This protocol applies to patients with idiopathic multicentric Castleman disease (iMCD) who meet at least 2 of the following 5 severity criteria, indicating a high-acuity presentation with significant systemic involvement:
Patients meeting these criteria have evidence of multi-organ dysfunction — renal failure, anasarca, severe anemia, and/or pulmonary compromise — resulting in poor performance status that may require critical care. Meeting at least 2 of the 5 criteria above defines this severe iMCD population.
Individualized therapy for this setting may involve immunomodulators, immunosuppressants, or salvage cytotoxic approaches — with expert guidance strongly recommended given the complexity and severity of this presentation.
DOI: 10.1182/blood-2018-07-862334
Patients with severe iMCD have evidence of organ dysfunction such as renal failure, anasarca, severe anemia, and pulmonary dysfunction resulting in poor performance status likely requiring critical care.
Patients with severe iMCD must have at least 2 of the 5 criteria listed above.
Others may respond to immunomodulators/immunosuppressants or salvage cytotoxic therapy more commonly used in plasma cell malignancies (eg, VTD [bortezomib, thalidomide, dexamethasone]).
Among TAFRO cases, cyclosporine A can be useful therapy for anti–IL-6-refractory cases particularly to improve persistent ascites and thrombocytopenia.
A comprehensive analysis of a treatment-refractory iMCD-TAFRO patient who sustained multiple relapses after repeated cycles of chemotherapy showed upregulation of the mTOR pathway, and remission was successfully maintained with sirolimus.
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