Glucocorticoids combined with tofacitinib in the treatment of Castleman's disease: A case report

Table 3 Idiopathic multicentric Castleman's disease-thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly diagnostic criteria

Diagnostic criteria	Contents
Histopathological criteria	(1) The pathological results of lymph nodes are consistent with the characteristic manifestations of TAFRO lymph nodes1; and (2) Negative LANA-1 for HHV-8
Main diagnostic criteria	(1) Three of the five TAFRO related symptoms were met: Thrombocytopenia, fever (body temperature ≥ 38 ℃), anasarca, organomegaly, reticulin fibrosis; (2) Absence of hypergammaglobulinemia; and (3) Small volume lymphadenopathy
Secondary diagnostic criteria	(1) Normal or proliferative of megakaryocytes in bone marrow; and (2) The level of serum alkaline phosphatase increased, but the level of transaminase did not increase significantly

¹TAFRO lymph nodes are characterized by atrophy of germinal centers, enlargement of endothelial cell nuclei, the proliferation of endothelial venules, enlargement of nuclei in the interlobular region, and a small number of mature plasma cells.

Note: The diagnosis of iMCD-TAFRO should meet the histopathological diagnosis, primary criteria, and at least one secondary criterion; Besides, rheumatic diseases (such as SLE), infectious diseases (such as tuberculosis) and tumor diseases (such as lymphoma) should be excluded. HHV: Human herpesvirus; LANA: Latency-associated nuclear antigen; TAFRO: Thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly.