PRIMARY ANTIPHOSPHOLIPID SYNDROME - CLINI COEVOLUTIONARY ASPECTS OF 24 CASES

Objectives: Describe the clinico-evolutionary characteristics of cases of primary antiphospholipid syndrome (PAPLS) diagnosed at our center. Patients and methods. Retrospective study of all patients with circul ating anticoagulant type lupus (AL) (62 patients) and/or anticardiolip in antibodies (ACLA) found in our center since 1981 and 1988, respecti vely. TTPA, Kaolin's test (KT), and Russel's viper venom time (RVVT) a nd ACLA type IgG and IgM by ELISA. Only those patients meeting the cri teria for PAPLS are included. Results. Twenty-four patients were diagn osed 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 d iagnosed. The diagnostic criteria were from the very beginning: venous thrombosis in nine patients, arterial thrombosis in four, aborted rep etitions in three, and plateletopenia in eight. Six patients presented pulmonary thromboembolism, the cause of death in one patient. Thrombo penia was manifested in the evolutionary course of 14 patients, only o ne of which being severe. Two patients have died (one of them from neo plasia), and two others have had antiphospholipid antibodies become ne gative. Seven patients remain without treatment, the others undergoing anti-aggregating or anti-coagulant treatment. Conclusions. Plateletop enia, alone or accompanied by other symptoms, is the most common alter ation of PAPLS in our area, followed by venous thrombosis which caused death by pulmonary thromboembolism in one patient. Arterial thromboti c events and aborted repetitions are by no means infrequent.