Castleman Disease

Castleman disease (CD) is one of the idiopathic reactive lymphadenopathies, relatively rare clinically, characterized by marked deep or superficial lymphadenopathy; some cases have systemic symptoms and/or multi-system damage. Most cases do well after surgical excision of enlarged nodes. Its pathologic feature is varying degrees of lymphoid follicle, vascular and plasma-cell proliferation.

Common Symptoms

Keratitis; lymphadenopathy; hepatosplenomegaly; low-grade fever

Causes and Pathogenesis

(A) Etiology: CD cause is unknown; plasma-cell type may relate to infection/inflammation. Some authors propose immune dysregulation as the initiating factor; clinically 25% of unicentric cases have HHV-8 infection. At least some CD patients are at risk of malignant B-cell proliferation; a few multicentric cases transform to malignant lymphoma, but most do not on follow-up. (B) Pathogenesis: Because nodal follicles contain more than one vascular germinal center, some cases have angiolipoma components, and lesions occur at sites without normal lymphoid tissue, it was once considered a hamartoma; angiography resembles other vascular hamartomas. Plasma-cell-rich CD is thought related to infection (mainly viral) and inflammation - pathologically inflammatory (plasma cells, immunoblasts, capillary proliferation) while preserving residual nodal architecture; clinically inflammatory signs (anemia of chronic disease, elevated ESR, hypoalbuminemia, polyclonal hypergammaglobulinemia). Some propose immune dysregulation as initiating factor: e.g. AIDS (typical immunodeficiency) may coexist with CD and Kaposi sarcoma; a few CD cases transform to Kaposi sarcoma; some patients have autoimmune cytopenia, ANA/RF/Coombs positivity; immunology shows loss of antigen reactivity and absence of T-suppressor cells. Some consider CD a pre-neoplastic lesion: lesional plasma cells are monoclonal on immunohistochemistry, individual patients have monoclonal immunoglobulin in blood, and a few multicentric cases transform to malignant lymphoma. Many reports implicate IL-6: IL-6 gene transfer into murine hematopoietic stem cells successfully produces a CD-like model; CD nodal germinal-center B cells secrete large amounts of IL-6; after lesion resection, elevated serum IL-6 falls with clinical improvement. Animal experiments also confirm human herpesvirus 8 (HHV-8), i.e. Kaposi sarcoma-associated herpesvirus (KSHV), participates in CD pathogenesis.

Treatment

Drug therapy, rehabilitation

Preventive Measures

Daily prevention of Castleman disease. Prognosis: focal disease has good prognosis; multicentric disease with monoclonal hypergammaglobulinemia has poor prognosis with easy malignant transformation/lymphoma.

Department to Visit

It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.