Unicentric Castleman’s disease associated with end stage renal disease caused by amyloidosis

Abstract

Castleman’s disease (CD), also known as angiofolicular lymph node hyperplasia, is a rare heterogenous group of lymphoproliferative disorders. Histologically, it can be classified as hyaline vascular type, plasma cell type, or mixed type. Clinically two different subtypes of the CD are present: Unicentric and multicentric. Unicentric CD is generally asymptomatic and associated with hyaline vascular type, and its diagnoses depend on the localized lymphadenopathy on examination or imaging studies. However, multicentric CD presents with generalized lymphadenopathy and systemic symptoms including malaise, fever, night sweats, weight loss, and it is associated with the plasma cell type and mix type. Herein, we report a patient with unicentric CD of the plasma cell type without systemic symptoms, who developed end stage renal failure caused by amyloidosis 6 years after onset of CD.

Keywords: Castleman’s disease; Amyloidosis; Plasma cell; Inflammation; End stage renal disease

Core tip: Castleman’s disease (CD), also known as angiofolicular lymph node hyperplasia, is a heterogeneous group of lymphoproliferative disorders. The clinically unicentric form is generally asymptomatic and often associated with hyaline vascular type. The unicentric form of the disease often shows mild to moderate clinical prognosis, however the multicentric form is a more severe form. After the complete surgical removal of the lymph node, remission is achieved in many cases and complications are very rare. However, this case of unicentric CD of the plasma cell type is unique due to the fact that it presented with amyloidosis and end stage renal disease six years after the onset of the disease.