PB0208 - Tittle: Spleen rupture in Afibrinogenemia: hemorrhagic or thrombotic

Background: Spleen rupture is described as a rare hemorrhagic manifestation of afibrinogenemia. Pathogenesis and best management are still not well defined.

Aims: We report the clinical characteristics and the management of spleen rupture in the Lebanese cohort with afibrinogenemia

Methods: It is a retrospective observational descriptive study in a well-defined cohort, “the Lebanese patient with afibrinogenemia”, during a follow up period of 21 years.
Variables studied were sex, age, gene mutation, bleeding score and thrombotic event, occurrence of spleen rupture, radiological finding of spleen, management of spleen rupture, and outcome.
All patients provided written informed consent and university ethic committee give his approval

Results: 17 (23%) out of 73 patients had spontaneous spleen rupture with no obvious trigger factor. There was 5/31 (16%) women and 12/42 (28.5%) men. Mean age of occurrence was 16.3 years [6;42]. Hom FGG R134X and hom FGA L212X were the 2 gene mutation. Mean bleeding score was 22.7 [12;34]. Ten had previous history of thromboembolic event while 17 out of the 73 in the studied population
Presenting symptoms were sudden abdominal pain, hypotension and hemoperitoneum.
Abdominal ultrasound and CT scan identifies patches of spleen parenchyma necrosis with hemoperitoneum
Splenectomy was performed in 11 patients, 4 had conservative treatment. Two died from hemorrhagic shock before any treatment.
Splenectomy was complicated in 4 patients by a portal thrombosis. The four patients were treated with Low-Molecular-Weight–Heparin with no recanalization.
Fibrinogen substitution was given as conservative treatment for 1 to 3 weeks than a prophylaxis regimen every 15 days. 2 patient received Low-Molecular-Weight-Heparin. Spleen returned to normal in one to 3 weeks. Recurrence was observed in one patient who stopped prophylaxis with a follow up of 3 years

Conclusion(s): Spleen rupture is significantly associated with thromboembolic events (p < 0,001) and could be an expression of thromboembolic event. Conservative treatment is effective.