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Castleman Disease: A Multicenter Case Series from Turkey

Eren Gündüz1, Hakkı Onur Kırkızlar2, Elif Gülsüm Ümit2

  • 1Eskişehir Osmangazi University Faculty of Medicine, Department of Hematology, Eskişehir, Turkey

Turkish Journal of Haematology : Official Journal of Turkish Society of Haematology
|February 18, 2022
PubMed
Summary

This study establishes a Turkish Castleman disease (CD) registry, detailing patient characteristics and treatment outcomes for unicentric CD (UCD) and multicentric CD (MCD). Findings aid early recognition and treatment decisions, especially where siltuximab is unavailable.

Keywords:
Castleman diseaseUnicentricMulticentricTreatment

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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.