TAFRO syndrome: A case report with biopsy findings from three sites

Abstract Introduction/Objective TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) is a rare systemic inflammatory disorder. First reported in 2010, the majority of cases are from Japan, where it currently regarded as subtype HHV8-negative Multicentric Castleman Disease. Methods/Case Report We report case occurring 17-year old Puerto Rico. She originally presented with abdominal pain, vomiting, was also found to have splenomegaly, elevated sedimentation rate C-reactive protein. Over course next several days she developed respiratory distress, requiring ventilatory support, anuric renal failure. Renal biopsy demonstrated evidence thrombotic microangiopathy, which interpreted atypical hemolytic syndrome, dialysis treated eculizumab. Because other diagnostic considerations at time included hemophagocytic lymphohistiocytosis catastrophic antiphospholipid bone marrow examination performed increased megakaryocytes, without fibrosis or hemophagocytosis. two months, anasarca ascites, pleural effusion, pericardial multiple enlarged axillary cervical lymph nodes, persistence splenomegaly. Biopsy left node features suggestive Castlemans disease, plasma cell variant. The constellation symptoms, laboratory investigations diagnoses support diagnosis syndrome. patient received rituximab, high dose steroids, hemodialysis clinical improvement. At 16 months follow up, remission. Results (if Case Study enter NA) NA Conclusion From pathology standpoint, provides findings three distinct organ systems all relatively unusual that together characteristic It highlights risk accepting “biopsy proven” such uremic require significant correlation for true confirmation, points out importance opportunity pathologists see forest along trees order recognize diseases TAFRO.