[Primary antiphospholipid syndrome. Clinical course features of 24 cases]

Describe the clinico-evolutionary characteristics of cases of primary antiphospholipid syndrome (PAPLS) diagnosed at our center.

Retrospective study of all patients with circulating anticoagulant type lupus (AL) (62 patients) and/or anticardiolipin antibodies (ACLA) found in our center since 1981 and 1988, respectively. TTPA, Kaolin's test (KT), and Russel's viper venom time (RVVT) and ACLA type IgG and IgM by ELISA: Only those patients meeting the criteria for PAPLS are included.

Twenty-four patients were diagnosed with PAPLS: Eleven patients were from the enlarged TTPA group, 11 of the total with positive ACLA, and two by KT and abnormal RVVT. As for gender, 18 were women. In the past three years, 19 patients were diagnosed. The diagnostic criteria were from the very beginning: venous thrombosis in nine patients, arterial thrombosis in four, aborted repetitions in three, and plateletopenia in eight. Six patients presented pulmonary thromboembolism, the cause of death in one patient. Thrombopenia was manifested in the evolutionary course of 14 patients, only one of which being severe. Two patients have died (one of them from neoplasia), and two others have had antiphospholipid antibodies become negative. Seven patients remain without treatment, the others undergoing anti-aggregating or anti-coagulant treatment.

Plateletopenia, alone or accompanied by other symptoms, is the most common alteration of PAPLS in our area, followed by venous thrombosis which caused death by pulmonary thromboembolism in one patient. Arterial thrombotic events and aborted repetitions are by no means infrequent.