Journal of Surgery
Volume 7, Issue 4, August 2019, Pages: 96-100
Received: May 6, 2019;
Accepted: Jun. 5, 2019;
Published: Jul. 11, 2019
Views 240 Downloads 43
Biying Huang, Department of Surgery, Karolinska Institute, Stockholm, Sweden
Ioannis Gkekas, Department of Surgery, Karolinska Institute, Stockholm, Sweden
Ernesto Sparellid, Department of Surgery, Karolinska Institute, Stockholm, Sweden
Liver rupture with severe liver bleeding in patients with amyloidosis is an unusual but generally difficult to deal with and potentially fatal complication in these patients. Even patients with known manifestations to the lever can present with acute lever bleeding. Here we present a case report of a 62-year-old male with systemic immunoglobulin light chain (AL) amyloidosis, known manifestations to the lever and spontaneous liver bleeding which was treated conservatively without the need of surgical intervention. The etiology, panorama of clinical manifestations of hepatic amyloidosis and the management of liver bleeding in association with this unusual disease are reviewed. 18 cases of hepatic rupture and haemorrhage in association with amyloidosis in previously published cases are summarized. The choice of management should be individualized and the patient´s hemodynamic status and extent of liver bleeding are important factors to be taken into consideration. In all patients it is crucial to exclude underlying malignancy as the reason of bleeding. The patient´s hemodynamic status and the extent of liver bleeding are the most important factors to be taken into consideration in order to establish individualized management plan. Increased awareness of severe liver bleeding from amyloidosis may achieve reduced mortality of this potentially fatal complication.
Amyloidosis and Spontaneous Liver Bleeding: A Case Report and Literature Review, Journal of Surgery.
Vol. 7, No. 4,
2019, pp. 96-100.
Syed U, Ching Companioni RA, Alkhawam H, Walfish A. Amyloidosis of the gastrointestinal tract and the liver: clinical context, diagnosis and management. Eur J Gastroenterol Hepatol. 2016; 28 (10): 1109-21.
Mahmood S, Bridoux F, Venner CP, Sachchithanantham S, Gilbertson JA, Rowczenio D, et al. Natural history and outcomes in localised immunoglobulin light-chain amyloidosis: a long-term observational study. Lancet Haematol. 2015; 2 (6): e241-50.
Madsen LG, Gimsing P, Schiødt FV. Primary (AL) amyloidosis with gastrointestinal involvement. Scand J Gastroenterol. 2009; 44 (6): 708-11.
Gertz MA, Kyle RA. Hepatic amyloidosis: clinical appraisal in 77 patients. Hepatology. 1997; 25 (1): 118-21.
Mumford AD, O'Donnell J, Gillmore JD, Manning RA, Hawkins PN, Laffan M. Bleeding symptoms and coagulation abnormalities in 337 patients with AL-amyloidosis. Br J Haematol. 2000; 110 (2): 454-60.
Loss M, Ng WS, Karim RZ, Strasser SI, Koorey DJ, Gallagher PJ, et al. Hereditary lysozyme amyloidosis: spontaneous hepatic rupture (15 years apart) in mother and daughter. role of emergency liver transplantation. Liver Transpl. 2006; 12 (7): 1152-5.
Lehmann TG, Scheble V, Miller S, Heininger A, Fend F, Koenigsrainer A. Spontaneous hepatic rupture in amyloidosis - report of a case. Z Gastroenterol. 2012; 50 (12): 1296-301.
Scott PP, Scott WW, Siegelman SS. Amyloidosis: an overview. Semin Roentgenol. 1986; 21 (2): 103-12.
Park MA, Mueller PS, Kyle RA, Larson DR, Plevak MF, Gertz MA. Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients. Medicine (Baltimore). 2003; 82 (5): 291-8.
Serra L, Poppi MC, Criscuolo M, Zandomeneghi R. Primary systemic amyloidosis with giant hepatomegaly and portal hypertension: a case report and a review of the literature. Ital J Gastroenterol. 1993; 25 (8): 435-8.
Zeijen RN, Sels JP, Flendrig JA, Arends JW. Portal hypertension and intrahepatic cholestasis in hepatic amyloidosis. Neth J Med. 1991; 38 (5-6): 257-61.
Peters RA, Koukoulis G, Gimson A, Portmann B, Westaby D, Williams R. Primary amyloidosis and severe intrahepatic cholestatic jaundice. Gut. 1994; 35 (9): 1322-5.
Kim SH, Han JK, Lee KH, Won HJ, Kim KW, Kim JS, et al. Abdominal amyloidosis: spectrum of radiological findings. Clin Radiol. 2003; 58 (8): 610-20.
Röllinghoff W, Braun HJ, Schad FJ. [Primary amyloidosis presenting as intrahepatic cholestasis (author's transl)]. Dtsch Med Wochenschr. 1976; 101 (50): 1838-41.
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 (6): 844-5.
Levy-Lahad E, Steiner-Salz D, Berkman N, Chisin R, Levensart P, Leitersdorf E. Reversible functional asplenia and subcapsular liver hematoma--two distinctive manifestations of amyloidosis. Klin Wochenschr. 1987; 65 (22): 1104-7.
Ades CJ, Strutton GM, Walker NI, Furnival CM, Whiting G. Spontaneous rupture of the liver associated with amyloidosis. J Clin Gastroenterol. 1989; 11 (1): 85-7.
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 (1): 151-2.
Ooi LL, Lynch SV, Graham DA, Strong RW. Spontaneous liver rupture in amyloidosis. Surgery. 1996; 120 (1): 117-9.
Satue JA, Ortuño T, Carabias E, Cisneros C, Araque A, Fernandez Zatarain G, et al. Fatal spontaneous liver rupture in a renal transplant patient with amyloidosis. Nephron. 1996; 73 (2): 355-6.
Bujanda L, Beguiristain A, Alberdi F, Cosme A, Ruíz de la Hermosa J, Gutiérrez-Stampa, et al. Spontaneous rupture of the liver in amyloidosis. Am J Gastroenterol. 1997; 92 (8): 1385-6.
Kacem C, Helali K, Puisieux F. Recurrent spontaneous hepatic rupture in primary hepatic amyloidosis. Ann Intern Med. 1998; 129 (4): 339.
Mukhopadhya A, Raghuram L, Justus A, Joseph AJ, Eapen CE, Chandy GM. Transcatheter hepatic artery embolization for spontaneous rupture of amyloid liver. Indian J Gastroenterol. 2004; 23 (1): 26-7.
Naito KS, Ichiyama T, Kawakami S, Kadoya M, Tabata T, Matsuda M, et al. AL amyloidosis with spontaneous hepatic rupture: successful treatment by transcatheter hepatic artery embolization. Amyloid. 2008; 15 (2): 137-9.
Tam M, Seldin DC, Forbes BM, Connors LH, Skinner M, Oran B, et al. Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: a case report with literature review. Amyloid. 2009; 16 (2): 103-7.
Szturz P, Kyclová J, Moulis M, Navrátil M, Adam Z, Vaníček J, et al. Extensive Al amyloidosis presenting with recurrent liver hemorrhage and hemoperitoneum: case report and literature review. Klin Onkol. 2013; 26 (1): 49-52.
Mousa AY, Abu-Halimah S, Alhalbouni S, Hass SM, Yang C, Gill G, et al. Amyloidosis and spontaneous hepatic bleeding, transcatheter therapy for hepatic parenchymal bleeding with massive intraperitoneal hemorrhage: a case report and review of the literature. Vascular. 2014; 22 (5): 356-60.
Suda T, Oguri H. A case of primary amyloidosis with spontaneous hepatic rupture. Nihon Shokakibyo Gakkai Zasshi. 2017; 114 (6): 1039-45.
Lohana AK, Abid S, Sayani R, Tahir U, Shaikh PA. Hepatobiliary and Pancreatic: Bleeding in amyloid liver: A rare presentation of primary liver amyloidosis. J Gastroenterol Hepatol. 2019.