Fulminant hepatitis in a patient with primary amyloidosis with a complete hematological response
DOI:
https://doi.org/10.32818/reccmi.a7n1a5Keywords:
immunoglobulin light-chain amyloidosis, amyloidosis, liver failure, nephrotic syndromeAbstract
Primary amyloidosis is a systemic disease that results from abnormal kappa or lambda protein misfolding. The prognosis is poor, and the treatment includes chemotherapy. Hepatic disorders related to primary amyloidosis are found in at least 70% of autopsies. They have a bad prognosis factor, but their clinical manifestations are rare.
We report a patient aged 41 with an initial diagnosis of nephrotic syndrome. Renal biopsy showed amyloid deposition. The treatment included quimiotherapy, reaching a complete hematological response. Even though the patient developed a hepatic failure dying.
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González de la Calle V, García-Sanz R, Mateos MV. Amiloidosis primaria. Med Clin (Barc) 2016; 147: 121-6. doi: https://doi.org/10.1016/j.medcli.2016.02.018.
Merlini G. AL amyloidosis: from molecular mechanisms to targeted therapies. Hematology Am Soc Hematol Educ Program. 2017 Dec 8; 2017(1): 1-12. doi: https://doi.org/10.1182/asheducation-2017.1.1.
Sattianayagam P, Gibbs S, Hawkins P, Gillmore J. Systemic AL (light-chain) amyloidosis of the gastrointestinal tract. Scand J Gastroenterol 2009; 44(11): 1384-5. doi: https://doi.org/10.3109/00365520903254296.
Palladini G, Hegenbart U, Milani P, Kimmich C, Foli A, Ho AD et al. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood 2014; 124(15): 2325-32. doi: https://doi.org/10.1182/blood-2014-04-570010.
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. doi: https://doi.org/10.1097/01.md.0000091183.93122.c7.
Norero B, Pérez-Ayuso RM, Duarte I, Ramirez P, Soza A, Arrese M et al. Portal hypertension and acute liver failure as uncommon manifestations of primary amyloidosis. Ann Hepatol. 2013 Jan-2014 Feb; 13(1): 142-9.
Hung HH, Huang DF, Tzeng CH, Su CH, Su TP, Chen HC et al. Systemic amyloidosis manifesting as a rare cause of hepatic failure. J Chin Med Assoc. 2010; 73(3): 161-5. doi: https://doi.org/10.1016/S1726-4901(10)70032-0.
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Copyright (c) 2022 Ileana Gefaell-Larrondo, David Roldán-Cortés, Juan Churruca-Sarasqueta, Ángel Torralba-Morón
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