Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent cases
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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

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Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent cases

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Multicentric castleman disease unusual clinical presentations and outcome in 6 recent cases

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

  • MulticentricCastleman 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 plasmablasticmicrolymphomas are often described in biopsied lymph nodes and risk for lymphomatousplasmablastic transformation is important (Oksenhendler2002).

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.

4 homosexuel men

2 africanheterosexuel

women

Results

3 MCD with

lymphomatous

transformation

1 MCD with

lymphomatous

transformation

1 pulmonary

MCD

  • 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)

  • Transformationin plasmablastic lymphoma 4/6

  • Bone marrow: No invasion 5/6

  • B monoclonality 1/6

  • MCD flare-up after HAART initiation1/2

  • Mortality 2/6 (33%)

MCD

1 fulminant death

Post-mortem diagn.

3 R-CHOP

+/- valganciclovir

3 CR alive:

FU 29-16-16 months

1 R-CHOP

1 CR alive:

FU 18 months

1 R-CHOP

then Etoposide

PR

Death at13 months.

R-CHOP= Rituximab and CHOP. CR = Complete remission. PR= Partial remission

Unusual findings: 2/6 heterosexual African women, 4/6 HIV-RNA<20 under HAART

1/2 flare-up of symptoms after HAART initiation, coexistent lymphoma 66%, 4/4 lymphomas treated with R-CHOP alive.

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.


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