Castleman Disease 12152010 Castlemans Disease Angiofollicular lymph node

Castleman Disease 12_15_2010

Castleman’s Disease • Angiofollicular lymph node hyperplasia – Lymphoproliferative disorder • characterized by nodal expansions that usually leave the structure of the underlying lymph node at least partially intact • linked to excessive release of interleukin IL-6 • May be an association with HHV-8

Unicentric Disease • isolated benign lymphoproliferative disorder of young adults that is unassociated with HHV-8 infection and generally curable with surgical resection • vast majority of patients are asymptomatic – Diagnosed incidentally on imaging • The median size of the lesion was 5 to 9 cm – 70 percent located in the mediastinum or hilum of the lung, with abdominal sites the next most frequent – Peripheral lymphadenopathy is unusual – laboratory abnormalities were seen in less than 25 percent • anemia, elevated sedimentation rate, hypergammaglobulinemia

Unicentric Disease • Treatment – Complete resection is curative – No reports of recurrence – Systemic symptoms resolve as well – Partially resected masses may remain asymptomatic for years • Radiation • Rituximab (case report)

Unicentric Disease • Complications – May have increased risk of lymphoma – Paraneoplastic pemphigus

Multicentric Disease • May be associated with HIV • Median age in 50’s, but may be earlier in HIV+ pts • Non-specific symptoms suggestive of inflammatory disease – Fever, night sweats, weight loss, fatigue – Less than 10% are asymptomatic

Multicentric Disease • peripheral lymphadenopathy • Hepato – splenomegaly in 50% of cases • anemia, hypoalbuminemia, hypergammaglobulinemia, and an elevated sedimentation rate • associated with increased levels of a variety of cytokines, most notably IL-10 and IL-6

Multicentric Disease • patterns of disease progression – Indolent – can persist for months to years without progression – episodic relapsing form may be aggressive for a short period and then remit spontaneously or in response to treatment, only to recur at a later time – rapidly progressive form that can lead to death within weeks may be more common in HIV+ patients

Multicentric Disease • Complications – Most pts die of fulminant infection, progressive disease or related malignancies • Kaposi sarcoma • Hodgkin and non-Hodgkin lymphoma

Multicentric Disease • Treatment – Steroids – 60 -70% response rate – Chemotherapy – Anti-viral agents – Inhibition of IL-6 – tocilizumab – Thalidomide – Rituximab