TAFRO TAFRO syndrome was first described in 2010, standing for: - Thrombocytopenia - Anasarca - Fever - Reticulin fibrosis - Organomegaly TAFRO syndrome is considered a variant of multicentric Castleman disease, which is a rare disorder involving an overgrowth of cells in the lymph nodes TAFRO syndrome as a distinct subtype of idiopathic MCD, and as such, iMCD was divided into 2 categories: iMCD with TAFRO (iMCD-TAFRO) and iMCD without TAFRO (iMCD-NOS). Idiopathic MCD without TAFRO typically presents with thrombocytosis, hypergammaglobulinemia, and less severe anasarca Epidemiology: - W > M - 30-40 years old DDX: - Malignancies, including lymphoma, myeloma, mesothelioma, etc - Autoimmune disorders, including systemic lupus erythematosus (SLE), Sjögren’s syndrome, ANCA-associated vasculitis - Infectious disorders, including acid fast bacterial infection, rickettsial disease, Lyme disease, severe fever with thrombocytopenia syndrome (SFTS) - POEMS syndrome - IGG4 - Hepatic cirrhosis - TTP/HUS - Angioimmunoblastic T cell lymphoma - Intravascular large B cell lymphoma Pathophysiology: Hypercytokinemia, with interleukin (IL)-6 along with (VEGF) vascular endothelial growth factor leads to histopathologic changes in LNs and the systemic symptoms Histopathological Diagnoses - The lymph node histopathology of TAFRO syndrome mimics that of MCD; thus, TAFRO syndrome is thought to be a variant of iMCD (iMCD-TAFRO) - Lymph nodes: Atrophic germinal centers with expansion of the interfollicular zone, highly dense endothelial venules, and few mature plasma cells Labs: - Leukocytosis - Thrombocytopenia - Elevated alkaline phosphatase - Normal immunoglobulin levels - Increased CRP/ESR - Hypoalbuminemia Treatment: - 1st line: Glucocorticoid, high dose - 2nd line: Rituximab, Tocilizumab, Cyclosporin A, Thrombopoietin receptor agonists #TAFRO #diagnosis #management #rheumatology #Thrombocytopenia
