LETTER
Recurrent Spontaneous Hepatic Rupture in Primary Hepatic Amyloidosis
Chaftik Kacem, MD;
Kamel Helali, MD; and
Francois Puisieux, MD
15 August 1998 | Volume 129 Issue 4 | Page 339
TO THE EDITOR:
We describe a 46-year-old woman who presented with a 2-year history of abdominal discomfort with recurrent extremity ecchymosis and 3 days of increased pain. Upon examination, her abdomen was distended with generalized guarding. Emergency laparotomy revealed hepatomegaly and a massive hemoperitoneum (1500 mL) caused be a partially ruptured subcapsular hematoma (diameter, 15 cm) involving the upper face of the left side of the liver. The hematoma was evacuated, and hemostasis was achieved with local coagulation. Surgical liver biopsy showed portal tracts containing intense amorphous infiltration of hyaline material confirmed to be amyloid by Congo red staining. Immunohistochemical studies yielded negative results. Coagulation studies revealed no coagulation defect. A complete evaluation showed no evidence of an underlying chronic disease. The patient was discharged 2 weeks after surgery with a diagnosis of primary hepatic amyloidosis and was being treated by colchicine.
Two months later, recurrent abdominal pain developed. Computed tomography of the abdomen showed a recurrent subcapsular hematoma on the lower face of the left side of the liver with an effusion around the spleen. We treated the patient without surgery, substituting cyclic melphalan and prednisone. Two subsequent computed tomographic scans showed progressive reduction of the hematoma. Eight months later, the patient is well.
Hemorrhage is a recognized but uncommon complication of hepatic amyloidosis; it primarily occurs after biopsy. Only seven other cases of spontaneous rupture have been reported [1-5]. Coagulation abnormalities, including factor X deficiency and vascular infiltration, could favor the occurrence of bleeding in patients with amyloidosis. Hepatic rupture is an ominous event. In all but one previously reported case, death occurred within hours or days [1-5]. Emergency liver transplantation was performed in the surviving patient [5]. Our case is unique because of the favorable outcome.
|
Author and Article Information
|
|---|
CHU Charles Nicolle; Tunis, Tunisia
Hopital les Bateliers; Lille, France
1. Hurd WW, Katholi RE. Acquired functional asplenia: association with spontaneous rupture of the spleen and fatal spontaneous rupture of the liver in amyloidosis. Arch Intern Med. 1980; 140:844-5.
2. Okazaki K, Moriyasu F, Shiomura T, Yamamoto T, Suzaki T, Kanematsu Y, et al. Spontaneous rupture of the spleen and liver in amyloidosis-a case report and review of the literature. Gastroenterol Jpn. 1986; 21:518-24.
3. Ades CJ, Strutton GM, Walker NI, Furnival CM, Whiting G. Spontaneous rupture of the liver associated with amyloidosis. J Clin Gastroenterol. 1989; 11:85-7.
4. Gastineau DA, Gertz MA, Rosen CB, Kyle RA. Computed tomography for diagnosis of hepatic rupture in primary systemic amyloidosis. Am J Hematol. 1991; 37:194-6.
5. Harrison RF, Hawkins PN, Roche WR, MacMahon RF, Hubscher SG, Buckels JA. "Fragile" liver and massive hepatic haemorrhage due to hereditary amyloidosis. Gut. 1996; 38:151-2.
About Letters
The Editors welcome submissions for possible publication in the Letters section. Authors of letters should:
•Include no more than 300 words of text, three authors, and five references
•Type with double-spacing
•Send three copies of the letter, an authors' form signed by all authors, and a cover letter describing any conflicts of interest related to the contents of the letter.
Letters commenting on an Annals article will be considered if they are received within 6 weeks of the time the article was published. Only some of the letters received can be published. Published letters are edited and may be shortened; tables and figures are included only selectively. Authors will be notified that the letter has been received. If the letter is selected for publication, the author will be notified about 3 weeks before the publication date. Unpublished letters cannot be returned.
Annals welcomes electronically submitted letters.
Top
Author & Article Info
References
Article
