Clinical and morphological features of different types of Castleman’s disease

Melikyan, AL; Егорова, Е К; Kovrigina, А М; Subortseva, Irina N.; Gilyazitdinova, E A; Karagyulyan, S R; Силаев, М. А.; Гемджян, Э Г; Vg, Savchenko

doi:10.17116/terarkh201587764-71

Cited by 21 publications

(15 citation statements)

References 22 publications

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“…Nevertheless, these patients experienced a worse OS than the present series (Yu et al , ). Furthermore, four large iMCD case series reported 5‐year OS of 55% (Shin et al , ), 55% (Melikyan et al , ), 65% (Dispenzieri et al , ) and 77% (Seo et al , ). This variability in outcomes may reflect too short follow‐up in the present series, geographic differences, a « grey zone » in staging UCD versus iMCD, or pathophysiological misclassification.…”

Section: Discussionmentioning

confidence: 99%

The full spectrum of Castleman disease: 273 patients studied over 20 years

et al. 2017

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The spectrum of Castleman disease (CD) has considerably extended since its first description in 1956. Recently, an international collaborative working group has reached consensus on the diagnostic criteria and classification of CD. We herein report 273 patients with lymph node histopathology consistent with CD and investigate the newly established diagnostic criteria. Twenty of these patients with Castleman-like histopathology were removed from analyses, because they were diagnosed with an exclusionary disorder (18 with haematological malignancy). Among the 253 remaining patients, 57 were considered unicentric CD (UCD), 169 were multicentric CD associated with Human Herpesvirus 8 (HHV-8+MCD), including 140 patients with human immunodeficiency virus (HIV) infection and 29 patients without HIV infection, and 27 were HHV-8 negative/idiopathic multicentric CD (iMCD). 2-( F)fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography was useful in 62 patients for staging/classification of the disease and for excluding associated lymphoma. UCD was mainly associated with hyaline-vascular histopathological features, and most patients were asymptomatic. Of the 27 patients that we had originally diagnosed with iMCD, 26 met the newly established diagnostic criteria. Patients with iMCD and HHV-8+ MCD demonstrated similar characteristics, including fever, splenomegaly, cytopenia and inflammatory symptoms. However, the disease was more aggressive in HHV-8+ MCD, particularly in HIV-infected patients.

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Section: Discussionmentioning

confidence: 99%

The full spectrum of Castleman disease: 273 patients studied over 20 years

et al. 2017

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“…Even so, no patients in our cohort died. In contrast, the adult five‐year overall survival (OS) rate for patients with UCD is 91–98%, and the five‐year OS for patients with MCD has been reported to be as low as 55–77% . The discrepancy between adult and pediatric survival may be related to the aforementioned lower rate of underlying viral illness associated with CD in pediatrics, because HHV8‐ and HIV‐associated CD have significantly worse two‐year survival than patients with idiopathic MCD .…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, the adult five-year overall survival (OS) rate for patients with UCD is 91-98%, 6,7,9 and the five-year OS for patients with MCD has been reported to be as low as 55-77%. 7,9,[26][27][28] The discrepancy between adult and pediatric survival may be related to the aforementioned lower rate of underlying viral illness associated with CD in pediatrics, because HHV8-and HIVassociated CD have significantly worse two-year survival than patients with idiopathic MCD. 6 In addition, our small sample size may contribute to our lack of deaths; to this end, one institution has since had a patient die of MCD.…”

Section: Discussionmentioning

confidence: 99%

Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two‐site retrospective cohort study

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Endres

Campbell

et al. 2019

Pediatric Blood & Cancer

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Background Castleman disease (CD) is an uncommon lymphoproliferative disorder that is rare in pediatric populations; the literature describing this population is sparse. We sought to describe pediatric CD, including unicentric CD (UCD) and human herpes virus‐8 (HHV8)‐negative multicentric CD (MCD), in a multi‐institutional cohort. Methods We retrospectively reviewed 24 patients, aged 0 to 26 years at diagnosis, who were diagnosed with CD between January 1, 2005, and May 16, 2017, at two tertiary children's hospitals. Demographic and clinical data were collected. Results Most patients (75%, 18/24) presented with UCD. All patients with MCD were HHV8‐negative. The most common histopathologic variant was hyaline vascular (75%, 18/24). Plasma cell variant occurred in 33% (2/6 [95% confidence intervals (CI), 4–78%]) of patients with HHV8‐negative MCD and 17% (3/18 [95% CI, 4–41%]) of patients with UCD. Systemic symptoms were present in 4 of 6 of patients with HHV8‐negative MCD and 8 of 18 of patients with UCD. Anemia and laboratory inflammation occurred in both UCD and MCD patients, with nonsignificantly higher rates of anemia and elevated C‐reactive protein in MCD patients. All but two UCD patients underwent gross total resection as definitive therapy. Among HHV8‐negative MCD patients, a combination of resection, chemotherapy, and immunotherapy was used. No UCD patients and three of six HHV8‐negative MCD patients experienced disease progression/relapse prior to lasting remission. There were no deaths. Conclusion Pediatric patients with CD most commonly have unicentric, hyaline vascular variant disease. Pediatric patients with both UCD and MCD commonly have systemic inflammation and, despite risk of progression/relapse in MCD patients, ultimately have excellent survival.

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“…Human Herpesvirus (HHV)‐8‐negative, idiopathic multicentric Castleman disease (iMCD) is a polyclonal lymphoproliferative disorder with an unknown etiology. Approximately 1000 individuals of all ages are diagnosed with iMCD each year in the USA, and 35%‐45% of patients die within 5 years of diagnosis . Patients experience heterogeneous clinical and laboratory abnormalities including constitutional symptoms, anemia, anasarca, renal failure, hypoalbuminemia, thrombocytopenia or thrombocytosis, and multicentric lymphadenopathy.…”

Section: Introductionmentioning

confidence: 99%

Plasma proteomics identifies a ‘chemokine storm’ in idiopathic multicentric Castleman disease

et al. 2018

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Human Herpesvirus-8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) is a poorly understood disease involving polyclonal lymphoproliferation with dysmorphic germinal centers, constitutional symptoms, and multi-organ failure. Patients can experience thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly, and normal immunoglobulin levels, - iMCD-TAFRO. Others experience thrombocytosis, milder effusions, and hypergammaglobulinemia, -iMCD-Not Otherwise Specified (iMCD-NOS). Though the etiology is unknown in both subtypes, iMCD symptoms and disease progression are believed to be driven by a cytokine storm, often including interleukin-6 (IL-6). However, approximately two-thirds of patients do not respond to anti-IL-6 therapy; alternative drivers and signaling pathways are not known for anti-IL-6 nonresponders. To identify potential mediators of iMCD pathogenesis, we quantified 1129 proteins in 13 plasma samples from six iMCD patients during flare and remission. The acute phase reactant NPS-PLA2 was the only significantly increased protein (P = .017); chemokines and complement were significantly enriched pathways. Chemokines represented the greatest proportion of upregulated cytokines, suggesting that iMCD involves a chemokine storm. The chemokine CXCL13, which is essential in homing B cells to germinal centers, was the most upregulated cytokine across all patients (log2 fold-change = 3.22). Expression of CXCL13 was also significantly increased in iMCD lymph node germinal centers compared to controls in a stromal meshwork pattern. We observed distinct proteomic profiles between the two iMCD-TAFRO patients, who both failed anti-IL-6-therapy, and the four iMCD-NOS patients, in whom all three treated with anti-IL-6-therapy responded, suggesting that differing mechanisms may exist. This study reveals proteomic differences between flare and remission and the potential to molecularly define iMCD subgroups.

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Clinical and morphological features of different types of Castleman’s disease

Cited by 21 publications

References 22 publications

The full spectrum of Castleman disease: 273 patients studied over 20 years

The full spectrum of Castleman disease: 273 patients studied over 20 years

Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two‐site retrospective cohort study

Plasma proteomics identifies a ‘chemokine storm’ in idiopathic multicentric Castleman disease

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