September 24, 2014:

We need your help to make this new classification system a reality!  

The deciding committee is accepting letters until November 21, 2014 from the public with comments on diagnostic proposals. We need them to receive dozens of emails stating that we need them to approve "Option #1" (see below).  You can either email them directly at or contact us to let us know that you're interested in writing an email, which we will help you draft.

Drs. David Fajgenbaum and Frits van Rhee proposed a new classification system for Castleman disease (CD) on behalf of the CDCN Scientific Advisory Board to the International Classification of Diseases (ICD) subcommittee, which includes members of the Centers for Disease Control & Prevention (CDC) and Centers for Medicare/Medicaid Services (CMS).

Establishing a unique ICD-10 coding system is considered to be a top priority for the CD field. Currently, the code for "Castleman's disease" is shared with several other diseases, so the particular code cannot contribute to research, improved patient care, or better understand of the disease. Drs Fajgenbaum and van Rhee's coding system includes sub-codes for: unicentric CD, HHV-8-positive MCD, HHV-8-negative MCD, and Castleman disease unspecified.  They proposed the four sub-codes because of the extremely difference clinical courses, outcomes, survival rates, and treatments needed for each of the diseases.

The CDCN believes that the new coding system, if adopted, will:

  • Facilitate faster and more accurate diagnoses,
  • Facilitate access to effective and often life-saving therapies,
  • Help to generate real-world data to improve understanding of epidemiology and patient care, and
  • Improve patient care and survival rates by encouraging physicians to determine appropriate sub-classification (i.e., unicentric CD, HHV-8-positive multicentric CD, or HHV-8-negative multicentric CD)

The ICD subcommittee will be accepting letters from the public, including patients, loved ones, and physicians, in support of or in opposition to the above proposed system.

They are considering two coding options. The CDCN strongly supports option #1 whereby the heterogeneous group of diseases referred to as "Castleman disease" is split appropriately into unicentric CD, HHV-8-associated MCD, HHV-8-negative MCD, and CD unspecified. Option #2 just calls for one code, "Castleman disease," which unfortunately will not informative for physicians or researchers, because of the tremendous differences between the diseases. Please contact us if you're interested in sending in a letter to the ICD.


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