Table 3. Proposed diagnostic criteria for TAFRO-iMCD.

1. Histopathological Criteria;

• Compatible with pathological findings of lymph nodes as TAFRO-iMCD*

• Negative LANA-1 for HHV-8

2. Major criteria;

• Presents 3 of 5 TAFRO symptoms at time of diagnosis **

√ Thrombocytopenia

√ Anasarca

√ Fever

√ Reticulin fibrosis

√ Organomegaly

• Absence of hypergammaglobulinemia

• Small volume lymphadenopathy

3. Minor criteria need 1 or more;

• Hyper/normoplasia of megakaryocytes in bone marrow

• High levels of serum ALP without marked elevated serum transaminase ***

Requirements; fulfilled histopathological criteria, major criteria, and meets 1 or more of minor criteria.

Differential diagnosis is necessary from other disorders, including rheumatologic disease; systemic lupus erythematosus (SLE), neoplastic disease; lymphoma, POEMS syndrome, and cancer.

*TAFRO characteristic findings of lymph node, ie, atrophic germinal centers with enlarged nuclei of endothelial cells, proliferation of endothelial venules with enlarged nuclear in interfollicular zone, and small numbers of mature plasma cells. **Thrombocytopenia: Platelet count < 100 × 103/μ L. Anasarca: the presence of pleural fluids and ascites on computed tomography, Fever: The body temperature of > 38.0 °C (100.4 °F). Reticulin fibrosis: evaluated via bone marrow biopsy, Organomegaly: including lymphadenopathy, hepatomegaly or splenomegaly on computed tomography. ***ALP: alkaline phosphatase.