Treatment response and prognosis of POEMS syndrome coexisting with Castleman disease

Suichi, Tomoki; Misawa, Sonoko; Sekiguchi, Yukari; Shibuya, Kazumoto; Tsuneyama, Atsuko; Suzuki, Yôichi; Nakamura, Keigo; Kondo, Hiroki; Kuwabara, Satoshi

doi:10.1016/j.jns.2020.116771

Cited by 8 publications

(10 citation statements)

References 29 publications

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“…Because the diagnosis of CD depends on histological findings, hyper-inflammation, and polyclonal lymphoproliferation conditions, patients with suspicion of malignant lymphoma without proven monoclonality can be diagnosed with MCD. Lymphadenopathy may also be present in patients with TAFRO syndrome, POEMS syndrome [ 19 ], and IgG4-related disease [ 20 ]. Lymph node biopsy of these patients may result in a histologic diagnosis of MCD or a condition undistinguishable from PC-, mixed-, or hyper-vascular-type MCD.…”

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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“…An important clinical feature is peripheral polyneuropathy, which is probably caused by VEGF and is often absent in iMCD. Although often milder than in POEMS syndrome alone [56], neuropathic symptoms in CD should raise the suspicion of a POEMS-associated MCD. This also applies to cutaneous hyperpigmentations, which, however, should be distinguished from cherry hemangiomatosis that are associated with iMCD.…”

Section: Poems-associated MCDmentioning

confidence: 96%

“…Therapy includes not only agents such as melphalan and dexamethasone but also lenalidomide and bortezomib. Response to thalidomide plus dexamethasone appears to be better than in isolated POEMS syndrome [56]. An autologous stem cell transplantation may also be considered in younger patients under 65 years of age, especially in patients with osteosclerotic lesions or peripheral neuropathy [71].…”

Section: Poems-associated MCDmentioning

confidence: 99%

Recent Advances in Castleman Disease

et al. 2022

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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.

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“…The features, prognosis, and management of each main subtypes of CD are summarized in Fig. 1 [17][18][19][20][21][22].…”

Section: Introductionmentioning

confidence: 99%

“…Diagnosis of HHV-8 associated MCD generally requires HHV-8 detection, Fig. 1 Summary of the main types of Castleman disease [17][18][19][20][21][22]. UCD, unicentric Castleman disease; MCD, multicentric Castleman disease; HHV-8, human herpesvirus-8; iMCD, idiopathic multicentric Castleman disease; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes lymphadenopathy in multiple regions, and plasmablastic histopathologic findings on lymph node biopsy [24].…”

Section: Introductionmentioning

confidence: 99%

Emerging role of 18F-FDG PET/CT in Castleman disease: a review

et al. 2021

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Castleman disease (CD) describes a group of rare hematologic conditions involving lymphadenopathy with characteristic histopathology and a spectrum of clinical abnormalities. CD is divided into localized or unicentric CD (UCD) and multicentric CD (MCD) by imaging. MCD is further divided based on etiological driver into human herpesvirus-8-associated MCD, POEMS-associated MCD, and idiopathic MCD. There is notable heterogeneity across MCD, but increased level of pro-inflammatory cytokines, particularly interleukin-6, is an established disease driver in a portion of patients. FDG-PET/CT can help determine UCD versus MCD, evaluate for neoplastic conditions that can mimic MCD clinico-pathologically, and monitor therapy responses. CD requires more robust characterization, earlier diagnosis, and an accurate tool for both monitoring and treatment response evaluation; FDG-PET/CT is particularly suited for this. Moving forward, future prospective studies should further characterize the use of FDG-PET/CT in CD and specifically explore the utility of global disease assessment and dual time point imaging.Trial registration ClinicalTrials.gov, NCT02817997, Registered 29 June 2016, https://clinicaltrials.gov/ct2/show/NCT02817997

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Treatment response and prognosis of POEMS syndrome coexisting with Castleman disease

Cited by 8 publications

References 29 publications

Castleman disease and TAFRO syndrome

Castleman disease and TAFRO syndrome

Recent Advances in Castleman Disease

Emerging role of 18F-FDG PET/CT in Castleman disease: a review

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