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[Multicentric Castleman's disease combined with polyserositis and POEMS syndrome: case report and review article]
Sevtap Tugce Ulas1, Süha Dasdelen2,3
1Klinik für Radiologie, Campus Mitte, Charité - Universitätsmedizin Berlin, Berlin, Deutschland.
Insights
Castleman disease (CD) is a rare disorder of lymphoid hyperplasia. This case highlights a delayed diagnosis of CD presenting as refractory polyserositis, emphasizing the need for timely recognition.
Area of Science:
- Immunology
- Oncology
- Pathology
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder.
- Etiologies include autoimmune, infectious, autoinflammatory, and paraneoplastic conditions.
- Cytokine dysregulation, notably IL-6 and VEGF, is a common feature.
Observation:
- A 79-year-old patient presented with refractory polyserositis.
- Diagnosis was significantly delayed, occurring after more than 8 years of symptoms.
- The patient's presentation mimicked other conditions, complicating early identification.
Findings:
- The case illustrates the diagnostic challenges posed by heterogeneous symptoms in Castleman disease.
- Refractory polyserositis can be a manifestation of Castleman disease.
- Timely diagnosis is crucial for appropriate management and improved outcomes.
Implications:
- This case underscores the importance of considering Castleman disease in patients with unexplained, refractory polyserositis.
- Enhanced awareness and diagnostic vigilance for CD are needed among clinicians.
- Prompt diagnosis and treatment of Castleman disease can significantly improve patient prognosis.
Abstract:
Castleman disease (CD) is a very rare disorder characterised by hyperplasia of the lymphoid tissue. The aetiology varies considerably and includes autoimmunological, infectious, autoinflammatory and paraneoplastic diseases (e.g. MGUS with POEMS syndrome). What they all have in common is usually a dysregulation/overproduction of certain cytokines and growth factors (including interleukin 6 and VEGF). The sum of these changes sometimes causes very heterogeneous symptoms and thus often makes early diagnosis difficult. The prognosis of unrecognised and untreated disease is very serious and has an average 5‑year survival rate of 55-77%. The present paper describes the case of a 79-year-old patient with refractory polyserositis who was correctly diagnosed after > 8 years.
