Castleman Disease: Pearls and Myths

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.


• 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.

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