Big strides were made recently for the diagnosis of Castleman disease patients! The first-ever diagnostic criteria for HHV-8-negative "idiopathic" Multicentric Castleman disease has been published in the top hematology journal, Blood. Though Castleman disease was first described in 1954, this is the first time there has ever been a diagnostic criteria for it. Read more about it from Penn Medicine here.
Previously, patients relied on their doctor's familiarity with how the disease had been described in the medical literature in order for them to even consider it as a possible diagnosis for their sickness. Even for those doctors considering the diagnosis, actually diagnosing HHV-8-negative/idiopathic MCD was very difficult because there was no reported diagnostic criteria!
Now, there is a criteria that defines what physicians should look out for and what they need to check off and rule out to make the diagnosis of Castleman disease. The full text of the diagnostic criteria can be found here.
For an overview of the diagnostic guidelines, check out this article in the Rare Disease Report here.
Finally, in October 2016, after 2 years of submissions and advocacy, Castleman disease received its first-ever unique ICD-10 code! Before getting a unique ICD-10 code, our ICD-9 code was shared with many other diseases making it difficult for patients to receive treatment as many were denied. Additionally, large-scale research wasn't possible without a unique code thus opening the door for additional research opportunities in the future!