Table 3.

Diagnostic criteria for iMCD-TAFRO

Criteria
Histopathological criteria: need all 
 Typical LN pathology (atrophic GCs with enlarged nuclei of ECs, proliferation of endothelial venules, small numbers of mature PCs) 
 Negative LANA-1 for HHV8 
Major criteria: need 3 of 5 
 Thrombocytopenia (<100 000/μL) 
 Anasarca (pleural effusions and ascites on CT) 
 Fever (>38°C) 
 Reticulin fibrosis 
 Organomegaly 
Minor criteria: at least 1 
 Hyper/normoplasia of megakaryocytes 
 High alkaline phosphatase without markedly elevated transaminases 
Criteria
Histopathological criteria: need all 
 Typical LN pathology (atrophic GCs with enlarged nuclei of ECs, proliferation of endothelial venules, small numbers of mature PCs) 
 Negative LANA-1 for HHV8 
Major criteria: need 3 of 5 
 Thrombocytopenia (<100 000/μL) 
 Anasarca (pleural effusions and ascites on CT) 
 Fever (>38°C) 
 Reticulin fibrosis 
 Organomegaly 
Minor criteria: at least 1 
 Hyper/normoplasia of megakaryocytes 
 High alkaline phosphatase without markedly elevated transaminases 

Adapted from Iwaki et al10  with permission.

CT, computed tomography scan; EC, endothelial cell; GC, germinal center; LN, lymph node.

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