TAFRO Syndrome: A Case of Significant Endocrinopathy in a Caucasian Patient

Abstract

Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disorder that manifests as multiorgan dysfunction secondary to widespread inflammation. The underlying pathogenesis is driven by an excessive and inappropriate cytokine storm. TAFRO syndrome is a rare subtype of iMCD, characterized by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Multiorgan dysfunction is a known consequence of this syndrome, although endocrine involvement has yet to be reported. We present a case of TAFRO in a previously healthy Caucasian male who presented with abdominal pain, dysuria, diffuse anasarca, and ascites. On presentation, the patient was found to have acute kidney injury, thrombocytopenia, elevated inflammatory markers, elevated interleukin-6 (IL-6), and endocrinopathy. Following an extensive infectious and autoimmune workup, lymph node biopsy confirmed the diagnosis of TAFRO. The patient was started on prednisone, rituximab, and anti-IL-6 therapy with siltuximab. He achieved clinical remission after 4 months of treatment, with normalization of renal function, thrombocytopenia, inflammatory markers, and endocrinopathy. He has continued on siltuximab for maintenance therapy. It is our hope that this unique case of TAFRO syndrome with significant endocrinopathy will add to the growing literature surrounding iMCD, and help clinicians better understand the pathogenesis and treatment of this rare disease.