Diagnosing Castleman’s Disease

Because it resembles many other disorders, early identification of Castleman’s Disease can be challenging.1 The lymph node abnormalities can resemble those in conditions such as chronic viral infections, rheumatoid arthritis, lupus, Sjögren syndrome, HIV infection, lymphoma, and drug sensitivity.2

Diagnosis of Castleman’s Disease involves a work-up to rule out other possible causes, and to identify histology and laboratory abnormalities that are characteristic of the disease.3

Work-up for Castleman’s Disease3

Excisional lymph node biopsy

Pathology, exclude clonal malignancy

Virology

HIV and HHV-8 serology with quantitative PCR, if positive

Cytokines

IL-6, VEGF

Acute phase reactants

WESR, CRP, fibrinogen

General

CBC, renal and liver function

Immunology

ANA, others as indicated 

Endocrine abnormalities

Thyroid function tests, others as indicated

Plasma cell dyscrasias

Immunoglobulins, urine and protein electrophoresis and immunofixation, light chains, bone marrow, 24-hour urine for protein quantification

Organ function

Echocardiogram, pulmonary function

Imaging

CT of neck, chest, abdomen, pelvis; CT-PET

Neurology

Nerve conduction as indicated

ANA = anti-nuclear antibody; CBC = complete blood count;
CRP = C-reactive protein; CT = computed tomography;
CT-PET = computed tomography–positron emission tomography;
HHV-8 = human herpesvirus-8; PCR = polymerase chain reaction;
VEGF = vascular endothelial growth factor; WESR = Westergren erythrocyte sedimentation rate.

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  1. Bonekamp D, Horton KM, Hruban RH, Fishman EK. Castleman disease: the great mimic. Radiographics. 2011;31(6):1793-1807.
  2. Casper C. The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005;129(1):3-17.
  3. van Rhee F, Stone K, Szmania S, Barlogie B, Singh Z. Castleman's disease in the 21st century: an update on diagnosis, assessment, and therapy. Clin Adv Hematol Oncol. 2010;8(7):486-498.

This short video will provide additional information about the mechanism of disease.