Recommended workup of UCD
| Assessment category . | Tests . |
|---|---|
| Imaging | CT-PET or CT of neck, chest, abdomen, pelvis |
| Histopathology | HV/mixed/PC |
| Immunostaining | Staining for EBER, LANA-1, and lymphoma or plasmacytoma markers if suspected |
| Clonality | IgH gene rearrangement, TCR clonality |
| Virological status | HIV serology, HHV-8 qPCR (peripheral blood) |
| Inflammatory response | CBC, renal function, liver function, CRP, ESR, fibrinogen, immunoglobulins and free light chains, albumin |
| Autoimmunity (if suspected) | ANA, rheumatoid factor |
| Monoclonal gammopathy (if suspected) | Serum protein electrophoresis with immunofixation, quantitative immunoglobulins, and bone marrow if indicated |
| Organ function (depending on location relative to neighboring vital structures) | ECHO, pulmonary function |
| Assessment category . | Tests . |
|---|---|
| Imaging | CT-PET or CT of neck, chest, abdomen, pelvis |
| Histopathology | HV/mixed/PC |
| Immunostaining | Staining for EBER, LANA-1, and lymphoma or plasmacytoma markers if suspected |
| Clonality | IgH gene rearrangement, TCR clonality |
| Virological status | HIV serology, HHV-8 qPCR (peripheral blood) |
| Inflammatory response | CBC, renal function, liver function, CRP, ESR, fibrinogen, immunoglobulins and free light chains, albumin |
| Autoimmunity (if suspected) | ANA, rheumatoid factor |
| Monoclonal gammopathy (if suspected) | Serum protein electrophoresis with immunofixation, quantitative immunoglobulins, and bone marrow if indicated |
| Organ function (depending on location relative to neighboring vital structures) | ECHO, pulmonary function |
Workup should include excisional lymph node biopsy for histopathological examination to confirm features consistent with UCD, establish histopathological subtype, and rule out Epstein-Barr virus and HHV-8 infection by Epstein-Barr virus–encoded small RNAs (EBER) and latency-associated nuclear antigen-1 (LANA-1) staining. Blood work is helpful to exclude HIV infection, autoimmune disorders, and monoclonal gammopathy of undetermined significance/myeloma as well as measure inflammatory markers and determine organ function. CT and CT–positron emission tomography (PET) scanning help to visualize the extent of the disease. Bone marrow examination can exclude a concomitant plasma cell dyscrasia.
ANA, antinuclear antibody; CBC, complete blood count; ECHO, echocardiogram; IgH, immunoglobulin H; qPCR, quantitative polymerase chain reaction; TCR, T-cell receptor.