Siltuximab Monotherapy in Tafro Syndrome: A Case Report and Review of the Literature


TAFRO syndrome is characterized by the presence of thrombocytopenia, anasarca, fever, reticular myelofibrosis, organomegaly, and is frequently associated with kidney damage in the form of membranoproliferative glomerulonephritis (MPGN) or thrombotic microangiopathy (TMA). Treatment is based on corticosteroids. A 59-year-old man who suffered from heart disease, pancytopenia and hepatosplenomegaly of unknown etiology developed nephrotic syndrome and progressive renal insufficiency, with a kidney biopsy suggestive of MPGN with a “full-house” immunofluorescence pattern. Positron emission tomography (PET) revealed multiple lymphadenopathies which histologically were compatible with multicentric Castleman's disease. The patient was diagnosed with TAFRO syndrome and treatment with siltuximab was started, with evident improvement at 3 months. TAFRO syndrome is a rare entity which may present with severe kidney involvement and histological findings of MPGN or TMA, with or without immune complex deposits. Our case suggests that a corticosteroid-free regimen with siltuximab could be an attractive therapeutic option.