Recommended workup of iMCD
| Purpose . | Tests . |
|---|---|
| Inflammatory response | CBC, renal function, liver function, CRP, ESR, fibrinogen, immunoglobulins & free light chains, albumin, ferritin* |
| Histopathology | Hypervascular/mixed cellularity/plasmacytic variant |
| Virologic status | HIV serology, HHV-8 qPCR (peripheral blood), EBER (lymph node), LANA-1 (lymph node) |
| Cytokine profile | IL-6, VEGF, sIL-2 receptor† |
| Imaging | CT-PET or CT neck, chest, abdomen, pelvis |
| Bone marrow evaluation | MGUS, myeloma, reticulin fibrosis |
| Immunology | ANA, rheumatoid factor |
| Organ function | ECHO, pulmonary function |
| Purpose . | Tests . |
|---|---|
| Inflammatory response | CBC, renal function, liver function, CRP, ESR, fibrinogen, immunoglobulins & free light chains, albumin, ferritin* |
| Histopathology | Hypervascular/mixed cellularity/plasmacytic variant |
| Virologic status | HIV serology, HHV-8 qPCR (peripheral blood), EBER (lymph node), LANA-1 (lymph node) |
| Cytokine profile | IL-6, VEGF, sIL-2 receptor† |
| Imaging | CT-PET or CT neck, chest, abdomen, pelvis |
| Bone marrow evaluation | MGUS, myeloma, reticulin fibrosis |
| Immunology | ANA, rheumatoid factor |
| Organ function | ECHO, pulmonary function |
Workup should include excisional lymph node biopsy for histopathologic examination to confirm features consistent with iMCD, establish histopathologic variety, and to rule out EBV and HHV-8 infection by EBER and LANA-1 staining. Blood work is helpful to exclude HIV infection, autoimmune disorders, and monoclonal gammopathy of undetermined significance (MGUS)/myeloma as well as measure inflammatory markers, determine organ function, and evaluate cytokines levels, including IL-6 and VEGF.
ANA, antinuclear antibody; CBC, complete blood count; EBER, Epstein-Barr virus-encoded small RNAs; ECHO, echocardiogram; LANA, latency-associated nuclear antigen; qPCR, quantitative polymerase chain reaction; sIL-2, soluble interleukin-2.
Ferritin is measured as an acute phase reactant.
Soluble IL-2 receptor marks T-cell activation. CT and CT-PET scanning help to visualize the extent of the disease. Bone marrow examination can exclude a concomitant plasma cell dyscrasia and screen for megakaryocyte hyperplasia and reticulin fibrosis often observed in TAFRO-iMCD. The diagnostic criteria have recently been published.23 Additional organ assessment may be needed in severe cases.