preanalytical issues. From those 29, 4 pts were diagnosed with CD61 deficiency, and 25 – with different granule defects. Isolated deficiency or mobilization defect of dense and alpha granules was found in 9 and 4 children, respectively. Combined storage pool deficiency/mobilization defect (CSPD/MD) was diagnosed in 8, while we found 1 combination of CSPD/MD and PAC-1 deficiency, and 3 combinations of CSPD/MD and PS deficiency.
Conclusion
Different platelet disorders could be found in up to 93,5% of patients with bleeding without clotting factor deficiency. Most of the alterations include storage alpha/dense granule pool deficiency/mobilization defect or CSPD/MD.
Session topic: E-poster
Abstract: E1001
Type: Eposter Presentation
Background
Diagnosis in a bleeding child without thrombocytopenia or clotting factor deficiency is always challenging. In these cases using a platelet flow cytometry (PFC) method might be helpful because it can detect several glycoprotein deficiencies on platelet surface and confirm the diagnosis of platelet dysfunction. Here we present a case series of patients with bleeding due different platelet disorders, established by a modification of PFC method – functional platelet activity analysis (FPAA).
Aims
to asses the role of a modification of platelet flow cytometry (PFC) method – functional platelet activity analysis (FPAA) in children with bleeding.
Methods
32 patients (1-17 yo) with mild to severe bleeding without von Willebrand disease, factor XIII deficiency, or other coagulopathy were included. Whole blood platelets were studied by FC either in native or activated with collagen-related peptide (0.18 µg/ml) and thrombin receptor activating peptide (12.5 µM). Fluorescencently labeled antibodies against CD42b, CD61 and PAC-1, phosphatidylserine (PS) and CD62p were used; dense granule (DG) release was studied using loading with mepacrine.
Results
After FPAA from 32 children 2 pts were excluded because of normal results and 1 because of preanalytical issues. From those 29, 4 pts were diagnosed with CD61 deficiency, and 25 – with different granule defects. Isolated deficiency or mobilization defect of dense and alpha granules was found in 9 and 4 children, respectively. Combined storage pool deficiency/mobilization defect (CSPD/MD) was diagnosed in 8, while we found 1 combination of CSPD/MD and PAC-1 deficiency, and 3 combinations of CSPD/MD and PS deficiency.
Conclusion
Different platelet disorders could be found in up to 93,5% of patients with bleeding without clotting factor deficiency. Most of the alterations include storage alpha/dense granule pool deficiency/mobilization defect or CSPD/MD.
Session topic: E-poster
Type: Eposter Presentation
Background
Diagnosis in a bleeding child without thrombocytopenia or clotting factor deficiency is always challenging. In these cases using a platelet flow cytometry (PFC) method might be helpful because it can detect several glycoprotein deficiencies on platelet surface and confirm the diagnosis of platelet dysfunction. Here we present a case series of patients with bleeding due different platelet disorders, established by a modification of PFC method – functional platelet activity analysis (FPAA).
Aims
to asses the role of a modification of platelet flow cytometry (PFC) method – functional platelet activity analysis (FPAA) in children with bleeding.
Methods
32 patients (1-17 yo) with mild to severe bleeding without von Willebrand disease, factor XIII deficiency, or other coagulopathy were included. Whole blood platelets were studied by FC either in native or activated with collagen-related peptide (0.18 µg/ml) and thrombin receptor activating peptide (12.5 µM). Fluorescencently labeled antibodies against CD42b, CD61 and PAC-1, phosphatidylserine (PS) and CD62p were used; dense granule (DG) release was studied using loading with mepacrine.
Results
After FPAA from 32 children 2 pts were excluded because of normal results and 1 because of preanalytical issues. From those 29, 4 pts were diagnosed with CD61 deficiency, and 25 – with different granule defects. Isolated deficiency or mobilization defect of dense and alpha granules was found in 9 and 4 children, respectively. Combined storage pool deficiency/mobilization defect (CSPD/MD) was diagnosed in 8, while we found 1 combination of CSPD/MD and PAC-1 deficiency, and 3 combinations of CSPD/MD and PS deficiency.
Conclusion
Different platelet disorders could be found in up to 93,5% of patients with bleeding without clotting factor deficiency. Most of the alterations include storage alpha/dense granule pool deficiency/mobilization defect or CSPD/MD.
Session topic: E-poster
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