AUTOINFLAMMATORY DISEASES IN HEMATOLOGY FROM DIAGNOSIS TO TREATMENT

AUTOINFLAMMATORY_DISEASES_IN_HEMATOLOGY_FROM_DIAGNOSIS_TO_TREATMENTkapak

CASTLEMAN DISEASE

Gülcan Kökcü

Ankara University, Faculty of Medicine, Department of Hematology, Ankara, Türkiye

Kökcü G. Castleman Disease. In: Kurt Yüksel M, editor. Autoinflammatory Diseases in Hematology from Diagnosis to Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.87-105.

ABSTRACT

Castleman disease (CD) is a heterogeneous group of lymphoproliferative disorders. CD is clinically classified based on lymph node distribution: unicentric CD (UCD), multicentric CD (MCD), oligocentric. CD is also divided based on etiology: Human Herpes Virus-8-associated (HHV-8) MCD, POEMS-associated MCD, and idiopathic MCD (iMCD). Idiopathic MCD is further subdivided based on clinical subtype as iMCD-TAFRO (Thrombocytopenia, Anasarca, Fever, Reticulin fibrozis, Organomegaly), iMCD-IPL (Idiopathic Plasmacytic Lymphadenopathy) and iMCD-NOS (Not Otherwise Specified). While HHV-8 plays a role in etiology in some patients, the triggering factor in idiopathic MCD patients is unknown. Interleukin-6 (IL-6) and some cytokines are linked to causing systemic symptoms. Anti–interleukin–6 therapies are highly effective for many iMCD patients; however, additional therapies are necessary for those with refractory cases.

Keywords: Castleman disease; Multicentric castleman disease; Anti-interleukin-6 therapy; Siltuximab

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