Antiphospholipid antibody syndrome (APS) is an autoimmune disorder characterised by thrombosis and the presence of antiphospholipid antibodies (aPL): lupus anticoagulant and/or IgG/IgM anti-β2-glycoprotein I and anticardiolipin antibodies. APS carries significant morbidity for a relatively young patient population from recurrent thrombosis in any vascular bed (arterial, venous, or microvascular), often despite current standard of care, which is anticoagulation with vitamin K antagonists (VKA). Platelets have established roles in thrombosis at any site, and platelet hyperreactivity is clearly demonstrated in the pathophysiology of APS. Together with excess thrombin generation, platelet activation and aggregation are the common end result of all the pathophysiological pathways leading to thrombosis in APS. However, antiplatelet therapies play little role in APS, reserved as a possible option of low dose aspirin in addition to VKA in arterial or refractory thrombosis. This review outlines the current evidence and mechanisms for excessive platelet activation in APS, how it plays a central role in APS-related thrombosis, what evidence for antiplatelets is available in clinical outcomes studies, and potential future avenues to define how to target platelet hyperreactivity better with minimal impact on haemostasis.
Waldenström Macroglobulinaemia is an indolent disease which is recognised as contributing to a diverse range of disabling symptoms which can impact quality of life. Little data are available in evaluating patient and healthcare professional views on quality-of-life questionnaires (QLQ) used to evaluate quality of life in Waldenström Macroglobulinaemia. We aimed to assess patient perception of their disease and the relevance of the currently employed QLQs in practice by patients and healthcare professionals. One-hundred-and-twenty patients (22% treatment-naïve) and thirty-four healthcare professionals completed an international survey to understand their perception of the relevance of the currently employed QLQ. Out of 415 questions, 257 questions were classified by patients as relevant. Five themes were discerned as relevant: fatigue, mobility, dyspnoea, paraesthesia, pain in the hands and feet, sleep (physical health limitations); contentment (psychological impact); social and family support. The following were considered irrelevant: lumps or swelling, dry mouth, trouble hearing, sexual dysfunction (physical symptoms); post-traumatic stress (psychological impact); education and training and financial support. Healthcare professionals identified EORTC QLQ-C30 as the most commonly used instruments for Waldenström Macroglobulinaemia trials.