Expert consensus on the integrated diagnosis of idiopathic multicentric Castleman disease

Area of Science:

• Hematology
• Pathology
• Rheumatology

Background:

• Idiopathic multicentric Castleman disease (iMCD) is a rare systemic condition.
• Differential diagnosis includes inflammatory, autoimmune, and neoplastic disorders.
• Lymph node histopathology is crucial for diagnosing Castleman disease.

Purpose of the Study:

• To standardize the diagnosis of Castleman disease.
• To provide recommendations for the integrated diagnosis of iMCD.
• To establish guidelines for sample handling and result interpretation.

Main Methods:

• A multi-disciplinary consensus document was created by 53 experts from three medical societies (SEMI, SEHH, SEAP).
• The Delphi method was employed to reach consensus on diagnostic recommendations.
• Recommendations cover initial clinical, laboratory, and imaging studies.

Main Results:

• Specific recommendations for integrated iMCD diagnosis were developed.
• Guidelines for optimal sample acquisition for histopathology were established.
• Standardized procedures for laboratory testing and result reporting were defined.

Conclusions:

• Standardized diagnostic criteria and procedures are essential for iMCD.
• Expert consensus provides a framework for consistent and accurate diagnosis.
• This consensus aims to improve patient management through reliable diagnosis.