TAFRO syndrome, variant of Castleman's disease: case report

Multicentric Castleman’s disease is a rare benign lymphoproliferative disorder that involves multiple enlarged lymph nodes in different areas of the body. Recently, a unique clinic pathological variant of this disease called TAFRO syndrome has been described.

A 23-year-old male with a history of one year and six months of evolution characterized by generalized lymphadenopathy with, night diaphoresis, dyspnea and weight loss. At the third month, hypertension was diagnosed and 8 months later type 2 diabetes mellitus. After his admission, microcytic anemia, thrombocytopenia, renal dysfunction, pleural effusion and ascites were documented. Based on the findings of the physical examination, a computed tomography scan was performed, where multiple lymph nodes and hepatosplenomegaly were identified. A lymph node biopsy was performed, which reported Castleman’s disease, considering then a diagnosis of TAFRO syndrome due to its association with thrombocytopenia, microcytic anemia, anasarca, fever, renal dysfunction, adenomegaly and hepatosplenomegaly.

There are still doubts about whether it corresponds to a different disease or a subtype of the Castleman disease. It is more frequent in women, occurs in the middle-aged and elderly and given its heterogeneous presentation, it usually represents a diagnostic challenge. Although its management is not standardized, therapeutic options include immunosuppressant such as steroids, cyclosporine, rituximab and anti-IL6.