Systemic Reactive Amyloidosis Associated with Castleman's Disease
A 51-year-old African-American man who had been born and raised in the United States first presented to the medical clinic with complaints of generalized weakness, fatigue, unintended weight loss, anorexia and progressively worsening abdominal distension of 3 months duration. His medical comorbidities included essential hypertension and gout. Physical examination at the time was significant for a firm, indurated right-sided submandibular mass, hepatomegaly and mild epigastric tenderness.\nAn initial set of laboratory studies showed abnormal liver enzymes (alanine transaminase of 61 IU/l, aspartate transaminase of 83 IU/l, markedly elevated alkaline phosphatase of 1,003 IU/l and gamma-glutamyl transferase of 1,879 IU/l with normal bilirubin levels). Additional work-up done for evaluation of abnormal liver enzymes including viral hepatitis panels (hepatitis A, B and C), anti-nuclear antibody, anti-smooth muscle antibody, anti-liver kidney microsomal antibodies and anti-mitochondrial antibody were negative. A computed tomography scan of the abdomen showed hepatomegaly (18 cm).\nThe patient underwent biopsy of the submandibular mass that revealed features of Castleman's disease (fig. ). A subsequent liver biopsy revealed perisinusoidal deposition of eosinophilic, amorphous material within the extracellular matrix with hepatocyte atrophy, consistent with hepatic amyloidosis (fig. ). Bone marrow biopsy revealed diffuse extracellular eosinophilic, amorphous material consistent with amyloidosis with increased kappa light chain-restricted plasma cell count (6% of hematopoietic bone marrow cells) (fig. ). The patient underwent a colonoscopy that revealed no gross mucosal lesions; biopsies were unremarkable. A definitive diagnosis of secondary (AA) reactive amyloidosis with hepatic involvement was made.\nThe patient had subsequent hospitalized with new-onset, rapidly progressing tense ascites. He underwent large-volume paracentesis. Ascitic fluid analysis revealed an elevated serum-ascites albumin gradient of 1.7 and very low protein of 0.9 g/dl. Cell count analysis was consistent with spontaneous bacterial peritonitis. He was treated with intravenous antibiotics. A computed tomography scan of the abdomen done at the time revealed dilated loops of small bowel consistent with small bowel obstruction, which resolved with conservative management. He was not considered for chemotherapy in view of active infection and was not a liver transplant candidate because of his poor physical condition. The patient was discharged to be readmitted only 2 weeks later with recurrent ascites from decompensated liver disease. He was found to have worsening jaundice. His clinical condition rapidly deteriorated with superimposed severe metabolic acidosis resulting from acute renal dysfunction. He ultimately expired from multiorgan failure.