Recent Advances in Castleman Disease

Abstract

Background: Castleman disease (CD) encompasses a spectrum of rare disorders with characteristic histopathological features. Unicentric Castleman disease (UCD) is a benign, local hyperplasia of lymphoid tissue that is usually curable. Multicentric Castleman disease (MCD) manifests as a potentially life-threatening systemic disease with complex symptomatology which is mostly due to an overproduction of interleukin-6 (IL-6) or dysregulation of IL-6-related signaling pathways. From a therapeutic perspective, it is important to distinguish idiopathic MCD (iMCD) from those cases that are associated with the human herpes virus 8 (HHV-8+MCD). Summary: During recent years, it has become increasingly clear that even HHV-8-negative MCD is not a homogeneous entity and that there are clinically distinct variants. International consensus guidelines for diagnosis and treatment have been developed for iMCD and UCD. Key messages: We herein summarize recent advances in diagnosis, treatment and novel insights into the pathogenesis of this disease.

Castleman Disease in an Older Patient With the Onset of Right Pleural Effusion

“…Polyclonal gammopathy with elevated levels of immunoglobulin G (IgG) and IgA was consistent with the literature on MCD, supporting an eventual diagnosis [ 12 ]. However, these findings are nonspecific and can be observed in many other disorders, further highlighting diagnostic challenges [ 1 , 13 ]. The diagnosis of CD is based on tissue histopathology.…”

“…The diagnosis of CD is based on tissue histopathology. Despite multiple clinical indicators pointing to other diagnoses, a cervical lymph node biopsy confirms the diagnosis [ 1 ]. This highlighted the need for a biopsy in unexplained chronic inflammatory conditions, particularly when the clinical picture does not fit a common diagnosis.…”

“…Castleman disease (CD) is a rare, refractory lymphoproliferative disorder first reported by Benjamin Castleman in 1956 [ 1 ]. Histologically, CD is classified into two types: hyaline vascular type (HV type), characterized by lymphoid follicle proliferation, marked capillary proliferation, and hyalinization both inside and outside the follicles, and plasma cell type (PC type), characterized by clustering of numerous plasma cells between follicles [ 2 ].…”

“…There was no discernible sex disparity [ 5 ]. Furthermore, based on the presence or absence of lymph node metastasis, CD is classified as unicentric CD (UCD) or multicentric CD (MCD) [ 1 ]. Although CD can manifest in a broad age range, from children to older individuals, MCD tends to be prevalent around the age of 50, and is slightly more common in males.…”

Castleman disease. A rare clinical case of retroperitoneal tumor localization in an elderly patient

“…При мультицентрической форме манифестирование в большинстве случаев происходит в 50-60 лет, в ряде случаев имеется вторичное поражение почек и лёгких, паранеопластическая пузырчатка, периферическая нейропатия. Лабораторно выявляются анемия, тромбоцитоз, гипергаммаглобулинемия, повышенный С-реактивный белок [9][10][11][12].…”