Treatment of castleman’s disease

Abstract

Castleman's disease (CD) was first described in 1954 and further defined in 1956 by Castleman. Since then much has been learned about the heterogeneity of this condition. Subsequently, three pathologic classifications have been developed (hyaline vascular variant, plasma cell variant, and mixed variant) and two clinical classifications (unicentric [unifocal or localized] and multicentric [multifocal or generalized]). The pathology found with the unicentric presentation is most commonly that of the HV variant. It responds well to surgical resection and is associated with a benign course. The multicentric presentation is rarely composed of lymph nodes with HV pathology, but rather with the plasma cell or mixed pathology. This presentation requires systemic therapy and prognosis is guarded. Associated systemic symptoms are common. There is an increased incidence of CD in patients with HIV. The human herpes virus-8 is associated with nearly all of the HIV-associated CD cases and nearly 50% of non-HIV cases. Interleukin (IL)-6 has also been shown to play a significant role in the pathogenesis of the disease. Paraneoplastic and autoimmune entities are not uncommon in the disorder. Variable benefit has been achieved with single agent chemotherapy, combination chemotherapy, interferon (IFN)-alpha, rituximab, anti-IL-6 receptor antibodies, and thalidomide. Patients with CD are at increased risk for developing frank malignant lymphoma.

Mixed-type Multicentric Castleman's Disease Developing during a 17-year Follow-up of Sarcoidosis

“…Although many treatments for MCD are available, including steroids, chemotherapy, antiviral agents, rituximab and tocilizumab (8,17), no standard therapy has yet been established. MCD is often refractory to steroids and chemotherapy.…”

Hyaline-Vascular Type Castleman’s Disease, Sarcoidosis, and Crohns Disease

“…Outcome is generally excellent [15,20]. Management of multicentric CD is more complex and involves managing concurrent infections (HIV with ART) along with rituximab, anti IL-6 agents, cytotoxic and radiation therapy [21].…”

Abdominal giant lymph node hyperplasia (Castleman’s disease): report of two cases

“…Treatment for multicentric Castleman's disease has been challenging and there are no standard therapeutic regimens today. As the disease involves lymph nodes at multiple locations, surgery is mainly used for the diagnosis and for debulking the disease in some patients [11]. Corticosteroids, chemotherapy, radiotherapy and immune therapy have been helpful [11,12].…”

“…As the disease involves lymph nodes at multiple locations, surgery is mainly used for the diagnosis and for debulking the disease in some patients [11]. Corticosteroids, chemotherapy, radiotherapy and immune therapy have been helpful [11,12]. Interferon (IFN)-alpha, rituximab, anti-IL-6 receptor antibodies, and thalidomide have been used in the management of multicentric Castleman's disease with some success [11,13,14].…”

“…Corticosteroids, chemotherapy, radiotherapy and immune therapy have been helpful [11,12]. Interferon (IFN)-alpha, rituximab, anti-IL-6 receptor antibodies, and thalidomide have been used in the management of multicentric Castleman's disease with some success [11,13,14]. Patients with multicentric Castleman's disease are at increased risk for developing frank malignant lymphoma such as non-Hodgkin lymphoma or Kaposi sarcoma.…”