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

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Presentation on theme: "The incidence of HIV associated Castleman’s disease Professor Mark Bower Chelsea & Westminster Hospital London, UK."— Presentation transcript:

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

2 Castleman’s disease

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

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

5 IgM light chain restricted (monotypic polyclonal)

6 HHV8 in plasmablasts in MCD

7 MCD incidence in CWH cohort 1983-2007 10,997 patients 56,202 patient years follow-up

8 Overall incidence KS & MCD MCDKS Cases241180 Incidence per 10,000 PY (95%CI) 4.3 (2.7-6.4) 210 (198-222)

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

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

11 Risk by nadir CD4 count

12 MCD rising incidence Incidence /10 4 patient years of follow up Pre HAART (83-96) 0.58 (95 % CI: 0.02-4.2) P <0.05 Early HAART (97-01) 2.8 (95% CI: 0.9-6.5) Post HAART (02-07) 8.3 (95% CI: 4.6-12.6)

13 Changing incidence over time

14 Plasma HHV8 viraemia HHV8 detectable HIV+ MCD25/30 (83%)  2 p <0.0001 HIV+ KS26/73 (35%) HIV+ Lymphoma2/74 (3%) HIV+ controls0/53 (0%) Lightcycler quantitative PCR for orf-72 240 HIV+ individuals

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

16 Management of MCD Rituximab Splenectomy if splenomegally Etoposide if severe symptoms

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

18 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

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