Περίληψη:
Splenomegaly is a common finding in beta-thalassemia; however, its
hemodynamic features and its potential correlations with high output
state and hepatic disorders, both also frequent in thalassemia, have not
yet been assessed in these patients. Eight beta-thalassemia patients
with the indication for splenectomy and no symptoms or signs of heart
disease, aged 25.6 +/- 5.5 years, were studied. Preoperative assessment
included hematological profile, liver biology, hepatitis virus serology,
and echocardiography. During splenectomy, splenic artery blood flow and
splenic vein pressure were directly measured and liver biopsies were
taken. Preoperative echocardiographic data were compared with those of
34 healthy controls. The preoperative cardiac index was significantly
elevated in patients ( 4.8 +/- 1.3 vs 3.4 +/- 1.1 l/min per m(2) in
controls, p< 0.001). Splenic blood flow, although increased, was not
particularly high, being 285 +/- 56 ml/min or 0.13 +/- 0.04 ml/min per g
of splenic mass, representing 4.1 +/- 0.9% of total cardiac output (
CO). Splenic vein pressure was considerably elevated (29.7 +/- 5.5
cmH(2)O). Hepatic fibrosis, iron deposition, and extramedullary foci
were found in all eight biopsies. Serology was positive in five of eight
cases. beta-thalassemia patients with extensive splenomegaly requiring
splenectomy are characterized by high output state, increased splenic
blood flow, which probably makes a limited contribution to CO elevation,
and portal hypertension, manifest by increased splenic vein pressure and
hepatic histopathological abnormalities.
Συγγραφείς:
Aessopos, A
Farmakis, D
Tsironi, M
Deftereos, S and
Tassiopoulos, S
Konstantopoulos, K
Rombos, J
Papalambros, E
