A catastrophic seronegative anti-phospholipid syndrome: case and literature review

Abstract

Background Antiphospholipid Syndrome (APS) is a multisystemic autoimmune disease characterized by arterial and venous thrombosis and / or obstetric morbidity in the presence of at least one circulating anti-phospholipid antibody. The spectrum of vascular events varies from deep venous thrombosis to catastrophic APS, a rare form characterized by acute multiorgan thrombosis and high mortality. Case report We present the case of a 32-week pregnant woman arriving in the hospital emergency room with bilateral acute lower limb ischemia. In the obstetric evaluation, fetal death was declared. Computerized Tomography angiography showed pulmonary embolism of both pulmonary arteries, areas of splenic and right renal infarction and multiple arterial and venous thrombosis. The patient underwent urgent caesarean section and axillary-bifemoral bypass. No events registered. In the postoperative period, in an intensive care unit, treatment with rituximab and plasmapheresis were added to anticoagulant therapy. The laboratorial investigation was negative for thrombophilia and autoimmune diseases. Conclusion Catastrophic APS develops quickly, with multiorgan involvement and high mortality rate. The presented case poses a multidisciplinary challenge, with the surgical approach of extra-anatomical revascularization being less invasive and guaranteeing immediate perfusion of the lower limbs. Although the serological tests were negative for anti-phospholipid antibodies, this case hardly fits into another diagnosis. Therefore, it was treated as a catastrophic APS, having shown a favorable evolution.

Clinical, Hematological, Biochemical and Radiological Characteristics for Patients With Splenic Infarction: Case Series With Literature Review

“…Hereditary spherocytosis [52] Sickle cell disease [53][54][55][56][57] Hemoglobin SE disease [58] β-Thalassemia [59] Protein C deficiency [60] Antiphospholipid syndrome [61] Vasculitis ANCA-associated vasculitis [62][63][64] Eosinophilic granulomatosis with polyangiitis [65] Cardiovascular disease, atrial fibrillation Atrial fibrillation [66,67] COVID-19: coronavirus disease 2019; ANCA: anti-neutrophil cytoplasmic antibody. rate (30.7%) was found in patients with infective endocarditis [4].…”

An advanced understanding of the heterogeneous clinical features of “non-criteria” obstetric antiphospholipid syndrome: Two case reports and a literature review

“…APS can occur alone, called primary APS; it can also coexist with other autoimmune diseases (most commonly systemic lupus erythematosus, SLE), defined as secondary APS ( 2 ). The clinical presentation spectrum of APS varies in severity and ranges from asymptomatic “carrier” for aPLs, seronegative APS, and OAPS to a life-threatening catastrophic APS (CAPS) ( 3 ).…”