Objective:

We present a case of young female with idiopathic multicentric CD (iMCD)-TAFRO variant with rare CNS involvement.

Background:

Castleman Disease (CD) is group of lymphoproliferative disorders that affect lymph nodes and non-nodal tissues, usually in the chest or abdomen. TAFRO is rare subtype of  iMCD that involves thrombocytopenia, anasarca, fever, reticulin myelofibrosis and organomegaly. Involvement of the central nervous system is extremely rare

Design/Methods:

Case Report

Results:

15 yo female with history of hypothyroidism, Albright hereditary osteodystrophy & celiac disease presented with a 2-month history of abdominal pain, arthralgias, fatigue and anasarca. She was found to have anemia, elevated inflammatory markers, pancytopenia, and lymphadenopathy. Lymph node biopsy confirmed diagnosis of iMCD –TAFRO. She was started on siltuximab-an IL-6 inhibitor-which is FDA approved for treatment. Shortly after she developed left upper and lower extremity weakness and facial droop. MRI demonstrated right corona radiata and left basal ganglia infarct. MRA Brain demonstrated multifocal irregularities in bilateral supraclinoid ICA and MCAs suggestive of an inflammatory vasculopathy. This was seen to involve mainly the medium sized arteries with no involvement of the cervical vasculature. She was started on low dose aspirin and intravenous pulse steroids x 5 days then prolonged taper for the CNS vasculitis with clinical improvement of her left sided weakness. She was maintained on low dose steroids and Siltuximab treatments with followup MRA at 3 months demonstrating some improvement; One year follow-up scans showed significant improvement in caliber with significant resolution of the previously noted vasculopathy of bilateral ICA and MCAs

Conclusions:

We present the first reported case of iMCD-TAFRO syndrome in pediatric patient with CNS involvement including ischemic infarcts and CNS vasculitis. While the pathophysiology of CNS involvement remains unclear, it is important for treating clinicians to be cognizant of potential CNS involvement in this rare condition among pediatric population.