Castleman Disease: A Multicenter Case Series from Turkey

Area of Science:

• Hematology
• Oncology
• Rare Diseases

Background:

• Castleman disease (CD), or angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder.
• CD has two main histological subtypes: hyaline vascular and plasma cell variants.
• It is classified as unicentric CD (UCD) or multicentric CD (MCD) based on lymph node involvement.

Purpose of the Study:

• To establish a national registry for Castleman disease in Turkey.
• To evaluate patient characteristics, treatment strategies, and clinical outcomes for CD in Turkey.
• To improve early recognition, treatment, and follow-up of CD patients.

Main Methods:

• Retrospective evaluation of 140 adult and pediatric patients diagnosed with CD.
• Inclusion criteria: lymph node or involved field biopsy results positive for CD.
• Data collected included demographics, clinical and laboratory findings, imaging, treatments, and outcomes.

Main Results:

• A total of 140 patients (73 UCD, 67 MCD) were analyzed.
• Hyaline vascular subtype was most common in both UCD and MCD.
• MCD patients showed higher rates of anemia, elevated acute phase reactants, and hypoalbuminemia; 13 MCD patients died during follow-up, while all UCD patients survived.

Conclusions:

• This study provides crucial data on CD patient characteristics and treatment strategies in Turkey.
• Findings can inform treatment decisions, particularly in resource-limited settings lacking access to specific therapies like siltuximab.
• Larger prospective studies are recommended for definitive conclusions on Castleman disease management.