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Soluble Mediators of Cellular Cooperation: The Cytokines
Published in Constantin A. Bona, Francisco A. Bonilla, Textbook of Immunology, 2019
Constantin A. Bona, Francisco A. Bonilla
Clinical considerations. An elevated level of serum IL-6 has been associated with several disease processes. Castleman’s disease is a localized or systemic anomalous proliferation of polyclonal plasma cells in lymph nodes or other lymphoid tissue. Over-production of IL-6 in germinal centers has been implicated in the etiology of this disease. Autoimmune phenomena (see Chapter 12) are frequently associated with a cardiac myxoma (a type of connective tissue tumor); some myxomas produce IL-6. The synovial fluid of patients with rheumatoid arthritis contains elevated levels of IL-6. Both synovial cells and infiltrating T cells are stained with labelled antibodies binding IL-6. Kidney mesangial cells may also produce IL-6, and patients with mesangial proliferative glomerulonephritis have increased amounts of IL-6 in their urine. Renal adenocarcinoma (also called hypernephroma or Grawitz tumor) has also been shown to secrete IL-6. Increased IL-6 production has been observed in T cells from patients with Behcet’s disease when stimulated with streptococcal antigens.
Benign Lymph Node Lesions
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
The multicentric type of Castleman’s disease is important to recognize because the patient may have a declivitous course. The median survival of patients with the multicentric type is 26 months, with a survival range from 8 to 170 months. In contrast to the localized type of Castleman’s disease, the multicentric type tends to occur predominantly in an older age group, although cases have been reported in children. The multicentric type involves multiple peripheral and visceral lymph nodes; histologically these lymph nodes are typically of the plasma cell type, although in the latter stages of the process, hyaline vascular forms occur. Patients with the multicentric form of Castleman’s disease may develop malignant lymphoma, Kaposi’s sarcoma, plasmacytomas, POEMS syndrome, and glomeruloid hemangiomas.
Benign Neck Disease
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Ricard Simo, Jean-Pierre Jeannon, Enyinnaya Ofo
Castleman disease is a rare entity which is characterized by hyperplasia of lymph nodes and capillary proliferation that usually affects adolescents and young adults. Three histological patterns have been described: hyaline vascular type, plasma cell type and mixed type. Two clinical types have been identified; a localized type (ECL) usually of benign clinical course and a multicentric type (ECM) of worst prognosis as it may lead to the development of non-Hodgkin lymphoma.93–95
POEMS syndrome: origination from clonal plasma cells or B cells?
Published in Hematology, 2023
Lu Zhou, Jinfeng Lu, Zenghua Lin, Xinfeng Wang, Lan Luo, Chenhui Wang, Lemin Hong, Ruirong Xu, Hongmin Huang
Recently, a partial overlap between chromosomal abnormalities in POEMS syndrome and the monoclonal gammopathy of undetermined significance (MGUS) was shown [21]. As a result, it seemed that POEMS syndrome originated from clonal plasma cells. However, RNA sequencing revealed a transcription profile specific to POEMS syndrome when compared with normal plasma cells, MGUS, and multiple myeloma [21]. Moreover, the level of plasma clone and M-protein produced (mostly IgA λ) are significantly lower in POEMS syndrome than that of multiple myeloma [22]. It is not clear why high levels of IgA λ light chain in multiple myeloma would not manifest POEMS-like symptoms. Castleman disease is characterized by systemic inflammation, lymph node pathology and multi-organ dysfunction resulting from hypercytokinemia, and it can be associated with POEMS syndrome [2,22]. Several cases have shown that lymphoma including DLBCL, T-cell lymphoma, Waldenström macroglobulinemia, primary cutaneous diffuse large B-cell lymphoma (leg type), follicular lymphoma can be manifested as POEMS syndrome [4–8] (shown in Table 1). Furthermore, cases of POEMS syndrome without M-protein and clonal plasma cells were also reported, which were called variant POEMS syndrome [23,24] (shown in Table 1). These facts suggest that further study is needed to reveal the origination of POEMS syndrome.
Intrapericardial pneumonectomy for unicentric hilar castleman disease
Published in Acta Chirurgica Belgica, 2021
Murat Kara, Berker Ozkan, Melike Ulker, Deniz Tugcu, Gulcin Yegen
Castleman disease is a rare benign lymphoproliferative disorder of the lymph nodes with an unknown etiology. It was first reported by Castleman and Towne from Massachusetts General Hospital in 1954 [1]. The primary pathology is angiofollicular lymph node hyperplasia. Histologically, it has three forms as hyaline vascular, plasma cell and mixed types. Clinically, it presents with either unicentric or multicentric disease. Unicentric Castleman Disease is usually of the hyaline vascular type, whereas multicentric disease is usually the plasma cell or mixed type. The unicentric form is often a localized solitary tumor, whereas the multicentric form is characterized by generalized lymphadenopathy and systemic symptoms. The unicentric form is mostly located at the mediastinum and abdomen; however, intrapulmonary localization with parenchymal involvement is extremely rare. Preoperative diagnosis is often a challenge before the histologic examination of the lymph nodes, and no specific radiologic diagnostic criteria exist for Castleman disease. Thus, surgical resection becomes the diagnosis and treatment of choice with favorable results for the unicentric form of Castleman disease with intrapulmonary involvement [2].
The benefits and prospects of interleukin-6 inhibitor on systemic sclerosis
Published in Modern Rheumatology, 2019
IL-6 is also known to be involved in the pathogenesis of various diseases. Castleman disease is a benign tumor in which the symptoms are chronic fever and increases of CRP, SAA level, platelet cell count, and γ-globulins. These symptoms are explainable in view of the functions of IL-6. Indeed, the level of IL-6 in patients with Castleman disease was reported to be high, but decreased after resection of the focal lymph node. Immunohistochemical analysis also revealed positivity for IL-6 staining in the germinal center of the resected lymph node [9]. Rheumatoid arthritis (RA) shows swelling and pain in the vicinity of inflammation, and CRP elevation or blood sediment increase has been observed in blood tests in patients with this condition. These phenomena are thought to be caused by the biological functions of IL-6 [10].