Systemic Reactive Amyloidosis Associated with Castleman's Disease

Abstract

We report this case of secondary amyloidosis associated with Castleman's disease. A 51-year-old man presented with systemic symptoms of generalized weakness, fatigue, unintended weight loss, anorexia and progressively worsening abdominal distension. On examination he was found to have an indurated right-sided submandibular mass and tense ascites. He was found to have multiorgan dysfunction with deranged liver function tests and renal failure. Ascitic fluid analysis revealed evidence of spontaneous bacterial peritonitis. Biopsy of the submandibular mass revealed angiofollicular lymph node hyperplasia consistent with a diagnosis of Castleman's disease. A subsequent liver biopsy showed extensive deposition of amyloid protein. Bone marrow biopsy also showed the presence of amyloid and increased kappa light chain-restricted plasma cells. The patient was not considered a candidate for chemotherapy or solid organ transplantation in view of active sepsis and poor physical condition. Secondary systemic amyloidosis complicating Castleman's disease is very rare. Untreated secondary systemic amyloidosis often has a rapidly fatal course, such as seen in our patient.

Castleman disease. A report of six cases.

“…The rarity of the disease also influences the therapeutic approach, no unique therapeutic regimens being unanimously accepted [ 3 , 9 , 10 , 11 , 12 ]. Disease associations include the POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin changes) [ 13 , 14 ], Kaposi sarcoma, pemphigus, refractory anemia, nephrotic syndrome, amyloidosis [ 15 , 16 , 17 ]. Associations with lymphoid malignancies have been described and these include: diffuse large- B cell lymphoma, mantle cell lymphoma, peripheral T-cell lymphoma and lymphoplasmacytic lymphoma, follicular lymphoma [ 18 ].…”

Amyloidosis secondary to intrapulmonary Castleman disease mimicking pulmonary hyalinizing granuloma-like clinical features

“…The relationship between CD and secondary amyloidosis was reported before, and most cases revealed amyloid deposits in kidney. [6–9] Secondary amyloidosis is believed to be caused mainly by chronic inflammation, and large amounts of IL-6 produced in the germinal center of CD may enhance the production of precursor of amyloid. [10,11]…”

Synchronous Unicentric Castleman Disease and Inflammatory Hepatocellular Adenoma: a Case Report

“…9,10 More than 50 reports of amyloidosis associated with CD have been published thus far in the medical scientific literature including hepatic amyloid deposition. [11][12][13][14][15] Of note, Morita-Hoshi, et al 11 reviewed 45 cases of systemic amyloidosis related to CD and showed that:…”