Castleman, disease

(Lymphoid angiofollicular hyperplasia)

Very rare. Non-cancerous lymphoproliferative syndrome of unknown origin. In adult patients, it is mainly observed in a context of HIV infection or immunosuppression. A link with infection by human herpes 8 virus  appears to be established: this virus is localized in the immunoblasts and in the CD20 + B lymphocytes in the lymph nodes. In children, the physiopathogeny is different: it implies a disorder of innate immunology possibly by an infectious factor.


There are two forms:

-         localized (75 % in children versus 20 % in adults): slowly growing tumoral mass (mediastinum or mesentery), isolated, more often associated with fever and systemic inflammatory signs in children than in adults.

-         multifocal: lymphadenopathy, splenomegaly, fever; in adults, in 25 % of cases, a  POEMS syndrome (see this term) [acronym for Peripheral neuropathy (P), paraneoplastic manifestations including the Organomegaly (O), Endocrinopathy (E),  abnormal Monoclonal plasma cells (M), alterations in Skin (skinS )] will appear later. A few cases of Kaposi sarcoma or lymphoma have been reported.


Diagnosis: angiofollicular hyperplasia is present at the ganglionic biopsy.


-        either of hyaline vascular type (78 % of the pediatric isolated forms and 68 % of the adult isolated forms): abnormal germinal centers invaded by hyalinized vessels and associated with abnormal dendritic follicular cells;

-        either of plasmocytic type: normal or enlarged germinal centers with plasmocytic interfollicular infiltration;

-        either of mixed type (those two last types are present in about 80 % of the multicentric pediatric and adult forms)


and immunochemistry shows a prevalence of lymphocytes B CD20+.


Treatment: surgery for localized forms, systemic treatment (tocilizumab, anakinra, rituximab, IV immunoglobins, corticoids) in the multicentric forms.


In patients under immunosuppression following solid organ transplantation, Castleman disease is a possible complication of human herpes 8 virus: the virus can be transmitted with the transplanted organ or the infection occurs in the context of immunosuppression. Treatment then consists in the reduction of immunosuppression (switching to sirolimus could be an alternative) associated with an antiviral treatment (ganciclovir); surgery or radiotherapy are rarely necessary.

Diagnosis: lymph node biopsy that shows  lymphoid hyperplasia, and immunohistochemistry which shows a predominance of CD20 + B lymphocytes.


Anesthetic implications: 

immunosuppression, drugs interactions with medical treatment. Be cautious if a mediastinal mass is present.


References : 

-         Bonatti HJR, Hunter EB, Lott SL, Frangoul H, Gillis L, Correa H, Kelly B. 
Castleman disease in a pediatric liver transplant recipient : a case report and literature review.
Pediatr Transplantation 2012; 16: e229-34.  

-        Borocco C, Ballot-Schmit C, Ackermann O, Aladjidi N, Delaleu J et al.
The French paediatric cohort of Castleman disease : a retrospective report of 23 patients.
Orphanet J Rare Dis 2020 ; 15 :95.


Updated: May 2020