From the Guidelines
The workup for Castleman disease typically involves a comprehensive approach including laboratory tests, imaging studies, and tissue biopsy, with a focus on identifying lymphadenopathy, assessing disease extent, and evaluating for associated conditions such as POEMS syndrome and lymphoma. Initial evaluation should include:
- Complete blood count
- Comprehensive metabolic panel
- Inflammatory markers (ESR, CRP)
- Serum protein electrophoresis
- Testing for HIV and HHV-8 virus Imaging studies such as CT scan of the chest, abdomen, and pelvis with contrast are essential to identify lymphadenopathy and assess disease extent 1. PET-CT may be useful for evaluating metabolic activity of affected lymph nodes. The definitive diagnosis requires excisional lymph node biopsy with histopathological examination, which will show characteristic features like follicular hyperplasia, vascular proliferation, and plasma cell infiltration. Additional specialized testing may include IL-6 levels, VEGF levels, and flow cytometry. Bone marrow biopsy may be indicated in cases with hematologic abnormalities, as recommended by the ESMO clinical practice guidelines for diagnosis, treatment, and follow-up of follicular lymphoma 1. This comprehensive workup is necessary because Castleman disease has different variants (unicentric vs. multicentric) and can be associated with other conditions, requiring accurate diagnosis for appropriate treatment planning. The multicentric form particularly requires thorough evaluation due to its association with POEMS syndrome, paraneoplastic manifestations, and increased risk of lymphoma.
From the Research
Diagnostic Approach
The workup for Castleman's disease (Angiofollicular Lymph Node Hyperplasia) involves a combination of clinical evaluation, imaging studies, and histopathological examination.
- The diagnostic criteria for Castleman's disease include major criteria such as histopathologic examination and minor criteria 2.
- Imaging techniques like CT, MRI, and PET-CT play a crucial role in diagnosis, staging, and differentiation from other diseases 3, 4.
- A positron emission tomography (PET) scan can help identify the optimal lymph node biopsy site for diagnostics 5.
Histopathological Examination
Histopathological examination is essential for the diagnosis of Castleman's disease.
- The disease can be classified into unicentric (UCD) and multicentric (MCD) types, with MCD further divided into HHV-8-associated and idiopathic (iMCD) forms 2, 4.
- The histopathological features of Castleman's disease can be classified as hyaline-vascular (HV), plasma cell (PC), or mixed cellularity (MC) type 2.
Imaging Studies
Imaging studies are important for the initial staging and response assessment of Castleman's disease.
- FDG-PET/CT can be used for initial staging and response monitoring of Castleman's disease, especially in patients with a multicentric form 3.
- Imaging techniques like CT, MRI, and PET-CT can help differentiate Castleman's disease from other diseases 4.
Clinical Evaluation
Clinical evaluation is crucial for the diagnosis and management of Castleman's disease.
- The clinical manifestations of Castleman's disease include fever, weight loss, night sweats, and organomegaly, with specific symptoms depending on the subtype 4.
- A thorough evaluation is necessary to differentiate Castleman's disease from other diseases, and treatment strategies like IL-6 blockade can improve patient outcomes 6.