Diagnostic criteria for iMCD
| Inclusion criteria . | Exclusion criteria . |
|---|---|
| I. Major criteria (need both) | Infection-related disorders |
| 1. Histopathologic lymph node | 1. HHV8 |
| 2. Enlarged lymph nodes in ≥2 lymph node stations | 2. EBV LPD |
| II. Minor criteria (need ≥2 of 11 with ≥1 laboratory criterion) | 3. Inflammation and adenopathy by other infection |
| Laboratory | Autoimmune/inflammatory disease |
| 1. Elevated ESR or CRP | 1. SLE |
| 2. Anemia | 2. Rheumatoid arthritis |
| 3. Thrombocytopenia/tosis | 3. Adult-onset Still disease |
| 4. Renal dysfunction or proteinuria | 4. Juvenile idiopathic arthritis |
| 5. Polyclonal hypergammaglobulinemia | 5. Autoimmune LPS |
| 6. Hypoalbuminemia | Malignant LPD |
| Clinical | 1. Lymphoma |
| 1. Constitutional symptoms | 2. Multiple myeloma |
| 2. Large spleen and/or liver | 3. Primary lymph node plasmacytoma |
| 3. Fluid accumulation | 4. FDC sarcoma |
| 4. Eruptive cherry angiomata or violaceous papules | 5. POEMS syndrome |
| 5. Lymphocytic interstitial pneumonitis |
| Inclusion criteria . | Exclusion criteria . |
|---|---|
| I. Major criteria (need both) | Infection-related disorders |
| 1. Histopathologic lymph node | 1. HHV8 |
| 2. Enlarged lymph nodes in ≥2 lymph node stations | 2. EBV LPD |
| II. Minor criteria (need ≥2 of 11 with ≥1 laboratory criterion) | 3. Inflammation and adenopathy by other infection |
| Laboratory | Autoimmune/inflammatory disease |
| 1. Elevated ESR or CRP | 1. SLE |
| 2. Anemia | 2. Rheumatoid arthritis |
| 3. Thrombocytopenia/tosis | 3. Adult-onset Still disease |
| 4. Renal dysfunction or proteinuria | 4. Juvenile idiopathic arthritis |
| 5. Polyclonal hypergammaglobulinemia | 5. Autoimmune LPS |
| 6. Hypoalbuminemia | Malignant LPD |
| Clinical | 1. Lymphoma |
| 1. Constitutional symptoms | 2. Multiple myeloma |
| 2. Large spleen and/or liver | 3. Primary lymph node plasmacytoma |
| 3. Fluid accumulation | 4. FDC sarcoma |
| 4. Eruptive cherry angiomata or violaceous papules | 5. POEMS syndrome |
| 5. Lymphocytic interstitial pneumonitis |
Features supportive of, but not required for, diagnosis include: elevated IL-6, soluble IL-2 receptor (sIL-2R), VEGF, immunoglobulin A (IgA), IgE, lactate dehydrogenase (LDH), and/or β2-microglobulin (B2M); reticulin fibrosis of bone marrow (particularly in patients with TAFRO syndrome); paraneoplastic pemphigus, bronchiolitis obliterans–organizing pneumonia, autoimmune cytopenias, polyneuropathy (without diagnosing POEMS), glomerular nephropathy, inflammatory myofibroblastic tumor. Reprinted from Fajgenbaum et al.11
CRP, C-reactive protein; EBV LPD, Epstein-Barr virus lymphoproliferative disease; ESR, erythrocyte sedimentation rate; FDC, follicular dendritic cell; LPD, lymphoproliferative disorder; LPS, lymphoproliferative syndrome; SLE, systemic lupus erythematosus.