The incidence of HIV associated Castleman’s disease Professor Mark Bower Chelsea & Westminster Hospital London, UK.

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INTRODUCTION OBJECTIVES METHODS RESULTS DISCUSSION

Presentation transcript:

The incidence of HIV associated Castleman’s disease Professor Mark Bower Chelsea & Westminster Hospital London, UK

Castleman’s disease

Synonyms Multicentric Angiofollicular Hyperplasia Angiofollicular Lymph Node Hyperplasia Angiomatous Lymphoid Hyperplasia Castleman Tumor Giant Benign Lymphoma Hamartoma of the Lymphatics Giant Lymph Node Hyperplasia

Castleman’s disease Hyaline vascularPlasmablastic Localised Multi-centricHIV-MCD

IgM light chain restricted (monotypic polyclonal)

HHV8 in plasmablasts in MCD

MCD incidence in CWH cohort ,997 patients 56,202 patient years follow-up

Overall incidence KS & MCD MCDKS Cases Incidence per 10,000 PY (95%CI) 4.3 ( ) 210 ( )

MCD incidence multivariate analysis Unlike KS, incidence not related to: Gender Prior AIDS diagnosis

MCD incidence multivariate analysis MCD risk associated with: Increasing age Non-Caucasian ethnicity Shorter duration HIV positive Higher CD4 nadir count Not on HAART

Risk by nadir CD4 count

MCD rising incidence Incidence /10 4 patient years of follow up Pre HAART (83-96) 0.58 (95 % CI: ) P <0.05 Early HAART (97-01) 2.8 (95% CI: ) Post HAART (02-07) 8.3 (95% CI: )

Changing incidence over time

Plasma HHV8 viraemia HHV8 detectable HIV+ MCD25/30 (83%)  2 p < HIV+ KS26/73 (35%) HIV+ Lymphoma2/74 (3%) HIV+ controls0/53 (0%) Lightcycler quantitative PCR for orf HIV+ individuals

Median HHV8 VL Median in detected MCD26,00041,000 KS03,900 Higher viral load HHV8 in MCD

Management of MCD Rituximab Splenectomy if splenomegally Etoposide if severe symptoms

Overall survival N=42 5 year overall survival = 67%

Conclusions The incidence of MCD is rising MCD is not associated with degree of immunosuppression Plasma HHV8 is a diagnostic marker of MCD Long term remissions and 5 year survival of 67% can be achieved