Castleman's Disease: From Basic Mechanisms to Molecular Therapeutics

Abstract

Castleman's disease is a rare lymphoproliferative disorder in which there has been recent progress in elucidating underlying mechanisms with potential therapeutic implications. Unicentric Castleman's disease is an indolent condition that is often treated with local approaches. In contrast, patients with multicentric Castleman's disease (MCD) have a less favorable prognosis and require systemic treatment. Cytotoxic chemotherapy, with its attendant risk for toxicity, has been widely used to treat MCD, with variable efficacy. The discovery of putative etiologic factors and targets in MCD, particularly human herpes virus 8, CD20, and interleukin (IL)-6, has been translated into the use of rituximab and anti-IL-6-based therapy, as well as antiviral agents. In this article, we review the current state of the art of our understanding of Castleman's disease and its treatment and we provide insight into future treatment strategies based on disease biology. The Oncologist 2011;16:497-511

Clinical and morphological features of different types of Castleman’s disease

“…Данный вирус чаще выявляется у больных БК, серопозитивных по ВИЧ (по данным Н. El-Osta и соавт. [14], в 100% случаев), одна-ко описаны случаи инфицированности HHV-8 и у сероне-гативных по ВИЧ пациентов [15][16][17][18][19][20][21].…”

Selective killing of Kaposi's sarcoma-associated herpesvirus lytically infected cells with a recombinant immunotoxin targeting the viral gpK8.1A envelope glycoprotein

“…23 While standardized treatment modalities have not yet been established, several promising approaches are being pursued. 16,24,25 Antineoplastic chemotherapy with single or combination agents has yielded clinical remissions, though the responses are typically transient and drug toxicities limit extended use. Successes have been reported with immunotherapy using monoclonal antibodies (mAbs) tocilizumab (Actemra ® ), 26 and siltuximab 27 against interlekin-6 or rituximab against the B-cell antigen CD20, 18,[28][29][30][31] though the latter agent has been complicated by aggravation of KS lesions.…”

Single-Region Castleman's Disease: Case report

“…Klinikte tek anatomik bölge tutulumu "tek-bölgeli", birden fazla anatomik bölge tutulumu ise "çok-bölgeli" tip olarak adlandırılır. İlk kez 1978'de bildirilen çok-bölgeli CH, klinik ve laboratuvar bulgularıyla daha çok yaygın bir hastalık şeklinde seyretmesiyle tek-bölgeli hastalık-tan belirgin şekilde farklıdır ve kesin belirlenmiş bir tedavi şeması yoktur (2,7,8) .…”