Oligocentric Castleman Disease (OligoCD): A Novel Diagnostic Entity in the Spectrum of Castleman Disease.

Castleman disease (CD) is a rare lymphoproliferative disorder traditionally classified as unicentric (UCD) or multicentric (MCD). However, some cases exhibit features that do not fit either category. These atypical cases warrant recognition as a distinct subtype, proposed here as oligocentric Castleman disease (OligoCD). A 38-year-old woman presented with bilateral lacrimal gland swelling and cervical lymphadenopathy, fatigue, and low-grade fever. Laboratory investigations, including hemogram, liver and kidney function tests, inflammatory markers, and interleukin-6 (IL-6), were within normal limits, except for mildly elevated erythrocyte sedimentation rate (ESR) and immunoglobulin G4 (IgG4) levels, elevated to approximately twice the upper limit of normal. Imaging did not reveal any other lymphadenopathy or systemic involvement. Lymph node biopsy revealed features of the hyaline-vascular variant of Castleman disease, confirmed on immunohistochemistry. The differential diagnosis of IgG4-related disease (RD) was considered but ruled out due to the non-fulfillment of the clinical diagnostic criteria and the absence of plasma cell infiltrate on histopathological examination. The presentation, however, did not fulfil the criteria for either UCD or MCD. She had an excellent response to prednisolone initially and a complete response to rituximab. This case underscores the need to broaden the existing diagnostic framework for Castleman disease. The clinical and histopathological findings favor recognizing oligocentric Castleman disease as a novel intermediate subtype within the disease spectrum.