Castleman disease and TAFRO syndrome: To improve the diagnostic consciousness is the key

Table 1 Diagnostic criteria for TAFRO syndrome proposed by Masaki et al (updated in 2019)

Diagnostic criteria for TAFRO syndrome
Primary criteria
Edema: Including pleural and abdominal effusion and systemic edema
Thrombocytopenia: Platelet count ≤ 105/uL before myelosuppression
Systemic inflammation: Fever of unknown origin, body temperature exceeding 37.5°C, and/or serum CRP ≥ 2 mg/dL
Secondary criteria
Pathological manifestations of CD-like lymph nodes
Bone marrow reticular fibrosis and/or increased bone marrow megakaryocyte count
Mild organ enlargement: Including liver, spleen, and lymph node enlargement
Progressive renal dysfunction
TAFRO syndrome can be diagnosed after meeting at least two of all three main criteria and four secondary criteria, and excluding malignant tumor, autoimmune disease, infection, POEMS syndrome, liver cirrhosis, TTP/HUS, etc.

CRP: C-reactive protein; CD: Castleman disease; TTP: Thrombotic thrombocytopenic purpura; HUS: Hemolytic uremic syndrome.