IMEX   05356
INSTITUTO DE MEDICINA EXPERIMENTAL
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
Usefulness of Optical Aggregometry and Flow Cytometric Studies as a Complementary Tools in Patients with Acquired Platelet Defects (APD)
Autor/es:
ALBERTO MF; AGAZZONI M; ASENSIO M; ROMERO ML; BERMEJO E; SÁNCHEZ LUCEROS A
Lugar:
Dublin
Reunión:
Congreso; 64th Annual Scientific and Standardization Committee (SSC); 2018
Institución organizadora:
International Society on Thrombosis and Haemostasis, Scientific and Standardization Committee (SSC)
Resumen:
Background: Acquired nature of bleeding disorder is suggested by a negative personal and family bleeding history. In many cases, diagnosis could be difficult to elucidate. In APD, the association of functional and non functional methods, including mixing assays, may contribute to diagnosis.Aims: To evaluate the usefulness of optical aggregation (OA) and flow cytometry (FC) to characterize APD.Methods: Patient 1 (P1): a 4-year-old boy with sudden spontaneous bruising, petechia, epistaxis and gums bleeding. P2: a 65 years old man with a recent diagnosis of multiple myeloma (monoclonal IgG 2.4 g/dL), presenting spontaneous severe bilateral epistaxis. P3: a 31-year-old woman on a surgery plan with precedent of PTI on childhood. Hemostatic routine tests; VWF:Ag; VWF:RCo; FVIII; FI; OA on platelet rich plasm (PRP) and mixing studies using ADP, ADR, arachidonic acid, collagen and ristocetin; expression of GPIb, GPIIb, GPIIIa, direct and indirect platelet associated immunoglobulin (PAIgG, PAIgM) and mepacrine uptake (MU) measured by FC; were performed.Results: Results are shown on Tables 1 and 2. P1: showed Glanzmann′s thrombasthenia like pattern on OA; no inhibitory effect of patient′s poor platelet plasm (P′s PPP) upon a normal PRP and elevated levels of both PAIgG -PAIgM on direct assay. P2: had exclusive absence of ristocetin aggregation (up to 2 mg/mL) that not normalized when VWF was added; inhibition of normal PRP ristocetin aggregation (1.2 mg/mL) induced by P′s PPP and normal levels ofPAIgG-PAIgM.P3: showed exclusive absence of collagen aggregation; aggregation of normal PRP induced by P′s PPP (without agonist) and elevated levels of PAIgM. All of them presented normal expression of GPIb, GPIIb, GPIIIa and MU.Conclusions: We presented three patients with different clinical context where the combination of functional studies as OA and the evaluation of platelet glycoproteins and PA immunoglobulins by FC was the key to reach a diagnosis of APD, necessary to guide to the most appropriate treatment.