Review of the literature and identification of 31 patients, including our own case report, with atraumatic
splenic rupture in
amyloidosis. Analysis of the clinical presentation, the surgical management, the nomenclature and definition of predisposing factors of
splenic rupture.
RESULTS: We identified 15 women and 16 men (mean age 53.3 +/- 12.4 years; median 52, range: 27-82 years) with an atraumatic
splenic rupture. Easy skin bruisability and
factor X deficiency were detected in four (13%) and five patients (16%), respectively. The diagnosis of
splenic rupture was made either by computed tomography (n = 12), ultrasound (n = 5), exploratory
laparotomy (n = 9) or autopsy (n = 4). All patients underwent surgery (n = 27) or autopsy (n = 4).
Amyloidosis was previously diagnosed in nine patients (29%). In the remaining 22 patients (71%), the atraumatic
splenic rupture represented the initial manifestation of
amyloidosis. Twenty-five patients (81%) suffered from primary (AL) and four patients (13%) from secondary
amyloidosis (AA). In two patients, the type of
amyloidosis was not specified. A moderate
splenomegaly was a common feature (68%) and the characteristic intraoperative finding was an extended subcapsular
hematoma with a limited parenchymal
laceration (65%). In five patients with known
amyloidosis, the atraumatic
splenic rupture was closely associated with autologous
stem-cell transplantation (ASCT) (16%). Three patients were suffering from
multiple myeloma (10%). A biopsy-proven amyloidotic liver involvement was present in 14 patients (45%), which lead to atraumatic liver
rupture in two patients. The
splenic rupture related 30-day mortality was 26% (8/31).
CONCLUSIONS: