https://www.onlinelibrary.wiley.com/doi/epdf/10.1111/j.1538-7836.2006.01753.x

International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).

https://onlinelibrary.wiley.com/doi/pdf/10.1111/jth.13379

IgG/IgM antiphospholipid antibodies present in the classification criteria for the antiphospholipid syndrome: a critical review of their association with thrombosis.

http://www.apsfa.org/docs/Antiphospholipid%20antibodies%20and%20malignancies.pdf

Antiphospholipid antibodies and malignancies.

The presence of antiphospholipid antibodies has been reported in a large variety of patients with malignancies. Many case reports and reviews have appeared indicating that the presence of the antiphospholipid antibodies is related to thrombotic associations with the antiphospholipid syndrome (APS) in a proportion of these patients. We investigated the frequency of the thrombotic manifestations in 58 patients demonstrating antiphospholipid antibodies and with a history of neoplasia, including haematologic and lymphoproliferative malignancies. Antiphospholipid antibodies were detected by clotting assay [lupus anticoagulant (LAC)] or by enzyme-linked immunosorbent assay [anticardiolipin antibodies (aCL)] according to the Sapporo criteria. Patients, 39/58, suffered from solid tumours and 19/58 patients from malignant haematologic or lymphoproliferative diseases. One patient was suffering simultaneously from two solid tumours and a malignant lymphoma. Among the patients with solid tumours, 18/39 (46%) patients had thromboembolic complications of the antiphospholipid syndrome. Among the patients with haematologic and lymphoproliferative malignancies, only 6/19 (32%) suffered from thromboembolic complications. There was, however, no relation between the titres of aCL antibodies and the clinical manifestations. The presence, but not the titres, of antiphospholipid antibodies may identify a subset of cancer patients with a high risk of developing thrombotic complications. The frequency of thrombosis, however, is lower in aPL-positive patients with lymphoproliferative and haematological malignancies.

Thrombotic manifestations of the antiphospholipid syndrome in patients with malignancies.

It is essential to be aware of both neoplastic and paraneoplastic vasculitides, vasculopathy, and hypercoagulability, considering the importance of an accurate diagnosis and timely treatment of the underlying malignancy. Characteristics such as the type of vasculitis, age, gender, atypical presentation, and lack of response to common therapies should prompt investigation for an occult malignancy, whereas vasculitis such as GPA require due malignancy vigilance given a significantly increased risk of malignancy at the time of diagnosis and in the following years. Vasculopathies are rarer than vasculitides, but are associated with specific malignancies and, in the context of such malignancies, should be kept in mind. Hypercoagulability is a well-documented neoplastic phenomenon with an increased risk of thrombosis in the setting of positive aPLs. Most neoplastic and paraneoplastic vascular syndromes require no specific treatment outside of treatment of the underlying malignancy. The two key exceptions are PACNS, because of its poor prognosis, and erythromelalgia, in which aspirin is an effective agent.

https://www.rheumatic.theclinics.com/article/S0889-857X(11)00057-3/pdf

Neoplastic and paraneoplastic vasculitis, vasculopathy, and hypercoagulability.

The association of antiphospholipid antibodies (APA) has been reported in several cases of patients with non-Hodgkin's lymphoma (NHL) with or without thromboembolic complications. The purpose of this study was to analyse systematically the prevalence of APA and its clinical significance in lymphoma patients. Sera of 90 consecutive unselected patients with NHL were tested for the presence of anticardiolipin (aCL) antibodies and anti-beta2-glycoprotein-I (anti-beta2-GPI) antibodies. The patients were followed up over a median period of 14 months to note the occurrence of thromboembolism. We found APA in 24 out of 90 NHL patients (26.6%). Elevated APA were more often detected in women and in the elderly. The presence of elevated APA was not correlated with the histology and the stage of the lymphoma. None of the 24 patients with elevated APA developed a thromboembolic event in the follow-up period. Thromboembolic events were observed in 12 patients (13.3%), all with negative APA. High APA titres and the combination of positive aCL- and anti-beta2-GPI antibodies, features which are known to be more strongly correlated with thrombosis among patients with antiphospholipid syndrome and systemic lupus erythematous (SLE), were very uncommon in our cohort of NHL patients (3.3%). Vessel compression by lymphoma but not elevated APA remains the main cause of thrombosis in NHL patients.

Prevalence and clinical significance of anticardiolipin and anti-beta2-glycoprotein-I antibodies in patients with non-Hodgkin's lymphoma.

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Prevalence and clinical significance of anticardiolipin and anti-beta2-glycoprotein-I antibodies in patients with non-Hodgkin's lymphoma.