MULTIPLE PHENOTYPIC EXPRESSION OF HARRIS PLATELET SYNDROME:
(Abstract release date: 05/19/16)
EHA Library. Das A. 06/09/16; 134971; PB2071
Dr. Avash Das
Contributions
Contributions
Abstract
Abstract: PB2071
Type: Publication Only
Background
Harris platelet syndrome (HPS) is an autosomal dominant macrothrombocytopenia with mild to moderately severe thrombocytopenia and normal platelet function. HPS has been reported from healthy blood donors of the Indian subcontinent, particularly from the northeastern part of India including West Bengal.
Aims
A prospective study was done to characterize the different phenotypes of HPS among blood donors from West Bengal.
Methods
HPS is defined as healthy blood donors having a mean platelet volume (MPV) greater than 12 fL (normal <10 fL) with or without concomitant thrombocytopenia in the absence of any significant past history of bleeding disorder in either themselves or their first degree relatives. Blood samples were collected in ethylenediaminetetraacetic acid (EDTA) and analyzed using impedancemethod (Coulter SYSMEX X100, Tokyo, Japan).
Results
A total of 326 eligible blood donors were included in this study. Two hundred (61%) of the blood donors fulfilled criteria for a diagnosis of HPS. Three distinct phenotypes were observed. Fourteen ( 4% ) was noted to have varying thrombocytopenia with normal MPV; 166 ( 51% ) had anormal platelet count with giant platelets and 20 (6%) donors had classic HPS characterized by thrombocytopenia with giant platelets . HPS donors with low platelet count had a lower median platelet bio-mass compared with their normal counterparts, (13.62 vs. 29.38, p<0.001).In comparing peripheral smears between controls (i.e normal platelet count and normal MPV) and the variants of HPS, no obvious difference was noted in the red blood cell morphology.Table.
Conclusion
A prospective population based study will be ideal to further explore the clinico-pathological significance of these variants of HPS.This study shows that HPS may manifest with isolated thrombocytopenia without giant platelets. It is important to reccognize this variant to avoid unnecessary investigations and treatment.
Session topic: E-poster
Type: Publication Only
Background
Harris platelet syndrome (HPS) is an autosomal dominant macrothrombocytopenia with mild to moderately severe thrombocytopenia and normal platelet function. HPS has been reported from healthy blood donors of the Indian subcontinent, particularly from the northeastern part of India including West Bengal.
Aims
A prospective study was done to characterize the different phenotypes of HPS among blood donors from West Bengal.
Methods
HPS is defined as healthy blood donors having a mean platelet volume (MPV) greater than 12 fL (normal <10 fL) with or without concomitant thrombocytopenia in the absence of any significant past history of bleeding disorder in either themselves or their first degree relatives. Blood samples were collected in ethylenediaminetetraacetic acid (EDTA) and analyzed using impedancemethod (Coulter SYSMEX X100, Tokyo, Japan).
Results
A total of 326 eligible blood donors were included in this study. Two hundred (61%) of the blood donors fulfilled criteria for a diagnosis of HPS. Three distinct phenotypes were observed. Fourteen ( 4% ) was noted to have varying thrombocytopenia with normal MPV; 166 ( 51% ) had anormal platelet count with giant platelets and 20 (6%) donors had classic HPS characterized by thrombocytopenia with giant platelets . HPS donors with low platelet count had a lower median platelet bio-mass compared with their normal counterparts, (13.62 vs. 29.38, p<0.001).In comparing peripheral smears between controls (i.e normal platelet count and normal MPV) and the variants of HPS, no obvious difference was noted in the red blood cell morphology.Table.
| Blood Indices | Giant platelets with Normal Platelet Count(N=166) | Giant platelets with thrombovytopenia(N=20) | Normal MPV with thrombocytopenia(N=14) |
| Haemoglobin | 13.74 ± 2.102 | 13.07 ± 2.250 | 12.84 ± 2.213 |
| MCV | 89.47 ± 6.720 | 88.45 ± 8.703 | 89.43 ± 6.915 |
| Platelet Count | 2.37 ± 0.609 | 1.02 ± 0.282 | 1.01 ± 0.278 |
| MPV | 13.52 ± 1.137 | 13.22 ± 0.879 | 10.61 ± 0.896 |
| PDW | 20.77 ± 3.001 | 20.48 ± 2.726 | 18.09 ± 1.889 |
| Platelet Biomass | 29.38 ± 6.761 | 13.62 ± 4.061 | 11.02 ± 3.494 |
Conclusion
A prospective population based study will be ideal to further explore the clinico-pathological significance of these variants of HPS.This study shows that HPS may manifest with isolated thrombocytopenia without giant platelets. It is important to reccognize this variant to avoid unnecessary investigations and treatment.
Session topic: E-poster
Abstract: PB2071
Type: Publication Only
Background
Harris platelet syndrome (HPS) is an autosomal dominant macrothrombocytopenia with mild to moderately severe thrombocytopenia and normal platelet function. HPS has been reported from healthy blood donors of the Indian subcontinent, particularly from the northeastern part of India including West Bengal.
Aims
A prospective study was done to characterize the different phenotypes of HPS among blood donors from West Bengal.
Methods
HPS is defined as healthy blood donors having a mean platelet volume (MPV) greater than 12 fL (normal <10 fL) with or without concomitant thrombocytopenia in the absence of any significant past history of bleeding disorder in either themselves or their first degree relatives. Blood samples were collected in ethylenediaminetetraacetic acid (EDTA) and analyzed using impedancemethod (Coulter SYSMEX X100, Tokyo, Japan).
Results
A total of 326 eligible blood donors were included in this study. Two hundred (61%) of the blood donors fulfilled criteria for a diagnosis of HPS. Three distinct phenotypes were observed. Fourteen ( 4% ) was noted to have varying thrombocytopenia with normal MPV; 166 ( 51% ) had anormal platelet count with giant platelets and 20 (6%) donors had classic HPS characterized by thrombocytopenia with giant platelets . HPS donors with low platelet count had a lower median platelet bio-mass compared with their normal counterparts, (13.62 vs. 29.38, p<0.001).In comparing peripheral smears between controls (i.e normal platelet count and normal MPV) and the variants of HPS, no obvious difference was noted in the red blood cell morphology.Table.
Conclusion
A prospective population based study will be ideal to further explore the clinico-pathological significance of these variants of HPS.This study shows that HPS may manifest with isolated thrombocytopenia without giant platelets. It is important to reccognize this variant to avoid unnecessary investigations and treatment.
Session topic: E-poster
Type: Publication Only
Background
Harris platelet syndrome (HPS) is an autosomal dominant macrothrombocytopenia with mild to moderately severe thrombocytopenia and normal platelet function. HPS has been reported from healthy blood donors of the Indian subcontinent, particularly from the northeastern part of India including West Bengal.
Aims
A prospective study was done to characterize the different phenotypes of HPS among blood donors from West Bengal.
Methods
HPS is defined as healthy blood donors having a mean platelet volume (MPV) greater than 12 fL (normal <10 fL) with or without concomitant thrombocytopenia in the absence of any significant past history of bleeding disorder in either themselves or their first degree relatives. Blood samples were collected in ethylenediaminetetraacetic acid (EDTA) and analyzed using impedancemethod (Coulter SYSMEX X100, Tokyo, Japan).
Results
A total of 326 eligible blood donors were included in this study. Two hundred (61%) of the blood donors fulfilled criteria for a diagnosis of HPS. Three distinct phenotypes were observed. Fourteen ( 4% ) was noted to have varying thrombocytopenia with normal MPV; 166 ( 51% ) had anormal platelet count with giant platelets and 20 (6%) donors had classic HPS characterized by thrombocytopenia with giant platelets . HPS donors with low platelet count had a lower median platelet bio-mass compared with their normal counterparts, (13.62 vs. 29.38, p<0.001).In comparing peripheral smears between controls (i.e normal platelet count and normal MPV) and the variants of HPS, no obvious difference was noted in the red blood cell morphology.Table.
| Blood Indices | Giant platelets with Normal Platelet Count(N=166) | Giant platelets with thrombovytopenia(N=20) | Normal MPV with thrombocytopenia(N=14) |
| Haemoglobin | 13.74 ± 2.102 | 13.07 ± 2.250 | 12.84 ± 2.213 |
| MCV | 89.47 ± 6.720 | 88.45 ± 8.703 | 89.43 ± 6.915 |
| Platelet Count | 2.37 ± 0.609 | 1.02 ± 0.282 | 1.01 ± 0.278 |
| MPV | 13.52 ± 1.137 | 13.22 ± 0.879 | 10.61 ± 0.896 |
| PDW | 20.77 ± 3.001 | 20.48 ± 2.726 | 18.09 ± 1.889 |
| Platelet Biomass | 29.38 ± 6.761 | 13.62 ± 4.061 | 11.02 ± 3.494 |
Conclusion
A prospective population based study will be ideal to further explore the clinico-pathological significance of these variants of HPS.This study shows that HPS may manifest with isolated thrombocytopenia without giant platelets. It is important to reccognize this variant to avoid unnecessary investigations and treatment.
Session topic: E-poster
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