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. Author manuscript; available in PMC: 2022 Nov 14.
Published in final edited form as: Am J Hematol. 2021 Jul 28;96(10):1241–1252. doi: 10.1002/ajh.26292

Figure 1: Concepts of TAFRO syndrome and iMCD-TAFRO.

Figure 1:

TAFRO syndrome is a heterogenous clinical entity with a constellation of non-specific clinical symptoms including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis or renal insufficiency (R), and organomegaly (O). Due to its heterogeneity, TAFRO syndrome includes various clinical conditions such as malignancies, rheumatologic disorders, infections, and POEMS syndrome. The figure conceptualizes five different classifications related to TAFRO syndrome and iMCD-TAFRO. The present study included cases with iMCD-TAFRO, TAFRO with possible iMCD without lymph node biopsy and other co-morbidities (TAFRO with possible iMCD), and TAFRO without histologically proven iMCD and other co-morbidities (TAFRO without iMCD and other co-morbidities). Attention needs to be paid not to confuse TAFRO syndrome and iMCD-TAFRO.

Abbreviation: iMCD-TAFRO, TAFRO clinical subtype of idiopathic multicentric Castleman disease; iMCD-NOS, idiopathic multicentric Castleman disease not otherwise specified.