Thromboembolic events in beta thalassemia major: an Italian multicenter study.
Thromboembolic (TE) events have been frequently reported in beta-thalassemic patients in association with known risk factors such as diabetes, complex cardiopulmonary abnormalities, hypothyroidism, liver function anomalies, and postsplenectomy thrombocytosis. In a recent survey involving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia major and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, respectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and hemiparesis. Other localizations were the pulmonary (3 patients), mesenteric (1 patient) and portal (2 patients) sites. There were 6 cases of deep venous thrombosis (2 in the upper limbs, 4 in the lower ones). Intracardiac thrombosis was found in 2 subjects and clinical and laboratory signs of DIC were observed in 2 others during pregnancy. Since our patients with TE events present a statistically significantly higher incidence of associated dysfunction (cardiomyopathy, diabetes, liver function anomalies, hypothyroidism) than those without TE events (50 vs. 13.8%), we suggest close monitoring of those patients who are at higher risk of developing TE events because of the presence of one or more of these predisposing factors.
Borgna Pignatti
C
C
Department of Pediatrics, University of Verona, Italy.
Carnelli
V
V
Caruso
V
V
Dore
F
F
De Mattia
D
D
Di Palma
A
A
Di Gregorio
F
F
Romeo
M A
MA
Longhi
R
R
Mangiagli
A
A
Melevendi
C
C
Pizzarelli
G
G
Musumeci
S
S
D000293:Adolescent; D000328:Adult; D001777:Blood Coagulation; D002648:Child; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic; D013923:Thromboembolism / Q000209:etiology*; D017086:beta-Thalassemia / Q000150:complications*