There was a mistake in Table 2 as published. An incorrect file was erroneously published in place of Table 2. The corrected Table 2 appears below.
Table 2.
Comparison of diagnostic results in idiopathic multicentric Castleman disease with TAFRO features (iMCD-TAFRO), hemophagocytic lymphohistiocytosis (HLH), and other relevant mimics (such as iMCD-NOS, autoimmune diseases, and lymphoma) (3, 7–9, 14, 17).
| Feature | iMCD-TAFRO | HLH | Other Mimics (iMCD-NOS, Autoimmune, Lymphoma) |
|---|---|---|---|
| Clinical Presentation | Acute onset; fever; anasarca; abdominal pain; organomegaly (hepatosplenomegaly); may lack prominent lymphadenopathy | Acute onset; fever; hepatosplenomegaly; cytopenias; neurologic symptoms; rash | Subacute/chronic; variable fever; lymphadenopathy; organomegaly; less anasarca |
| Thrombocytopenia | Marked, often transfusion-resistant | Marked, part of bicytopenia | Mild or absent; iMCD-NOS may have thrombocytosis |
| Anasarca/Edema | Prominent, often with pleural effusions | May occur, but less prominent | Rare; may occur in severe autoimmune disease |
| Fever | High, persistent | High, persistent | Variable; less common in indolent mimics |
| Organomegaly | Hepatosplenomegaly, mild lymphadenopathy | Hepatosplenomegaly, lymphadenopathy | Lymphadenopathy prominent in lymphoma/iMCD-NOS |
| Renal Dysfunction | Common, often with proteinuria | May occur (secondary to multiorgan failure) | Variable; less common |
| Hemophagocytosis | Absent or rare | Characteristic (bone marrow, other tissues) | Absent |
| Hyperferritinemia | Mild to moderate | Marked, often >10,000 ng/mL | Mild or absent |
| CRP/ESR | Elevated CRP, variable ESR | Elevated CRP, variable ESR | Elevated in inflammation |
| IgG/Hypergamma-globulinemia | Normal or low IgG; polyclonal gammopathy rare | Normal or low IgG | iMCD-NOS: polyclonal hypergammaglobulinemia |
| Anti-SSA/Ro Antibodies | Often positive | Negative | Negative |
| Imaging | Mild lymphadenopathy; marked effusions/anasarca; organomegaly | Hepatosplenomegaly; lymphadenopathy; CNS lesions (MRI) | Prominent lymphadenopathy (lymphoma); variable effusions |
| Bone Marrow | Reticulin fibrosis; mild plasmacytosis | Hemophagocytosis; cytopenias | Plasmacytosis (iMCD-NOS); malignant cells (lymphoma) |
The original version of this article has been updated.
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