Dimitrios Mantas1, Christos Damaskos1, Panagiota Dailiani2, Michael Samarkos3, Penelope Korkolopoulou2. 1. a 2nd Department of Propedeutic Surgery , Athens Medical School, National and Kapodistrian University of Athens , Goudi, Athens , Greece. 2. b Department of Pathology , Athens Medical School, National and Kapodistrian University of Athens , Athens , Greece. 3. c 1st Department of Internal Medicine , Athens Medical School, National and Kapodistrian University of Athens , Athens , Greece.
Abstract
INTRODUCTION: Castleman's disease (CD), also known as giant or angiofolicular lymphoid hyperplasia or lymphoid hamartoma, is a group of atypical lymphoproliferative disorders that share common lymph node histological features and may be localized either to a single lymph node (unicentric) or occur systemically (multicentric). PATIENT AND METHOD: Herein, we present a rare case of a of 75-year-old female patient who was referred to our department and after a thorough work-up, underwent splenectomy with synchronous resection of an accessory spleen, splenic artery lymph nodes, and splenic hilar lymph nodes due to splenic involvement in a multicentric CD. RESULTS: The pathology of the specimens led to the conclusion that it was a case of polycentric HHV-8-positive CD, affecting the spleen, the accessory spleen, and the lymph nodes. CONCLUSIONS: Incidence of this rare condition is believed to be approximately 0.001-0.05%. CD has been linked to the human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and is associated with malignancies. The pathogenesis mechanism is considered to be a dysregulation and hypersecretion of cytokines, either idiopathic or secondary to a viral infection, with the latter considered the most frequent. Solid organ involvement is very rare as is splenic involvement.
INTRODUCTION:Castleman's disease (CD), also known as giant or angiofolicular lymphoid hyperplasia or lymphoid hamartoma, is a group of atypical lymphoproliferative disorders that share common lymph node histological features and may be localized either to a single lymph node (unicentric) or occur systemically (multicentric). PATIENT AND METHOD: Herein, we present a rare case of a of 75-year-old female patient who was referred to our department and after a thorough work-up, underwent splenectomy with synchronous resection of an accessory spleen, splenic artery lymph nodes, and splenic hilar lymph nodes due to splenic involvement in a multicentric CD. RESULTS: The pathology of the specimens led to the conclusion that it was a case of polycentric HHV-8-positive CD, affecting the spleen, the accessory spleen, and the lymph nodes. CONCLUSIONS: Incidence of this rare condition is believed to be approximately 0.001-0.05%. CD has been linked to the human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and is associated with malignancies. The pathogenesis mechanism is considered to be a dysregulation and hypersecretion of cytokines, either idiopathic or secondary to a viral infection, with the latter considered the most frequent. Solid organ involvement is very rare as is splenic involvement.
Authors: Paula Fernández-Rodríguez; Rosa Fernández López; Irene Acevedo Báñez; Javier Mohigefer Barrera; José Manuel Jiménez-Hoyuela García Journal: Eur J Nucl Med Mol Imaging Date: 2019-09-07 Impact factor: 9.236