Disease Overview

Castleman disease (CD) is a rare and complex lymphoproliferative disorder that can present as either unicentric, involving a single lymph node region, or multicentric, affecting multiple lymph node areas and leading to systematic inflammation. The condition can mimic both benign and malignant growths, commonly impacting the neck, chest, abdomen, and pelvis. CD is classified into distinct histological subtypes: hyaline vascular, plasma cell, mixed, and HHV-8-associated CD, the latter primarily seen in immunocompromised patients.

Diagnosis

Diagnosing Castleman disease (CD) can be particularly challenging due to its overlapping features with other lymphoproliferative, autoimmune, and infectious conditions. A precise diagnosis requires a combination of clinical evaluation, imaging, laboratory findings, and, most importantly, lymph node biopsy.

According to the international consensus diagnostic criteria for idiopathic multicentric Castleman disease (iMCD), diagnosis is based on two major criteria, at least two minor criteria (including at least one laboratory abnormality), and exclusion of mimicking diseases such as lymphoma, autoimmune disorders, or infectious etiologies.