TAFRO syndrome: A case report and review of the literature

Abstract

TAFRO syndrome is a rare clinicopathologic variant of idiopathic multicentric Castleman disease characterized by Thrombocytopenia, Ascites (anasarca), myeloFibrosis, Renal dysfunction, and Organomegaly. Here, we report a case of TAFRO syndrome in an HIV-negative young Caucasian male who presented with fever, normocytic anemia, thrombocytopenia, and acute renal insufficiency. The serum interleukin-6 (IL-6) level was elevated. Chest and abdominal CT revealed bilateral pleural effusion, ascites, splenomegaly, and multiple mildly enlarged lymph nodes. An excisional biopsy of inguinal lymph node showed a few atrophic follicles and expansion of interfollicular areas by marked vascular proliferation and polytypic plasmacytosis. HHV-8 was negative. Subsequent bone marrow biopsy was normocellular with moderately increased megakaryocytes and occasional megakaryocytic emperipolesis. His signs and symptoms improved after treatment with methylprednisolone and tocilizumab (anti-IL-6 receptor antibody). Our study confirms the distinctive nature of this syndrome, which should allow for better recognition and appropriate therapy.

Fig. 1.

Biopsy of an inguinal lymph node shows regressed germinal center with surrounded mantle zone with onion skinning change and penetrating blood vessel (A–C). Abundant histiocytes and plasma cells (D) and prominent vascular proliferation with plump endothelial cells are present in the expanded interfollicular zone (E). Bone marrow biopsy shows moderate hyperplasia of megakaryocytes (F) with occasional emperipolesis (F, inserts). A, ×40; B, ×100; C and E, ×200; D and F, ×400.

Fig. 2.

Immunohistochemistry staining, ×40. CD20 shows B-cells in small follicles and CD3 highlights the unremarkable paracortical T-cells. CD138, Kappa, and Lambda show polyclonal plasmacytosis. CD34 and Factor VIII-related antigen highlight the massive proliferation of small vessels. HHV-8 and EBER are negative.