Interleukin-6-producing Intravascular Large B-cell Lymphoma with Lymphadenopathy Mimicking the Histology of Multicentric Castleman Disease

Shiroshita, Kohei; Kikuchi, Taku; Okayama, Masanobu; Kasahara, Hidenori; Kamiya, Takahiro; Shioiri, Takayuki; Kurose, Nozomu; Yasukawa, Masaki; Okamoto, Shinichiro

doi:10.2169/internalmedicine.5046-20

Cited by 6 publications

(4 citation statements)

References 26 publications

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“…In contrast, IgG4-related disease is not characterized by high fever or markedly elevated CRP. Furthermore, patients with lymphomas, such as angioimmunoblastic T cell lymphoma and intravascular large B cell lymphoma [ 21 ], accompanied by hyper-cytokinemia and generalized inflammation, may have MCD-like clinical symptoms and satellite lymph node biopsies may yield MCD-like histopathological findings. Symptoms and histopathology may be similar in patients with cytokine-producing cancers and sarcomas [ 22 ]; various collagen vascular diseases, such as systemic lupus erythematosus, Sjögren’s syndrome [ 23 ], and vasculitis syndrome; and various infections accompanied by hyper-inflammation.…”

Section: Cytokine Storm and Hyper-il-6 Syndrome (Fig 2 )mentioning

confidence: 99%

Castleman disease and TAFRO syndrome

et al. 2022

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Although Castleman disease was first described in 1956, this disease includes various conditions, including unicentric Castleman disease with hyaline vascular histology, human herpesvirus-8 (HHV-8) related multicentric Castleman disease, idiopathic multicentric Castleman disease, and mimics of Castleman disease associated with other conditions. To date, Castleman disease remains incompletely understood due to its rareness and difficulties in clinical and pathological diagnosis. TAFRO syndrome was reported in Japan in 2010. Because lymph node histology is similar in patients with TAFRO syndrome and Castleman disease, TAFRO syndrome is described as a related disorder of Castleman disease. Clinically, however, these conditions differ markedly. Although elevated interleukin-6 (IL-6) expression is characteristic of Castleman disease, increased expression of IL-6 may occur in patients with other diseases, making elevated IL-6 unsuitable for differential diagnosis. Further understanding of these disorders requires the identification of novel disease-specific biomarkers. This review article therefore outlines the characteristics of Castleman disease and TAFRO syndrome.

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Section: Cytokine Storm and Hyper-il-6 Syndrome (Fig 2 )mentioning

confidence: 99%

Castleman disease and TAFRO syndrome

et al. 2022

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“…These Castleman-like histologic features likely represent a nonspecific immune response to the immunologic stimuli in the tumor microenvironment. In some cases, the Castleman-like histology is caused by cytokine-producing lymphoma cells, as reported in cases of intravascular large B-cell lymphoma secreting IL-6 97 . Angioimmunoblastic T-cell lymphoma often shows atrophic germinal centers and proliferation of high endothelial venules, which may mimic the hyaline vascular or hypervascular subtype of iMCD.…”

Section: Other Disease Conditions With Histopathology Mimicking Imcdmentioning

confidence: 87%

Multicentric Castleman disease and the evolution of the concept

et al. 2021

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Summary The term multicentric Castleman disease (MCD) encompasses a spectrum of conditions that share some overlapping clinicopathological manifestations. The fundamental pathogenetic mechanism involves dysregulated cytokine activity, causing systemic inflammatory symptoms as well as lymphadenopathy. Some of the histological changes in lymph nodes resemble the histology of unicentric Castleman disease (UCD). However, based on current knowledge, the use of this shared nomenclature is unfortunate, since these disorders differ in pathogenesis and prognosis. In Kaposi sarcoma-associated herpesvirus (KSHV)-associated MCD, cytokine overactivity is caused by viral products, which can also lead to atypical lymphoproliferations and potential progression to lymphoma. In idiopathic MCD, the hypercytokinemia can result from various mechanisms, which ultimately lead to different constellations of clinical presentations and varied pathology in lymphoid tissues. The authors review the evolving concepts and definitions of the various conditions under the eponym of multicentric Castleman disease.

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“…Due to the variety of the pathological manifestations of CD, sometimes it is di cult to distinguish CD from thymoma, FDCS, and Hodgkin's lymphoma [9][10][11] . The diagnosis of iMCD requires the exclusion of infections, autoimmune diseases, primary or acquired immunode ciency syndromes, and malignancies, of which the differentiation of PC-MCD and IgG4RD had always been a challenge in clinical practice [12][13][14][15] . Both of them have an appearance of generalized lymph node enlargement and extra-nodal involvement, and iMCD patients may have high serum IgG4 levels, whilst some IgG4RD patients may have CD-like pathological changes 16 .…”

Section: Discussionmentioning

confidence: 99%

A Real-world Study on a Large Retrospective Cohort of Castleman Disease from a Single Center

Guo

Lin

Wang

et al. 2022

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Castleman disease(CD) is a rare lymphoid tissue proliferative disease with an increasing focus on etiology and treatment in recent years. The consensus on CD diagnosis and treatment has been published by several organizations, playing an important role in promoting cooperation on CD managements and investigations among different teams. Based on the last 15-years retrospective real-world data from Peking University First Hospital (PKUFH), we re-classified and re-evaluated the clinical and pathological information of patients with pathologically suspected diagnosis of CD. A total of 203 patients were included in our study, in which the diagnosis of CD was confirmed in 189 cases, including 118 patients with unicentric CD (UCD, n = 118, 62.4%) and 71 patients with multicentric CD (MCD, n = 71, 37.6%). 44.1% (n = 52) of UCDs in our cohort were complicated with Paraneoplastic Pemphigus (PNP). The treatment of UCD is mostly surgical resection, with a 5-year overall survival(OS) 88.1%. Patients with PNP had a poorer prognosis than those without PNP [82.9% (95% CI 123–178) vs 92.8% (95% CI 168–196), log-rank P = 0.041). The rate of concurrent systemic symptoms was 74.6% (n = 53), and renal involvement occurred in 49.3% (n = 35) MCD patients. The MCD treatments were mainly chemotherapy regimens, with a 5-year OS of 77.6% (95% CI, 143–213). In conclusion, UCDs have a better overall prognosis than MCDs. But the prognosis of those complicated with PNP was poor. Differential diagnosis of MCD is difficult. MCD treatment in China is heterogeneous. The inaccessibility of anti-IL-6-targeted drugs in China may contribute to the poor prognosis of MCD.

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Interleukin-6-producing Intravascular Large B-cell Lymphoma with Lymphadenopathy Mimicking the Histology of Multicentric Castleman Disease

Cited by 6 publications

References 26 publications

Castleman disease and TAFRO syndrome

Castleman disease and TAFRO syndrome

Multicentric Castleman disease and the evolution of the concept

A Real-world Study on a Large Retrospective Cohort of Castleman Disease from a Single Center

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