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An Unusual Presentation of Plasma Cells - Castleman Disease: A Case Report
Insights
This case study highlights a rare instance of cardiac tamponade caused by Castleman disease. Diagnosis was confirmed through pericardial and lymph node biopsies, revealing Plasma Cells Castleman disease.
Area of Science:
- Cardiology
- Oncology
- Pathology
Background:
- A 76-year-old female presented with symptoms suggestive of cardiac tamponade, including dyspnea and physical asthenia.
- Initial assessment confirmed cardiac tamponade, necessitating surgical drainage of pericardial effusion.
Observation:
- Recurrent symptoms post-surgery led to a second drainage and pericardial biopsy, revealing serous pericarditis with chronic inflammation and xanthogranulomatous reaction.
- Further examination revealed systemic signs including cutaneous hyperpigmentation, nodular masses, and adenopathies, prompting extensive investigation.
Findings:
- Pathological and immunohistochemical analyses of skin, bone marrow, and lymph node biopsies confirmed the diagnosis of Castleman disease, specifically the Plasma Cells variant.
- CT scans indicated bilateral pleural effusion, minimal pericardial effusion, and multiple adenopathies.
Implications:
- This case underscores the importance of considering rare systemic diseases like Castleman disease in the differential diagnosis of cardiac tamponade and unexplained lymphadenopathy.
- Early and accurate diagnosis through comprehensive pathological examination is crucial for effective management and improved patient outcomes in such complex cases.
Abstract:
We present the case of a 76 year old female patient admitted in the Department of Cardiology for physical asthenia, profuse sweating and dyspnea with orthopnea for about one month. Clinical and paraclinical assessments performed at admission confirmed the diagnosis of cardiac tamponade. Surgical intervention was performed and 400 mL of clear effusion were drained. Post-operative evolution was marked by recurrence of symptoms, requiring after 3 weeks a new drainage of 600 mL of clear effusion, and biopsy of the pericardium was performed. Pathological exam described serous pericarditis with chronic inflammatory infiltrate, xanthogranulomatous reaction intricated in the pericardium and mesothelial hyperplasia. The patient was subsequently transferred to the Department of Internal Medicine for further investigations. Physical examination showed a patient with altered general status, pallor, vesicular murmur absent in both bases, presenting cutaneous hyperpigmentation at the level of the right hemi-abdomen and hip with posterior extension, and a peripheral indurated erythematous plaque. The patient presented nodular masses of 3 cm in the right latero-cervical and bilateral axillary regions, non-adherent to the superficial structures, as well as adenopathic blocks in both inguinal regions. CT scan of the thorax and abdomen showed moderate bilateral pleuresia, minimal pericardial effusion (15 mm) and multiple adenopathies on both sides of the diaphragm. Skin biopsy was performed, as well as bone marrow aspirate and excision of a right axillary lymph node. Pathological exams and immunohistochemistry tests confirmed the diagnosis of Plasma Cells Castleman disease.