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



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