Castleman disease (CD)
Conditions
Interventions
Siltuximab combined with BCD treatment group:None
Sponsors
Peking Union Medical College Hospital
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Over 18 years old; 2. Meet the diagnostic criteria for severe iMCD in iMCD: (1) iMCD diagnostic criteria: Patients need to meet the following 2 major criteria and 2 minor criteria to be diagnosed with iMCD (at least 1 must be a lab test), and other diseases that might cause "Castleman-like changes" in lymph nodes must be excluded. Major criteria include: 1) Pathology consistent with Castleman disease; 2) Enlargement of lymph nodes in at least two locations with a short axis over 1 cm. Minor criteria include (the first 6 are lab tests, the last 5 are clinical manifestations): 1) Elevated CRP; 2) Anemia; 3) Increased or decreased platelets; 4) Low albumin; 5) Kidney dysfunction or protein in urine; 6) Polyclonal hypergammaglobulinemia; 7) Systemic symptoms; 8) Enlarged liver or spleen; 9) Serous effusion or edema; 10) Characteristic rash; 11) Interstitial pneumonia. (2) Severe iMCD Classification Criteria: Patients need to meet 2 or more of the following 5 criteria to be diagnosed with severe iMCD: 1) ECOG performance status >= 2; 2) eGFR < 30 ml/min/1.73 m^2; 3) Severe edema and/or ascites, pleural effusion, or pericardial effusion; 4) Hb <= 80 g/L; 5) Lung involvement or interstitial pneumonia with shortness of breath. 3. Women of childbearing age need to agree to pregnancy screening before the study starts, and all patients must use contraception during the study and for 3 months after the last dose. 4. All patients must sign an informed consent form.
Exclusion criteria
Exclusion criteria: 1. Used immunosuppressants or anti-cancer drugs in the past 3 months (excluding patients who used corticosteroids within 1 week before enrollment). 2. Having other malignant tumors. 3. Peripheral neuropathy of grade 2 or higher. 4. Active infection. 5. Women who plan to get pregnant within a year, are pregnant, or breastfeeding. 6. Refuse to sign informed consent or cannot follow up at our hospital. 7. Have underlying diseases such as systemic lupus erythematosus, POEMS, lymphoma, or HHV-8 infection that may cause lymph node changes similar to Castleman’s disease. 8. Severe allergy to any component of the treatment regimen (siltuximab, bortezomib, cyclophosphamide, dexamethasone).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Overall response rate; | — |
Secondary
| Measure | Time frame |
|---|---|
| Laboratory indicators;Progression-free survival of siltuximab combined with BCD therapy;Safety analysis;Overall survival of the enrolled patients; | — |
Countries
China
Contacts
Public ContactLu Zhang
Peking Union Medical College Hospital
Outcome results
None listed