Acronym for Thrombopenia, Anasarca, Fever, Reticulin fibrosis and Organomegaly.

It is considered as a special form of idiopathic multicentric Castleman's disease (see this term). Rare systemic disease characterized by acute or subacute thrombocytopenia, hydrops (edema, pleural effusion, ascites), systemic inflammation (fever and/or elevated C-reactive protein) and organomegaly. Most patients show elevated serum alkaline phosphatase levels, while marked polyclonal hypergammapathy is rare.

Diagnosis is based on the combination of 3 major and at least 2 minor criteria:

-        3 major:

*        hydrops: pleural effusion, ascites, edema

*        thrombocytopenia:

*        systemic inflammation: fever of unknown origin and CRP > 2 mg/100ml

-        4 minor

*        lymph node biopsy consistent with Castleman's disease

*        reticulated myelofibrosis and numerous megakaryocytes on bone marrow biopsy

*        moderate organomegaly: hepato- or splenomegaly, lymph node hypertrophy

*        progressive renal failure

Histological examination of affected lymph nodes: three types

-        mixed type

-        plasma cell type

-        hypervascular-Castleman type

Given the severity of the initial presentation, treatment with high-dose corticosteroids is started, later combined with immunosuppressants such as tocilizumab, rituximab or cyclosporine A.

Anesthetic implications:

check importance of effusions and platelet count, corticosteroid therapy, immunosuppression;

References : 

-        Masaki Y, Ueda Y, Yanagisawa H, Arita K, Sakai T,Yamada K, Mizuta S, Fukushima T, Takai K, Aoki S, Kawabata H.
TAFRO syndrome: a disease requiring immediate medical attention.
Intern Med 2023 ; 62: 27-32, doi: 10.2169/internalmedicine.9622-22.

Updated: May 2024