Abnormal immunothrombosis and lupus anticoagulant in a catastrophic COVID-19 recalling Asherson’s syndrome

Abstract

Background Coronavirus disease 2019 (COVID-19) is a complex disease with many clinicopathological aspects, including abnormal immunothrombosis, and the full comprehension of its pathogenetic mechanisms is urgently required. Methods/Results By means of a multidisciplinary approach, we here report a catastrophic COVID-19 in a 44-year-old Philippine male patient, discovered lupus anticoagulant (LAC)-positive shortly before death, occurred 8 days after hospitalization in a clinical scenario refractory to standard high acuity care recalling Asherson's syndrome (catastrophic antiphospholipid syndrome). Conclusion A parallelism between this severe form of COVID-19 and Asherson's syndrome can be so drawn. Both the diseases in fact exhibit hypercytokinemia, thrombotic microangiopathy, disseminated intravascular coagulation and multiple organ failure, they show a relationship with viral infections, and they are burdened by a high mortality rate. A genetic predisposition to develop these two overlapping conditions may be supposed. Keywords Coronavirus disease 2019 (COVID-19) • Abnormal immunothrombosis • Lupus anticoagulant (LAC) • Megakaryocytes • Antiphospholipid syndrome (Hughes syndrome) • Asherson's syndrome Highlights• COVID-19 is the most dramatic pandemic of the new millennium characterized by many clinicopathological facets, among which abnormal immunothrombosis • Abnormal immunothrombosis is a leading cause of COVID-19 related deaths, and its impact can be amplified by the presence of antiphospholipid autoantibodies, such as LAC • In this setting, the simultaneous occurrence of venous and arterial thromboses should alert the clinicians towards a secondary antiphospholipid syndrome, sometimes burdened by a catastrophic evolution in genetically predisposed patients • The catastrophic variant of a secondary antiphospholipid syndrome takes also the name of Asherson's syndrome, from the rheumatologist Ronald Andrew Asherson, who described it in 1992 • The implications of all this for future directions are in favor of a routine antiphospholipid testing in severe COVID-19 patients, and its introduction under intensive care as potential prognostic risk marker

A Three-Case Series of Thrombotic Deaths in Patients over 50 with Comorbidities Temporally after modRNA COVID-19 Vaccination

“…Even if anti-PF4 immunohistochemistry cannot be used in VITT assessment since it is highly sensible but unspecific, the detection of CD61-positive PF4-positive platelets clots not only attached to the endothelial surface, where PF4 exerts its physiological action, but also inside the lumen of vessels appears useful to testify to pathological intravascular platelet activation and aggregation. Notably, in the two female patients of our series, the lung histopathological picture was overlapping and characterized by distinctive PF4-rich thrombotic phenomena inside the microcirculation (microthromboses), quite similar from a morphological point of view to those described in patients who have died from severe COVID-19, in which capillary dysfunction has been proven to interfere negatively with blood and tissue oxygenation [57][58][59]. It is reasonable to assume that postinfection or post-vaccination antibodies may behave as platelet-activating factors in rare circumstances, thus generating a hypercoagulable state and an abnormal immunothrom-bosis, as hypothesized by the Society of Thrombosis and Haemostasis Research [5]; a genetic predisposition to develop such dysimmune/autoimmune complications may be only supposed.…”

A novel case of lupus nephritis and mixed connective tissue disorder in a COVID-19 patient

“…Out of the total papers, eleven articles were from Asia [ 19 , 22 , 25 , 30 , 32 , 33 , 36 , 39 , 41 , 43 , 44 ], eight from Europe [ 15 , 17 , 18 , 20 , 24 , 27 , 38 , 45 ], eleven from North America [ 14 , 16 , 26 , 28 , 29 , 34 , 35 , 37 , 40 , 42 , 46 ], two from South America [ 21 , 31 ] and one from Africa [ 23 ].…”

Untangling the Intricacies of Infection, Thrombosis, Vaccination, and Antiphospholipid Antibodies for COVID-19

“…Others find that the presence of antiphospholipid antibodies may be a strong predictor of endothelial activation in vitro [73]. It remains tempting to propose that a rapid and severe COVID-19 illness may bear similarity to catastrophic antiphospholipid syndrome when associated antibodies are determined [75].…”