T. Barbui et al., THROMBOSIS AND THROMBOCYTOPENIA IN ANTIPHOSPHOLIPID SYNDROME (IDIOPATHIC AND SECONDARY TO SLE) - 1ST REPORT FROM THE ITALIAN REGISTRY, Haematologica, 78(5), 1993, pp. 313-318
Background. Since 1989 we have been promoting an Italian Registry of p
atients with APA (IR-APA), and we report here the analysis on 319 case
s (80 males and 239 females, aged from 2 to 76 years, median 31 years)
from 22 institutions. Methods. A two-page questionnaire was prepared,
divided into 4 parts: 1. personal data, 2. associated conditions, 3.
biology (for the diagnosis of APA), 4. history of thromboembolism, hae
morrhage and pregnancy. Every information was entered into a computeri
zed registry. Results. The IR-APA included 207 cases of idiopathic APA
and 112 cases of APA secondary to SLE. Seventyone out 207 (34.3%) and
47 out of 112 (42%) cases experienced arterial and/or venous thrombos
is, respectively (not significant). Among patients with a thrombotic h
istory, venous thrombosis were experienced by 60.5% vs 57.4%, arterial
thrombosis by 26.7% vs 31.9%. Venous thrombosis of the legs and arter
ial thrombosis of the cerebral vessels were the most common types of t
hrombosis in both groups. Thrombocytopenia was present in 27.7% of idi
opathic APA vs 22.4% of secondary APA (not significant). Four patients
experienced major haemorrhagic events. Conclusions. This retrospectiv
e study shows that the prevalence of thrombosis and thrombocytopenia i
n patients with idiopathic APA is similar to that observed in patients
with APA secondary to SLE.