Table 3.

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

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.