Spontaneous rupture of amyloid spleen

ABSTRACT

Primary systemic amyloidosis [AL] is characterized by the overproduction of immunoglobulin light chain proteins by a monoclonal, terminally differentiated B-lymphocyte or plasma cell clone. The free immunoglobulin light chains are deposited in an abnormal conformation as amyloid in a variety of organs in the body [1]. Splenic involvement is seen in 4 -13% of cases, however amyloidosis causing spontaneous rupture is very rare with only 31 cases reported so far. We report a case of 60-year old, suffering from Primary AL amyloidosis and multiple myeloma involving the liver, spleen and GI tract, who suddenly collapsed and developed rapid abdominal distension. CT scan suggested rupture of the spleen. She underwent emergency Splenectomy and had an uneventful recovery. It has been suggested that the deposition of amyloid in the basement membrane of the vascular endothelium leads to increased gap between junctions and leading to vascular fragility and propensity to rupture. The amyloid can also lead to defective interaction with platelets and predispose to spontaneous haemorrhage. Thus in a case of occult haemorrhage with Amyloidosis, diagnosis of rupture of spleen should be considered and CT scan should be performed if the patient is haemodynamically stable. Splenectomy is the ideal treatment for ruptured spleen