The Clinical Behavior of Localized and Multicentric Castleman Disease

Abstract

Localized and multicentric Castleman disease are different clinical disorders with overlapping histologic features. Localized disease can be cured with surgery, but complete remissions in patients with multicentric disease have been achieved only with chemotherapy or prednisone given at the time of diagnosis.

Hyaline-Vascular Type Castleman’s Disease, Sarcoidosis, and Crohns Disease

“…Castleman's disease (CD) is a lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with various pathologic features [1,2]. It is classified clinically as unicentric and multicentric types.…”

“…It is classified clinically as localized and multicentric types, and pathologically as hyaline-vascular and plasma cell type, and human herpes virus-8 (HHV-8)-associated plasmablastic multicentric Castleman's disease. The etiology remains unclear although immune dysregulation, genetic factors and infection may play a role in pathogenesis [1]. A similar pathophysiological picture is seen in sarcoidosis and Crohns disease.…”

Acute Interstitial Nephritis with Polyclonal B Cell Infiltration and Development of Mantle Cell Lymphoma.

“…Most patients with localized forms have no systemic symptoms (15,16,18,19). In the contrast, patients with multicentric forms are older and have systemic symptoms such as fatigue, fever, weight loss, night sweats, nausea, and physical abnormalities such as splenomegaly, hepatomegaly, edema, polyneuropathy and skin rash (15,16,(19)(20)(21)(22)(23), and laboratory abnormalities such as anemia, hypergammaglobulinemia, elevated IL-6, hypoalbuminemia, elevated erythrocyte sedimentation rate, proteinuria, elevated creatinine, elevated liver function tests and thrombocytopenia (16,(19)(20)(21)(22)(23). In this case, general fatigue before ARF, hepatomegaly and edema (after correction of dehydration) were observed.…”

“…Diagnosis is based on histologic characteristics of lymph nodes. Castleman's disease can be divided into three histologic subtypes (hyaline vascular type, plasma cell type and mixed type) (14) and two clinical types: localized Castleman' s disease and multicentric Castleman' s disease ( 15). Localized Castleman's disease usually occurs in young patients and presents with masses mainly in the abdomenand mediastinum (16)(17)(18).…”

Castleman’s Disease—A Rare Diagnosis in the Retroperitoneum

“…2). Follicles are surrounded by circumferentially arranged layer of small polyclonal B-lymphocytes, in an onion skin feature [4]. It may present with mass effect symptoms such as anorexia, vomiting, weight loss, urinary retention, abdominal or lumbar pain or a palpable mass.…”