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Published on: August 30, 2019
Atraumatic splenic rupture in amyloidosis.
Pietro Renzulli1, Alain Schoepfer, Esther Mueller
1Department of Visceral Surgery and Medicine, Inselspital, Bern University Hospital, andUniversity of Bern, Switzerland. pietro.renzulli@insel.ch
Summary
Atraumatic splenic rupture is a rare but serious complication of amyloidosis, often presenting as the first sign of the disease. This condition carries a high mortality rate, emphasizing the need for early recognition and management.
Area of Science:
- Hematology
- Gastroenterology
- Oncology
Background:
- Splenic involvement is common in amyloidosis (5-10%), but atraumatic splenic rupture is exceptionally rare.
- This study investigates the clinical presentation, management, and predisposing factors of atraumatic splenic rupture in amyloidosis.
Observation:
- A review of 31 cases, including a new case report, identified key clinical features and diagnostic methods.
- Predisposing factors such as easy skin bruisability and Factor X deficiency were noted in a subset of patients.
Findings:
- Atraumatic splenic rupture frequently occurs in undiagnosed amyloidosis (71%) and is associated with a high 30-day mortality (26%).
- Primary (AL) amyloidosis was the most common type (81%), with moderate splenomegaly and subcapsular hematomas being frequent findings.
- Autologous stem-cell transplantation (ASCT) for amyloidosis was linked to splenic rupture in 16% of cases.
Implications:
- Early recognition of atraumatic splenic rupture is crucial, especially in patients with undiagnosed amyloidosis.
- Coagulation abnormalities, splenomegaly, and ASCT should raise suspicion for splenic rupture in amyloidosis patients.
- This rare complication highlights the systemic impact of amyloidosis and the need for comprehensive patient monitoring.
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