Table 1 From the iMCD consensus diagnostic criteria proposed from The International Working Group for iMCD [4]

Major criteria

 1. Lymph nodes with histopathologic features consistent with iMCD spectrum

 2. Enlarged lymph nodes (≥ 1 cm in short-axis diameter) in ≥ 2 lymph node regions

Minor criteria

Laboratory

 1. Elevated CRP or ESR

 2. Anemia

 3. Thrombocytopenia or thrombocytosis

 4. Hypoalbuminemia

 5. Renal dysfunction or proteinuria

 6. Polyclonal hypergammaglobulinemia

Clinical

 1. B symptoms

 2. Hepatomegaly or splenomegaly

 3. Fluid accumulation

 4. Eruptive cherry hemangiomatosis

 5. Violaceous papules

 6. Lymphocytic interstitial pneumonitis

Supporting features

 1. Elevated IL-6, VEGF, IgA, IgE, LDH, and/or B2M

 2. Reticulin fibrosis of bone marrow

 3. Disorders associated with iMCD: paraneoplastic pemphigus, bronchiolitis obliterans organizing pneumonia, autoimmune cytopenias, polyneuropathy, inflammatory myofibroblastic tumor

Exclusion criteria

 1. Infection: HHV-8, EBV, CMV, toxoplasmosis, HIV, active tuberculosis

 2. Autoimmune/autoinflammatory: systemic lupus erythematosus, rheumatoid arthritis, adult-onset Still disease, juvenile idiopathic arthritis, autoimmune lymphoproliferative syndrome

 3. Malignancy: lymphoma, multiple myeloma, POEMS syndrome, primary lymph node plasmacytoma, follicular dendritic cell sarcoma