Clinical commissioning policy: siltuximab for idiopathic multicentric Castlemandisease (adults) Skip to main contentCookies on the NHS England websiteWe’ve put some small files called cookies on your device to make our site work.We’d also like to use analytics cookies. These send information about how our site is used to a service called Google Analytics. We use this information to improve our site.Let us know if this is OK. We’ll use a cookie to save your choice. You can read more about our cookies before you choose. Change my preferences I'm OK with analytics cookiesHome News Publications Statistics Blogs Events Contact usSearch SearchAbout us Our work Commissioning Get involved CoronavirusClinical commissioning policy: siltuximab for idiopathic multicentric Castlemandisease (adults
From Castlemandisease histopathological features to idiopathic multicentric Castlemandisease: a multiparametric approach to exclude potential iMCD histopathological mimickers. International consensus diagnostic criteria for idiopathic multicentric Castlemandisease (iMCD) includes lymph node Castlemandisease (CD) histopathological features as major criteria. Our aim was to apply those
Idiopathic multicentric Castlemandisease in children: a single-center retrospective analysis. To investigate the clinical features, pathological phenotype, treatment and prognosis of idiopathic multicenter Castlemandisease (iMCD)in children. From January 2017 to September 2023, basic information, laboratory tests, treatment and prognosis of children diagnosed with iMCD who attended Beijing
Retinal Vasculitis Associated With CastlemanDisease. This case report describes a diagnosis of retinal vasculitis associated with Castlemandisease in a man who presented with 6 months of progressive floaters bilaterally and no change in visual acuity.
Acquired Hemophilia: A Rare Complication of Pediatric Idiopathic Multicentric CastlemanDisease. Acquired hemophilia is caused by acquired autoantibodies to 1 of the factors of the coagulation cascade, usually factor VIII or IX, and is an exceedingly rare phenomenon in children. The finding of an acquired factor VIII inhibitor in a pediatric patient with idiopathic multicentric Castlemandisease has never been reported. Patients with acquired hemophilia can have life-threatening bleeds that are refractory to blood product support, requiring bypassing agents to manage bleeding symptoms. We present the novel finding of acquired hemophilia resulting from an autoantibody to factor VIII in a pediatric patient with idiopathic multicentric Castlemandisease and discuss the optimal management
Single-cell landscape of idiopathic Multicentric CastlemanDisease in identical twins. Idiopathic Multicentric CastlemanDisease (iMCD) is a rare cytokine-driven disorder characterized by systemic inflammation, generalized lymphadenopathy and organ dysfunction. Here, we present an unusual occurrence of iMCD in identical twins and examined the immune milieu within affected lymphoid organs
Diagnostic challenges of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of idiopathic multicentric Castlemandisease (iMCD): Factors to differentiate from IgG4-related disease. Idiopathic multicentric Castlemandisease (iMCD) is currently considered to be classified into three clinical subtypes, including idiopathic plasmacytic lymphadenopathy (IPL), thrombocytopaenia, anasarca, fever
Urologic Presentation of Unicentric Pediatric CastlemanDisease in the setting of Acute Renal Colic. 11-year-old otherwise healthy female presented with renal colic and during computed tomography (CT) imaging evaluation, she was found to have a right distal ureteral stone with associated hydroureteronephrosis, medially deviated ureter, and 4 cm solid retroperitoneal mass. The mass was palpable with histologic features of Castlemandisease (CD).
Continuous therapy in HHV-8 negative Multicentric CastlemanDisease and prolonged progression-free survival. The optimal treatment endpoints and duration of continuous therapy for multicentric Castlemandisease (MCD) remain controversial. We retrospectively analyzed data from 123 patients with Human Herpesvirus (HHV)-8 negative MCD. We demonstrated that continuous therapy significantly enhanced
The Transformation of Neck Unicentric Castleman'sDisease to Follicular Dendritic Cell Sarcoma. A patient with a large neck mass underwent appropriate imaging, routine blood tests, and serological evaluations. The medical history revealed the patient had undergone a lymphadenectomy in the same region 8 years ago, and the pathological diagnosis was the hyaline-vascular subtype of unicentric Castleman'sdisease (UCD). The incisional biopsy and subsequent histopathological and immunohistochemical examination revealed the diagnosis of follicular dendritic cell sarcoma, consistent with the malignant transformation of UCD. UCD is uncommon and the malignant transformation of UCD is extremely rare in the head and neck region. Regional lymph node resection of one or more adjacent regions
Plasma cell type Castleman'sdisease of lacrimal gland: a case report and literature review. Orbital Castleman'sdisease is a rare benign lymphoproliferative disorder of extranodal origin. A 72-year-old man presented with bilateral upper eyelid swelling. Radiology revealed enlarged lacrimal glands in both eyes. Castleman'sdisease (plasma cell type) was confirmed by histo-immunochemical examinations after excision of the left orbital mass. No extraorbital involvement was found in systemic evaluation. Postoperatively, corticosteroid therapy was given. After 1 year of follow-up, there was no recurrence in the left orbit, and the swelling of the right eyelid was improved. Here, we report a case of orbital Castleman'sdisease involving the lacrimal gland bilaterally and summarize previously
Hemophagocytic lymphohistiocytosis in HIV-associated HHV8-positive multicentric castlemandisease. The clinical and laboratory characteristics of HHV8-associated Multicentric CastlemanDisease (MCD) in people living with HIV (PLWH) overlap with those of Hemophagocytic Lymphohistiocytosis (HLH) disease and indeed the two diagnoses may co-exist. A risk-stratified treatment approach to MCD based
Systemic lupus erythematosus combined with Castlemandisease and secondary paraneoplastic pemphigus: a case report. The literature describes a case of systemic lupus erythematosus (SLE) complicated with Castleman'sdisease (CD) and secondary paraneoplastic pemphigus (PNP). A 12-year-old female presented with a neck mass, rash, arthralgia, and skin and mouth ulceration for 5 years were admitted
Whole-exome sequencing reveals the genomic profile and IL6ST mutations as a prognostic biomarker of paraneoplastic pemphigus associated unicentric Castlemandisease. Unicentric Castlemandisease (UCD) is a rare lymphoproliferative disorder. Paraneoplastic pemphigus (PNP) is a major complication associated with poor UCD prognosis. However, the genomic profiles and prognostic biomarkers of PNP
Symptom burden in patients with idiopathic multicentric Castlemandisease and its impact on daily life: an international patient and caregiver survey. Idiopathic Multicentric CastlemanDisease (iMCD) is a rare inflammatory lymphoproliferative disorder with heterogenous clinical presentations. The symptomatology in iMCD patients remains poorly understood. The aim of this study was to identify
Idiopathic multicentric castleman'sdisease mimicking immunoglobulin G4-related disease responding well to Bortezomib: a case report. Castleman'sdisease (CD) is a rare disease that has clinical and pathological similarities to lymphoma and is characterized by a high frequency of associated immunological dysfunction. ImmunoglobulinG4-related disease (IgG4-RD) is a collection of systemic did not recover. Ultimately, idiopathic multicentric Castlemandisease (iMCD) was diagnosed in relation to the patient's clinical presentation and laboratory tests, and after combination chemotherapy (VCD: Bortezomib, Cyclophosphamide and Dexamethasone), durable remission was achieved without serious adverse effects. During the follow-up period of one year and ten months, the patient remained stable
Evolution of Pulmonary Involvement in Idiopathic Multicentric CastlemanDisease-Not Otherwise Specified: From Nodules to Cysts or Consolidation. Previous studies about multicentric Castlemandisease-associated pulmonary manifestations have been limited by small cohorts and not following the CastlemanDisease Collaborative Network classification criteria of multicentric Castlemandisease . The pulmonary manifestations in idiopathic multicentric Castlemandisease-not otherwise specified (iMCD-NOS), a distinct clinical phenotype in the classification criteria, have not been reported. Which pulmonary abnormalities in iMCD-NOS are advanced manifestations and which are reversible after effective treatment? Patients diagnosed with iMCD-NOS with pulmonary involvement were enrolled. The baseline CT
[Clinicopathological and therapeutic analysis of Castleman'sdisease in children]. To investigate the clinical features, pathological phenotype, treatment and prognosis of Castleman'sdisease in children. Clinical data of 15 children diagnosed with Castleman'sdisease in Henan Provincial People's Hospital and the First Affiliated Hospital of Zhengzhou University from May 2010 to October 2019 were analyzed retrospectively. The clinical characteristics, laboratory examination and histopathological data were analyzed. Among the 15 Castlemansdisease patients, 12 were males and 3 females. The age of first visit was 12 (10, 15) years. The time from mass discovery to pathologic diagnosis was 9.0 (2.0, 13.0) months. The majority of patients were unicentric (13 cases), and the histopathological
Siltuximab is associated with improved progression-free survival in idiopathic multicentric Castlemandisease. Idiopathic multicentric Castlemandisease (iMCD) is a rare heterogeneous disorder involving multicentric lymphadenopathy, systemic inflammation, and cytokine-driven organ dysfunction. Despite the approval of siltuximab, a monoclonal antibody against interleukin-6, for the treatment