Case Reference No. CC-0298-08 A 39 year old African American male presents to an outside hospital complaining of left upper quadrant pain. His history included minimal trauma playing basketball. Physical exam showed a diffusely tender abdomen with mild distension. CT showed a subcapsular hematoma. The patient continued to bleed, dropped his hemoglobin from 13 to 10 g/dl, and was transferred to our institution for further management. Subsequent CT showed an increase in the size of the hematoma and massive hemoperitoneum. The patient's hemoglobin level continued to fall. Visceral angiography was performed to discern the source of the bleeding. The selective hepatic arteriogram (Fig 1) suggested the presence of multiple microaneurysms within the liver. An accessory right hepatic artery, originating from the superior mesenteric artery, revealed similar angiographic findings. No extravasation was seen. The diagnosis of polyarteritis nodosa was considered, though renal arteriograms failed to demonstrate any renal artery microaneurysms. Our differential diagnoses included other vasculitides such as Wegener's granulomatosis and systemic lupus erythematosus, aneurysms of infectious origin, and methamphetamine abuse. We elected not to empirically embolize the hepatic artery. Despite conservative treatment, the patient's condition continued to deteriorate, and he was taken to the operating room for evacuation of the subcapsular hematoma and intraoperative liver biopsy. On histologic exam, the hepatic sinusoids were expanded by lightly eosinophilic hyaline material which compressed the hepatocytes (Fig 2). Similar material was present in the walls of vessels and in the portal tracts. The hyaline material was diffusely but weakly positive on congo red stain and showed apple green birifringence under polarized light confirming the presence of amyloid (Fig 3). Immunohistochemistry for amyloid P component was strongly positive in the same locations (Fig 4). P component is present in low concentrations in all types of amyloid. Additional immunohistochemical studies were performed in an attempt to further type the amyloid. A stain for AA protein which is found in secondary amyloid was negative. Likewise, a stain for pre-albumin which can be seen in systemic senile amyloid was also negative. Immunohistochemistry for kappa and lambda light chains were also performed. These showed a staining restriction for kappa light chain. While AL amyloid usually shows lambda light chain restriction, this unidirectional staining raises the possibility of dysproteinemia associated amyloid deposition. Review of the patient's other radiographs demonstrated lytic bony lesions (Fig 5), strongly suggesting that this is a case of secondary amyloidosis related to multiple myeloma. Several cases of spontaneous hepatic rupture associated with amyloidosis have been reported in the medical literature. Thus far, there have been no reports linking hepatic microaneurysms and amyloidosis. Question 1) Would anyone have empirically embolized the left hepatic, the right hepatic, or the accessory right hepatic arteries, given no angiographic evidence of active extravasation? If so, with what agent?		Fig. 1 Selective hepatic arteriogram shows microaneurysms in the periphery of the liver parenchyma. The subcapsular hematoma compresses the edge of the liver magin. Fig. 2. Liver biopsy. Hyaline material within the sinusoids is compressing the hepatocytes. (hematoxylin and eosin, orig mag 100 X).   Fig. 3. Liver biopsy. A Congo Red stain of a vessel showing positive congophilia of the wall (orig mag 100X). This congophilia did show apple green birefringence under polarized light (not shown).   Fig. 4. Liver biopsy. Immunohistochemical stain for amyloid P component shows a brown label in the same sinusoidal location as the hyaline matrix material (orig mag 100X). Amyloid P component is present in all form of amyloid. Fig. 5. Chest X-ray shows lytic lesions in the left clavicle consistent with a diagnosis of multiple myeloma.
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