We have all experienced misinformation about our disease. The CDCN wants to bring accurate information to the community and one of the ways we are doing this is through a book and new publication by Dr. David Fajgenbaum and Dr. Luke Chen: A Clinician’s Pearls and Myths.
Overview
• There are at least 4 clinically distinct subtypes of Castleman disease: unicentric Castleman disease (UCD), human herpes virus (HHV) 8-associated multicentric Castleman disease (typically associated with HIV), POEMS-associated multicentric Castleman disease, and HHV-8-negative/idiopathic multicentric Castleman disease (iMCD).
• iMCD is often further subdivided into iMCD-TAFRO and iMCD-Not Otherwise Specified (NOS). TAFRO is an acronym for: thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. iMCDNOS patients tend to have thrombocytosis, plasmacytosis, hypergammaglobulinemia, and a less severe clinical course than iMCD-TAFRO.
• Histopathological features should not be the basis upon which UCD is differentiated from MCD. Rather, the distinction between UCD and MCD should be made by imaging alone.
• Historically, Castleman disease was categorized by the histopathologic appearance of lymph nodes but this provides limited clinical utility.
• First-line treatment of UCD always includes surgical resection, when possible. Complete surgical resection of the involved lymph node(s) is almost always curative.
• Highly active antiretroviral therapy (HAART) and rituximab are quite effective for HHV-8 associated MCD.
• Biologic therapies targeting interleukin-6 are first-line therapies for the treatment of iMCD.