PB1388 - Sticky platelet syndrome: clinical experience from a tertiary center

Tuesday, June 27, 2023

18:30 – 19:30 ET

Room: Exhibition

Co-Author(s)

CC

Cristina Calderón-Cabrera, PhD

Ph.D., M.D.
Hematology Department, Hospital Universitario Virgen del Rocío. Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.
Seville, Andalucia, Spain

Co-Author(s)

BP

Begoña Pedrote Amador

M.D.
Hematology Department, Hospital Universitario Virgen del Rocío. Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.
Seville, Andalucia, Spain
AD

Antonia Dorado Elías

Laboratory technician
Hematology Department, Hospital Universitario Virgen del Rocío. Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.
Seville, Andalucia, Spain

Presenting Author(s)

RN

Ramiro José Núñez Vázquez

Hematology Department
Hospital Universitario Virgen del Rocío. Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.
Sevilla, Andalucia, Spain

Co-Author(s)

MS

M Dolores Serrano Chacón

M.D.
Hematology Department, Hospital Universitario Virgen del Rocío. Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.
Seville, Andalucia, Spain
PJ

Patricia Jiménez Guerrero

M.D.
Hematology Department, Hospital Universitario de Jerez de la Frontera, Jerez (Cádiz), Spain.
Jerez, Andalucia, Spain
MV

María Teresa Vargas

Ph.D.
Hematology Department, Hospital Universitario Virgen del Rocío. Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.
Sevilla, Andalucia, Spain
JR

Javier Rodríguez-Martorell

Ph.D., M.D.
Hospital Universitario Virgen del Rocío, Sevilla, España
Seville, Andalucia, Spain

Background: Sticky platelet syndrome (SPS) is a rare thrombophilic platelet disorder defined by venous and arterial thromboembolic events with increased aggregation at low doses of adenosine diphosphate (ADP) and/or epinephrine (EPI) on laboratory tests. Treatment relies on attenuate the abnormal platelet hyperaggregability with antiplatelet drugs whereas other antithrombotics are usually ineffective. However, several issues remain controversial such as the pathogenic mechanism involved, diagnostic issues and the optimal therapeutic management.

Aims: To analyze our findings and clinical results regarding therapeutic management in patients with SPS, diagnosed in a reference center in the years 2021-22.

Methods: We report preliminary analysis of 10 patients with SPS. Baseline characteristics, clinical data, familiar occurrence and response to antiplatelet drugs are shown in table 1.

Results: Eight out of 10 patients were aged < 60 years and 7/10 were male. Stroke was diagnosed in 6 patients, ischemic heart disease in 5 and venous thromboembolic disease in 1, having recurrent events in most cases. All initially received acetylsalicylic acid (ASA) with no response by platelet function analyzer (PFA). Only one responded after the addition of clopidogrel whereas all responded after the addition of ticagrelor. A total of 9 cases (90%) had a family history so a hereditary genetic basis can be suspected and next-generation sequencing (NGS) is currently being carried out to identify possible genes involved.

Conclusion(s): SPS is an underdiagnosed pathology that should be suspected in young patients with recurrent arterial and venous thrombotic events, often with other affected relatives. The autosomal dominant pattern supports a genetic basis that requires further investigation. Although response to ASA (based on arachidonic acid aggregation) has been reported, we did not observe response by global tests (PFA) and thienopyridines were required in all cases. The lack of studies limits decision-making about diagnosis and management, so more studies should be addressed.