Castleman Disease Workup

Updated: Jul 13, 2017
  • Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Workup

Approach Considerations

Castleman disease should be suspected in patients who present with peripheral lymphadenopathy, fever, splenomegaly, and an elevated C-reactive protein (CRP) level. The diagnosis is confirmed by histologic examination of needle biopsy specimen from an involved lymph node. Histopathologically, Castleman disease is identfied by polyclonal nodal expansions that usually leave the structure of the underlying lymph node at least partially intact and histologic features that are consistent with the hyaline vascular or plasma cell variants.

IgH gene rearrangement studies should be performed on the lymph node specimen to rule out a clonal disorder (eg, occult lymphoma).

Chest radiography and other imaging findings are nonspecific.

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Imaging Studies

In patients with multicentric Castleman disease (MCD), the chest radiograph may show bilateral reticular or ground-glass opacities, mediastinal widening, and/or bilateral pleural effusions.

Chest computed tomography (CT) scans may demonstrate lymphadenopathy of multiple enlarged mediastinal and hilar lymph nodes (1-3 cm in diameter). Lung parenchymal findings may be seen, including subpleural nodules, interlobular septal thickening, peribronchovascular thickening, ground-glass opacities, and patchy rounded areas of consolidation. Small to moderate bilateral pleural effusions may also be present.

CT scanning of the neck, abdomen, and pelvis, with and without contrast, may also be helpful.

MCD is positron emission tomography (PET)–avid, usually with a relatively low standardized uptake value (SUV) of 2.5-5. [9] Currently, PET scanning is more often used for staging.

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Laboratory Studies

Laboratory studies used in the workup of Castleman disease, and characteristic findings, include the following:

  • Complete blood cell (CBC) count – Anemia (usually mild to moderate, occasionally, the hemoglobin level is <8 g/dL), thrombocytosis
  • Liver function tests (LFT) – Hypoalbuminemia
  • Serum protein electrophoresis, with immunofixation and quantitative immunoglobulins – Polyclonal hypergammaglobulinemia
  • Erythrocyte sedimentation rate (ESR) – Usually elevated
  • Serologies for hepatitis B, human herpesvius–8 (HHV-8), and HIV; with quantitative assays if initial results are positive
  • Interleukin-6 (IL-6), lactate dehydrogenase (LDH), and CRP levels – Usually elevated
  • Vascular endothelial growth factor (VEGF) – May be elevated
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Lymph Node Biopsy

CT-guided Needle Biopsy of Affected/Maximally Enlarged Easily-Accessible NodeBiopsy samples of an affected lymph node are sent for histopathology, flow cytometry, cytogenetics, fluorescent in situ hybridization (FISH) for lymphoma studies, and B-cell gene rearrangement studies.

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