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Published on: December 3, 2019
Multicentric Castleman's disease in HIV patients: a single-center cohort diagnosed from 2008 to 2018
Smaranda Gliga1, Hans Martin Orth2, Nadine Lübke3
1Department of Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Heinrich Heine University, Düsseldorf, Germany. smaranda.gliga@med.uni-duesseldorf.de.
Insights
Rituximab is an effective first-line therapy for HIV- and HHV-8-positive multicentric Castleman's disease (MCD). Further studies are needed to confirm the efficacy of other treatments for this rare condition.
Area of Science:
- Infectious Diseases
- Hematology
- Immunology
Background:
- Castleman's disease (CD) is a rare lymphoproliferative disorder.
- Multicentric Castleman's disease (MCD) associated with HIV and HHV-8 presents unique management challenges.
- Limited data exists on therapeutic strategies for HIV- and HHV-8-positive MCD.
Purpose of the Study:
- To evaluate the management and therapeutic outcomes of HIV- and HHV-8-positive multicentric Castleman's disease (MCD).
- To present single-center experience with a cohort of patients diagnosed with HIV-associated MCD.
Main Methods:
- Retrospective, descriptive study of patients with MCD admitted between 2008-2018.
- Inclusion criteria: HIV diagnosis, clinical signs of MCD, and evidence of HHV-8.
- Data collected on patient demographics, treatment regimens, and clinical outcomes.
Main Results:
- Nine male patients with HIV-associated MCD were included.
- Rituximab was the primary first-line therapy, achieving symptom resolution in 6/9 patients.
- Other treatments included tocilizumab, splenectomy, and antiviral/targeted therapies with varying outcomes.
- Relapse rate was 44%, with 1-year and 3-year survival rates of 87.5% and 71.4%, respectively.
Conclusions:
- Rituximab demonstrates effectiveness as a first-line and retreatment option for HIV- and HHV-8-positive MCD.
- The efficacy of alternative therapies such as splenectomy and other immunotherapies requires further investigation in larger cohorts.
Purpose:
Castleman's disease (CD) is a well-established entity but there is a lack of available data regarding the management and therapy of HIV- and HHV-8-positive multicentric CD (MCD). We provide our own single-center experience with HIV-associated MCD.
Methods:
We performed a retrospective, descriptive study on a cohort of patients with MCD, diagnosed and admitted to the infectious diseases or intensive care unit in the University Hospital Düsseldorf between 2008 and 2018. Included patients had a previous or new HIV diagnosis and clinical signs resembling MCD with evidence of HHV-8 replication or histological diagnosis for MCD.
Results:
Nine male patients were included in the study. All patients were treated with Rituximab after diagnosis of MCD, with six of them acquiring resolution of symptoms. Three patients received tocilizumab additionally. Other treatment options included: splenectomy (2/9), valganciclovir (2/9), vincristine and siltuximab (1/9), ruxolitinib and Cytosorb® (2/9). The relapse rate was 44% (4/9) and the survival rate 87.5% after 1 year (8/9) and 71.4% after 3 years (5/7).
Conclusion:
The most effective first-line therapy and retreatment option remains rituximab. The effectiveness of other treatment options like splenectomy or different immunotherapeutic approaches requires confirmation in larger-scale studies.
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