Recent Advances in Castleman Disease.

BACKGROUND

Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric CD (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric CD (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpesvirus-8 (HHV-8 + MCD).

SUMMARY

During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD.

KEY MESSAGES

We herein summarize recent advances in diagnosis, treatment, and novel insights into the pathogenesis of this disease.