|Year : 2021 | Volume
| Issue : 4 | Page : 463-464
Department of Pathology, Sri Devaraj Urs Medical College, Sri Devaraj Academy of Higher Education and Research, Kolar, Karnataka, India
|Date of Submission||06-Jun-2020|
|Date of Decision||24-Jun-2020|
|Date of Acceptance||03-Jul-2020|
|Date of Web Publication||17-Jun-2021|
Flat No. 5, Officers Quarters, Sri Devaraj Urs Medical College, Tamaka, Kolar, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Das S. Hepatic amyloidosis. Med J DY Patil Vidyapeeth 2021;14:463-4
Liver involvement by amyloidosis is not uncommon. The clinical presentation is usually subclinical or with mild elevation of liver enzymes. Liver failure and rupture have rarely been reported. We report a case that presented with new-onset ascites and rapidly progressed into liver failure and rupture with fatal outcome.
Liver biopsy still remains the definitive method of detecting liver involvement in amyloidosis. Three reported cases with clinically unsuspected primary amyloid light (AL) type of amyloidosis was diagnosed by liver biopsy. These cases demonstrate that primary amyloidosis can present with liver enzyme. Biopsy may be the initial diagnostic procedure of unsuspected primary amyloidosis. Very rare complications after liver biopsy can occur and include clinically significant intraperitoneal hemorrhage. In 1988, at the Mayo Clinic study, only two patients out of 80 had bleeding after liver biopsy.
The mean survival of patients with primary hepatic amyloidosis was 9–12 months. In 1986, Kyle and Greipp and, Gertz and Kyle established that the major risk factor affecting survival during the 1st year from diagnosis was the presence of congestive cardiac failure, followed by hepatomegaly, urinary light chains, and underlying myeloma.
Hepatic rupture in amyloidosis is extremely ominous; death occurs within hours to days. In review of literature, there are 13 cases of spontaneous hepatic rupture in liver amyloidosis, which were managed by different modalities ranging from conservative to liver transplant. Three reported cases in hepatic amyloidosis died shortly after hepatic rupture. Another two cases were managed successfully by conservative method. Hepatic artery embolization was successful in two patients, while surgical ligation of the hepatic artery was successful in another patient.
Currently, the amyloid associated/amyloid light chain ratio has been 1:17–1:38 due to fewer chronic infections and an increasing recognition of AL amyloidosis (amyloid light chain).
Hepatic amyloidosis is usually characterized by amyloid deposits in the liver parenchyma along the sinusoids within the spaces of Disse or within the blood vessel walls. As a result of extensive compression of hepatocytes by the amyloid deposits, there may be atrophy of hepatocyte. More massive infiltration results in enlarged liver with rubber elastic consistency. This results in “lardaceous liver” appearance on cut-surface.
The clinical spectrum of hepatic amyloidosis can range from hepatomegaly and borderline abnormal liver function test to more severe form, resulting in portal hypertension, hepatic failure, and rarely spontaneous rupture.
In another series, other frequent findings in cases of hepatic amyloidosis included proteinuria (88%), elevated serum alkaline phosphatase (86%), abnormal serum protein electrophoresis (monoclonal protein or hypogammaglobulinemia, 64%), hyposplenism on the peripheral blood smear (62%), defined by the presence of Howell–Jolly bodies, and hepatomegaly (81%) disproportional to the liver enzyme abnormalities. The median survival rate in these patients is 9 months.
Immunohistochemistry using anti-kappa and anti-lambda antibodies are useful in immunohistochemical classification and diagnosis of AL-type amyloidosis. However, it has its own limitations due to cross-reactivity between anti-kappa and anti-lambda antibodies. A study using antibodies against three different regions of immunoglobulin lambda light chain for the immunohistochemical analysis of liver biopsy samples from the cases of immunoglobulin lambda light chain amyloidosis showed that the amyloid deposits may not be homogeneous in the liver and that molecular heterogeneity of amyloid fibril protein or a difference in the mode of deposition results in the histopathological heterogeneity of AL amyloid deposits.
| References|| |
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Gertz MA, Kyle RA. Hepatic amyloidosis (primary [AL], immunoglobulin light chain): the natural history in 80 patients. Am J Med 1988;85:73-80.
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