How to Recognize Castleman’s Disease Striking

After lengthy testing to rule out lymphoma or an autoimmune disease, I was diagnosed with Castleman’s disease, which involves many lymph nodes in different parts of the body. Because it is a rare disease, it is difficult to find information about possible treatments. Can you help me?

He answers Charles SelmiHead of Rheumatology and Clinical Immunology, Humanitas Institute, Milan (GO TO THE FORUM)

Castleman’s disease is a rare disease of the lymphatic system which hits every year or so 3 in a million people of residents of the United States. It can be a unicentric or multicentric disease, depending on whether it involves one or more lymph nodes in a single area or in different parts of the body, such as the armpit or neck. While the unicentric form generally has a benign course and is responsible for the majority of cases, multicentric Castleman disease may be associated with infection with human herpes virus 8 (HHV-8) or idiopathic (i.e. with no known cause) and overall accounts for about 25% of total cases. The multicentric form causes systemic symptoms such as fever, night sweats or weight changes associated with a frequent change of inflammatory indices such as ESR and C-reactive protein and others an increase in the size of the spleen
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These signs and symptoms are also common with moles hematological tumors and thus patients in whom an increase in lymph node size is noted (defined lymphadenopathy), at the visit or during an ultrasound or CAT scan, are often referred to the hematologist specialist who will request a biopsy or the excision of a lymph node for a histological diagnosis. Once lymphoma has been ruled out, the diagnosis of Castleman’s disease can be made based on the same histology even if a clear diagnostic suspicion is often necessary to assist the pathologist in reading the tissue. Reading the biopsy and the clinical picture contribute to the distinguish between the main forms of the disease because from this the trend and response to therapy can be derived.

The multicentric idiopathic forms of Castleman disease recognize an inflammatory mechanism that is mainly based on a mediator that causes inflammation, interleukin 6 (IL-6), known from studies on rheumatoid arthritis and more recently on Covid-19. Immunological knowledge has recently enabled the introduction of targeted therapies aimed at blocking interleukin 6 with a biologic drug, siltuximab, causing the picture and symptoms to deteriorate in many cases. Indeed, in some cases, idiopathic multicentric Castleman disease can manifest with platelet reduction, effusion formation, bone marrow and kidney dysfunctionas well as enlargement of the liver and spleen in a form called TAFRO.