TAFRO syndrome (Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis, and Organomegaly)
is a newly recognized variant of idiopathic - human herpesvirus-8 (HHV-8) negative
- Multicentric Castleman Disease (iMCD) [
[1]
]. Although salient clinical and histopathological features are similar to most of
iMCD related disorders, TAFRO syndrome follows a more aggressive course characterized
by rapidly progressive multiorgan failure [
[2]
]. Due to its recent recognition, standard treatments for TAFRO syndrome are lacking
and mostly derived from immunosuppressive regimens used in iMCD including corticosteroids,
chemotherapeutic agents (thalidomide and bortezomib), and monoclonal antibodies targeting
CD20+ B-lymphocytes (rituximab) or interleukin (IL)-6 (siltuximab and tocilizumab) [
[1]
]. In analogy to iMCD, in fact, TAFRO syndrome seems to be sustained by hyper-activation
of innate and adaptive immunity, ultimately leading to hyper-inflammation and organ
damage [
[3]
]. As opposite to iMCD, however, administration of the aforementioned drugs poses relevant
safety and efficacy concerns in TAFRO syndrome because thalidomide and IL-6 inhibitors
might be detrimental in the context of multiorgan failure, while bortezomib and rituximab
might not act as promptly as needed. In addition, TAFRO syndrome appears to be steroid
refractory in most cases [
[1]
]. Based on these considerations, we successfully treated a case of TAFRO syndrome
with multiorgan failure with IL-1 inhibition.Keywords
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References
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Article info
Publication history
Published online: February 16, 2021
Accepted:
February 6,
2021
Received:
February 1,
2021
Identification
Copyright
© 2021 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
