multicentric castleman disease: unusual clinical presentations and outcome in 6 recent cases
DESCRIPTION
Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent cases Ch. Martin, D. Konopnicki, S. De Wit, N. Clumeck Saint-Pierre University Hospital, Brussels, Belgium. E-mail : [email protected]. 6 patients. Background - PowerPoint PPT PresentationTRANSCRIPT
Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent casesMulticentric Castleman Disease: unusual clinical presentations and outcome in 6 recent casesCh. Martin, D. Konopnicki, S. De Wit, N. Clumeck
Saint-Pierre University Hospital, Brussels, Belgium. E-mail : [email protected]
BackgroundBackground• Multicentric Castleman Disease (MCD) is a rare HIV-associated disease described mostly in caucasian homosexual men not treated for HIV. The precise incidence is unknown but some reports suggest a recent increasing possibly due to better diagnosis and awareness of clinicians.• Clinical presentation of MCD is polymorphic and sometimes fulminant. • It is a polyclonal lymphoproliferative disorder but monoclonal plasmablastic microlymphomas are often described in biopsied lymph nodes and risk for lymphomatous plasmablastic transformation is important (Oksenhendler 2002).
Methods
We describe 6 HIV-positive patients with MCD +/-
plasmablastic lymphoma transformation diagnosed and
managed in our institution during the last 3 years (2008-2011)
and compare our data’s with series of MCD described in the
literature.
ResultsAt time of MCD diagnosis:At time of MCD diagnosis: RangeRange MeanMean MedianMedian
Age (years)Age (years) 33-52 40.3 38.5
Duration of HIV infection (months)Duration of HIV infection (months) 12-67 26.5 21Nadir CD4 count (cells/mm³)Nadir CD4 count (cells/mm³) 46-706 236 160CD4 (cells/mm³)CD4 (cells/mm³) 46-1010 305 195
HIV-RNA (cp/ml)HIV-RNA (cp/ml) all patients (n=6)all patients (n=6) patients already on HAART (n=4) patients already on HAART (n=4)
0-731,000<20
126,805 <20
0<20
Duration of HAART (n=4) (months)Duration of HAART (n=4) (months) 10-21 10.6 11
Conclusion• We describe unusual presentation and outcome of Multicentric Castleman Disease :
Epidemiology: 33% in heterosexual African women and 66% in patients with undetectable HIV-RNA under HAART . Presentation: plasmablastic lymphoma transformation was more frequent than reported in literature (66% vs 6.8% in Mylona 2008) . Prognosis was better in patients with lymphoma treated by R-CHOP (4/4 alive in complete remission) than in patients without lymphoma (2/2 deaths).
• We suggest to implement a Belgian protocol to collect MCD characteristics from the post-HAART era and to treat MCD following recent recommendations.
6 patients6 patients
4 homosexuel men4 homosexuel men2 african heterosexuel
women2 african heterosexuel
women
MCD 1 fulminant death
Post-mortem diagn.
MCD 1 fulminant death
Post-mortem diagn.
3 MCD with lymphomatoustransformation
3 MCD with lymphomatoustransformation
1 MCD with lymphomatous transformation
1 MCD with lymphomatous transformation
1 pulmonary MCD
1 pulmonary MCD
3 R-CHOP +/- valganciclovir
3 CR alive:FU 29-16-16 months
3 R-CHOP +/- valganciclovir
3 CR alive:FU 29-16-16 months
1 R-CHOP1 CR alive:
FU 18 months
1 R-CHOP1 CR alive:
FU 18 months
1 R-CHOP then Etoposide
PRDeath at13 months.
1 R-CHOP then Etoposide
PRDeath at13 months.
•Symptoms: Lymph nodes 2/6 Anaemia with irregular antibody 3/6 B symptoms 4/6 Respiratory insufficiency 1/6 Palatin mass 1/6
• Association with Kaposi Sarcoma 3/6 (2 stomach, 3 lymph node capsule)•Transformation in plasmablastic lymphoma in plasmablastic lymphoma 4/6• Bone marrow: No invasion 5/6
B monoclonality 1/6• MCD flare-up after HAART initiation 1/2• Mortality 2/6 (33%)
Unusual findings: 2/6 heterosexual African women, 4/6 HIV-RNA<20 under HAART1/2 flare-up of symptoms after HAART initiation, coexistent lymphoma 66%, 4/4 lymphomas treated with R-CHOP alive.
R-CHOP= Rituximab and CHOP. CR = Complete remission. PR= Partial remission