The Issue of the Antiphospholipid Antibody Syndrome

Abstract

Antiphospholipid antibody syndrome (APS) is a state of hypercoagulability secondary to an autoimmune disorder. It is associated with thrombotic events in venous and arterial vessels, obstetric complications characterized by recurrent fetal losses, and increased perinatal morbidity. APS is classified as primary, when not associated with other pathologies; or secondary, when associated with an underlying autoimmune disease with, solid tumor, or hematological disorder. Clinical findings include livedo reticularis, thrombocytopenia or hemolytic anemia, maternal morbidity, and recurrent thrombotic episodes and others. Laboratory tests show circulating antiphospholipid antibodies (aPLs); however, even in the presence of these antibodies, patients can be asymptomatic. Estimates predict that about 5% of the populations have circulating aPLs, but the incidence of APS is only five cases per 100,000 people, as diagnosis of this syndrome requires clinical and laboratory findings to be simultaneously present. In cases of secondary APS, or in acute cases with imminent risk of death (as in catastrophic APS), it may be necessary to reduce aPL serum levels using immunomodulators, immunosuppressants, or plasmapheresis, in order to treat the associated pathologies. In other situations, the use of immunotherapy is not indicated. In other patients heparin, aspirin or anticoagulants either alone or associated should be administered depending on each specific case.

Adrenal Hemorrhage in a Cortisol-Secreting Adenoma Caused by Antiphospholipid Syndrome Revealed by Clinical and Pathological Investigations: A Case Report

“…In our present case, hemorrhage in the tumor possibly caused by antiphospholipid syndrome was accurately diagnosed through both pathological and clinical investigations. Antiphospholipid syndrome was reported to represent 1-5% in the general population (24). Taking the prevalence of antiphospholipid syndrome into consideration, adrenal hemorrhage due to antiphospholipid syndrome should exist to some extent as the previous reports indicated (6,(13)(14)(15)).…”

Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study

“…Antiphospholipid syndrome (APS) is an autoimmune disease characterized by persistent antiphospholipid antibodies (aPLs) positivity, leading to thrombotic events or obstetric morbidity. Despite considered as a rare disease with an annual incidence of 5 cases/100,000 and a low prevalence of 40~50 cases/100,000 [ 1 ], APS was responsible for 25–33% of early-onset (< 50 years old) cerebrovascular events, 15–30% of all deep venous thrombosis (DVT) episodes, and 10–15% of recurrent fetal loss [ 2 ]. It usually affected adults of reproductive age, with a female/male ratio of over 3:1 [ 3 ].…”

Comparison of Different Test Systems for the Detection of Antiphospholipid Antibodies in a Chinese Cohort

“…Since aPL detection comprise a large part of APS diagnosis, a detection system with high sensitivity and specificity is required in order to timely identify APS patients as well as provide accurate clinical intervention (3). Besides, evaluation of aPLs could also contribute to prognosis and risk assessment for associated clinical manifestations (4,5).…”