Materiały źródłowe

• #1 Castleman Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK576394/

  Castleman disease is a lymphoproliferative disorder with a nondefinite etiology and can be classified into subtypes based on clinical and histological manifestations. […] The lack of proper knowledge and even a formal definition of Castleman disease made it challenging to assess its epidemiology. A study was conducted in 2008 by Simpson which showed that: […] The incidence of UCD is 16 per million patient-years and affects all age groups. […] The incidence of HHV-8 associated CD varies widely, but it is more common in HIV-positive men. […] The incidence of iMCD is 5 per million patient-years. […] Another study conducted in the US from 2000 to 2009 concluded that out of 59 patients with MCD, 61% were males with the mean age of 53 years, and 68% were white. […] The hyaline-vascular variant is more common than the plasma-cell type, comprising 91% of the total cases. […] Its predominance in any specific sex or race is not noted. Castleman disease usually develops in most patients before reaching 30, although the age range is broad.

• #2 Castleman Disease | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/135910

  The lack of proper knowledge and even a formal definition of Castleman disease made it challenging to assess its epidemiology. A study was conducted in 2008 by Simpson which showed that: […] The incidence of UCD is 16 per million patient-years and affects all age groups. […] The incidence of HHV-8 associated CD varies widely, but it is more common in HIV-positive men. […] The incidence of iMCD is 5 per million patient-years. […] Another study conducted in the US from 2000 to 2009 concluded that out of 59 patients with MCD, 61% were males with the mean age of 53 years, and 68% were white. […] The hyaline-vascular variant is more common than the plasma-cell type, comprising 91% of the total cases. […] Its predominance in any specific sex or race is not noted. Castleman disease usually develops in most patients before reaching 30, although the age range is broad.

• #3 Castleman Disease: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/2219018-overview

  Castleman disease is rare. An estimated 4300-5200 new cases are diagnosed each year in the United States; approximately 1000 of those are HHV-8associated multicentric Castleman disease (HHV-8associated MCD) and 1000 are HHV-8negative MCD. […] UCD, HHV-8associated MCD, and HHV-8negative MCD can affect individuals of all ages (including young children), genders, and ethnicities. […] HHV-8associated MCD is more common in men and individuals with HIV infection. The incidence of HHV-8associated MCD has increased with better antiretroviral therapy (ART) for the management of HIV infection. On multivariate analysis, risk factors for the development of HHV-8associated MCD included the following: Nadir CD4 count higher than 200/L, Older age, No previous ART exposure, Nonwhite ethnicity. […] HHV-8negative/idiopathic MCD is similarly common in men and women. There are no known risk factors.

• #4 About Castleman Disease – Castleman Disease Collaborative Network

  https://cdcn.org/castleman-disease/

  Castleman disease (CD) is a rare disease but it does not discriminate; it can occur in people of all ages and genders. In the USA, there are an estimated 4,300 to 5,200 new CD cases diagnosed per year. […] It is therefore easier to understand why most physicians may not have much, or any, experience in treating CD.

• #5 Overview – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/overview/

  Approximately 4,300-5,200 cases of CD are diagnosed in the US each year; CD can occur in people of any age, gender, or ethnicity. […] Whether Castleman disease should be considered an autoimmune disease, cancer, or infectious disease is currently unknown.

• #6 Castleman Disease | Condition | UAMS Health

  https://uamshealth.com/condition/castleman-disease/

  Approximately 4,300-5,200 new cases of Castleman Disease are diagnosed each year in the U.S. […] Unicentric Castleman disease is more common than multicentric Castleman disease, and can occur in people of any age, gender, or ethnicity affecting children as well as adults. […] Multicentric CD (MCD) can be caused by a cancerous cell population (referred to as POEMS-associated MCD), an infection with human herpesvirus-8 (HHV-8-associated MCD), or an unknown cause (idiopathic MCD) where there are signs of autoimmunity but no evidence of an infection or a cancer. […] There are no known risk factors for UCD, POEMS-associated MCD, or iMCD; there is no evidence of any food, lifestyle, or environmental exposure associated with these diseases. […] The Myeloma Center participates with the Castleman Disease Collaborative Network to accelerate research and explore the causes of both unicentric and multicentric Castleman disease.

• #7 Epidemiology of Castleman Disease – PubMed

  https://pubmed.ncbi.nlm.nih.gov/29157611/

  Castleman disease is a rare entity, including unicentric Castleman disease (UCD), human herpesvirus-8 plus Castleman disease (HHV-8+MCD), and idiopathic multicentric Castleman disease (iMCD). […] UCD is the most common at 16 per million person years and occurs at every age. […] HHV-8+MCD incidence varies widely, mostly affecting human immunodeficiency virus-positive men. […] iMCD is likely a more heterogeneous disease with an estimated incidence of 5 per million person years. […] Improved definitions should improve understanding of the epidemiology of Castleman disease and its subtypes.

• #8 The management of Castleman disease

  https://b-s-h.org.uk/guidelines/guidelines/the-management-of-castleman-disease

  The incidence of all forms of CD is estimated at 2125 per million person-years, based on insurance registries in the USA. […] Application of this rate to the UK adult population provides an estimated incidence of CD in the UK of 1100-1300 patients per year. […] There is an approximately even distribution of CD between men and women. […] The introduction of the International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code D47.Z2 in 2016 should facilitate the understanding of the epidemiology of the disease.

• #9 Clinicopathological comparison and therapeutic approach to Castleman disease—a case-based review – Wojtyś – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/33164/html

  Castleman disease (CD) is a rare, B-cell lymphoproliferative disorder affecting lymph nodes and extranodal anatomical locations. […] With an occurrence rate of 21 to 25 cases per million person-years, it is classified as an orphan disease. […] In clinical practice, two variants exist: unicentric CD (UCD; ~75%) and multicentric CD (MCD; ~25%), the latter with various clinical manifestations. […] CD can occur in practically any part of the body, but arises predominantly in the thorax (~70%), followed by the abdomen and pelvis (~15%) and neck (~15%). […] Despite numerous case presentations since Castleman et al. first report in 1954, CD remains a diagnostic and therapeutic challenge. […] The accuracy of histopathological examination is essential and re-evaluation has to be performed in case of relapse or unexpected course of CD.

• #10 Castlemans disease

  https://atlasgeneticsoncology.org/haematological/2123/castlemans-disease

  The epidemiology of CD is difficult to characterize accurately due to its rarity and clinical heterogeneity. The incidence of CD is estimated at 21-25 cases per million person-years, with 23% of those cases potentially representing MCD (Munshi N et al.,2015). The median age at presentation for UCD is 30-34 years. For HIV-negative MCD is 49-66 years and HIV-positive MCD between 36-40 years. The sex distribution is approximately equal, though some series have reported a male predominance, generally in the HIV-positive population. UCD also has a slight female predominance (1.4:1) (Talat N, et al., 2011; Dispenzieri A et al., 2012).

• #11 Unicentric Castleman disease – Wikipedia

  https://en.wikipedia.org/wiki/Unicentric_Castleman_disease

  There are approximately 5000-6000 new cases of UCD diagnosed per year in the United States, making it the most common form of Castleman disease. […] UCD can occur at any age, but the median age at presentation is approximately 35 years old. There is a slightly increased incidence of UCD in women. […] There have been no published epidemiologic studies of Castleman disease outside of the United States; however, there is no evidence of increased or decreased incidence of Castleman disease in specific regions or ethnicities.

• #12 Castleman disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/castleman-disease?embed_domain=hackmd.io%252525252F%2525252540yIPUAFeCSL2JsU8smR5nJQ%252525252Fbnjhjgjghjghjgh&lang=us

  Unicentric Castleman disease typically occurs in children and young adults (3rd and 4th decades), with a slight female predominance (1.4:1) 15. […] Multicentric Castleman disease, on the other hand, occurs in an older population (5th and 6th decades), with a slight male predominance 15. HIV is a relevant risk factor for this condition, and it has been demonstrated that all the HIV patients with multicentric Castleman disease are coinfected with the human herpesvirus 8 (HHV-8).

• #13 Pathology Outlines – Castleman disease

  https://www.pathologyoutlines.com/topic/lymphnodescastleman.html

  UCD can affect all age groups and shows no gender difference with the median age of onset in the fourth decade (Hematol Oncol Clin North Am 2018;32:1) […] MCD affects patients of all ages with a median age of onset in the fifth to seventh decades […] HHV8 MCD affects mainly HIV positive individuals; however, individuals who are immunocompromised from other causes can also be affected (Leuk Lymphoma 2015;56:1252) […] HHV8 MCD in individuals who are HIV negative accounts for 2 – 50% of cases and this variation depends on the prevalence of HHV8 in the region (Blood 2020;135:1353) […] With the introduction of antiretroviral therapy (ART), the incidence of HHV8 MCD has increased […] UCD is more common in childhood (75%) than iMCD and only rare cases of HHV8 MCD are reported in children (Pediatrics 2012;129:e199, Pediatr Blood Cancer 2019;66:e27613)

• #14 HHV8-associated multicentric Castleman disease: a case report on a rar | RRTM

  https://www.dovepress.com/hhv8-associated-multicentric-castleman-disease-a-case-report-on-a-rare-peer-reviewed-fulltext-article-RRTM

  Epidemiological studies of this HHV8-MCD are lacking, especially in sub-Saharan Africa, where the prevalence of HHV8 is the highest, and access to diagnostic pathology is often limited. […] Incidence of HHV8-MCD appears to be increasing proportionally to antiretroviral therapy (ART) coverage, particularly highly active ART (HAART). […] A cohort study from the United Kingdom showed a dramatic increase in incidence of HHV8-MCD from 0.58 cases per 10,000 patient-years in the pre-HAART era to 8.3 in the later HAART era. […] This case highlights the challenges faced by patients and carers concerning complications associated with advanced HIV in Malawi. With increasing ART coverage, clinicians in Sub-Saharan Africa can expect to see increasing numbers of patients with HHV8-MCD. […] We suggest that clinicians working in settings like ours need not despair at a lack of available blood tests, as our case demonstrates that a diagnosis can be made without conforming to the established diagnostic criteria.

• #15 Idiopathic multicentric Castleman disease – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_multicentric_Castleman_disease

  There are approximately 1500-1800 new cases of iMCD diagnosed per year in the United States. iMCD can occur at any age, but the median age at presentation is approximately 50 years old. There is a slightly increased incidence of iMCD in women.[13] […] There have been no published epidemiologic studies of Castleman disease outside of the United States; however, there has been no published data demonstrating increased or decreased incidence of Castleman disease in specific regions or ethnicities.[citation needed]

• #16 How We Manage Idiopathic Multicentric Castleman Disease – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/september-2022/how-we-manage-idiopathic-multicentric-castleman-disease/

  Affecting roughly 1600 patients annually in the United States, MCD is difficult to diagnose and life-threatening, with a historical 5-year overall survival rate of 55% to 77%. […] Approximately half of all cases of MCD are caused by uncontrolled infection with human herpesvirus 8 (HHV8 -associated MCD) or a monoclonal plasma cell population associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes) syndrome (POEMS syndrome-associated MCD); the remaining cases are idiopathic (iMCD). […] Importantly, we strongly recommend consultation with the Castleman Disease Collaborative Network (CDCN), which can be facilitated through www.CDCN.org or by emailing [email protected]. […] The treatment of HHV8-associated MCD and POEMS-associated MCD is reviewed elsewhere.

• #17 Think iMCD – About CD / iMCD

  https://thinkimcd.us/about/

  iMCD is a rare disorder that could potentially be life-threatening. In a retrospective analysis in the the US, the annual incidence of iMCD was 3.1-3.4 cases per million. In the same study, the prevalence of iMCD was estimated at 6.9-9.7 cases per million. […] In a retrospective analysis of US claims data, a higher proportion of patients with iMCD presented with organ failure and/or thrombotic events, compared with a non-iMCD matched cohort. […] The same analysis found that patients with iMCD had higher prevalence of several malignancies, compared with a non-iMCD matched cohort.

• #18 Castleman Disease—Still More Questions than Answers: A Case Report and Review of the Literature

  https://www.mdpi.com/2077-0383/14/8/2799

  Idiopathic multicentric Castleman disease can affect individuals of all ages, but it typically presents in adults with a median age of diagnosis around 50 years. […] In contrast, the epidemiology of CD in adolescents remains poorly understood due to its rarity and the limited number of reported cases in this age group. […] Thus, our case makes a unique contribution to the existing literature, emphasizing the need for dedicated research on CD within adolescent populations.

• #19 Novel Algorithm Highlights the Burden of Idiopathic Multicentric Castleman Disease

  https://consultqd.clevelandclinic.org/novel-algorithm-highlights-the-burden-of-idiopathic-multicentric-castleman-disease

  A new study examining the epidemiology of idiopathic multicentric Castleman disease (iMCD) estimates its prevalence in the United States to be approximately 2,200 cases. The estimate was derived from the application of claims-based algorithm to identify newly diagnosed cases of iMCD. […] These findings, published in Blood Advances, are more precise than those generated from prior epidemiologic studies, which identified iMCD based on rigorous criteria unavailable to previous investigators, explains Sudipto Mukherjee, MD, MPH, co-leader of the Rare Cancers and Blood Diseases program at Cleveland Clinic. […] This data led to the realization that the disease burden of iMCD in the U.S. is higher than what we previously thought, which means that there are a lot of patients who could benefit from treatment if they are diagnosed properly in a timely manner, he explains.

• #20 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Castleman-Disease.aspx

  It should be emphasized that data from low- and middle-income countries (LMICs), as well as Sub-Saharan Africa, is lacking. There are only a few case studies that have been published. […] There hasn’t been a systematic assessment of the role of CD screening in any manner. They are rare diseases in general, and a comprehensive, population-based screening approach is unlikely to result in meaningful or cost-effective improvements in diagnosis or clinical outcomes for those affected. […] The incidence of POEMS is unknown; however, it is estimated to be around 5 per million.

• #21

  https://haematologica.org/article/view/8912

  Despite high burden of Kaposi sarcoma (KS) and HIV, multicentric Castleman disease (MCD) is rarely reported in sub-Saharan Africa (SSA). […] Malawi is a low-income country in SSA with 9% HIV prevalence and 66% ART coverage. […] To our knowledge, this is the first prospective cohort description of HIV MCD from SSA, where KS-associated herpesvirus (KSHV) and HIV are endemic. […] The infrequent reports of MCD in SSA are probably due to under-diagnosis; IHC is often not performed thus limiting the pathology spectrum. […] Once diagnosed, MCD treatment options in SSA are limited. […] Our experience demonstrates the value of close collaboration between SSA clinicians and pathologists. […] MCD awareness in Lilongwe has increased, which is important given that MCD may increase with ART scale-up given the data in high-income countries and the profile of MCD patients in Malawi who typically had well-controlled HIV on long-term ART.

• #22

  https://haematologica.org/article/view/8912

  In conclusion, MCD occurs relatively frequently in Malawi, and unlike HICs, is uniformly associated with HIV and KSHV. MCD presents significant diagnostic and treatment challenges in SSA. Improved awareness, laboratory capacity, and treatments are needed to address this emerging and under-recognized problem, but rapid progress is achievable even in low-income country settings through sustained multidisciplinary collaboration.

• #23 Multicentric Castleman’s Disease | IntechOpen

  https://www.intechopen.com/chapters/49408

  Castlemans disease (CD), from being rare, is now more commonly encountered. […] In the last three decades since the discovery of HIV, the incidence of MCD has progressively increased over time. […] The median age at presentation of MCD is 40 years, with a male predominance of 90%, based on a systematic review of published cases in the literature up to 2007. […] A younger age at presentation is noted in HIV-seropositive individuals with MCD compared to HIV-seronegative MCD. […] At Chris Hani Baragwanath Academic Hospital (CHBAH), Soweto, Johannesburg, a total of 38 patients were seen with CD over a 25-year period. […] The increase in the number of patients with CD in the last 5 years is primarily as the result of the ongoing HIV pandemic in South Africa and the contribution from HIV.

• #24 Multicentric Castleman’s Disease | IntechOpen

  https://www.intechopen.com/chapters/49408

  In South Africa, CD is rare. However, there has been a noticeable increase in MCD in the last five years, with more than doubling of the number of patients in the last five years, compared to the previous twenty years. […] The majority of patients in this series with MCD are HIV seropositive (86%).

• #25 Comprehensive analysis of 65 patients with Castleman disease in a single center in China | Scientific Reports

  https://www.nature.com/articles/s41598-022-12797-y

  This study aimed to investigate the epidemiologic, clinical, pathological characteristics, and treatment of patients with Castleman disease (CD) in a single center in China. […] Detailed epidemiologic, clinicopathological, and treatment data were analyzed and discussed. […] The mean age of onset of total was 38.5 years with PC higher than HV (45.5 vs. 35.1 years, P=0.0413). […] The median diagnosis delay of MCD was longer than that of UCD (3.00 vs. 1.25 months, P=0.0436). […] These results provided the featured and detailed profile of Castleman disease in Henan province in China with a considerable number of cases, which presented distinct evidence with other studies. […] Despite all this, the very nature of CD still remains unclear enough, especially the little published information about incidence from China.

• #26 Epidemiology and Treatment Patterns of Idiopathic Multicentric Castleman Disease in the Era of IL-6-Directed Therapy Outcomes | Cleveland Clinic

  https://my.clevelandclinic.org/departments/cancer/outcomes/1151-epidemiology-and-treatment-patterns-of-idiopathic-multicentric-castleman-disease-in-the-era-of-il-6-directed-therapy

  The epidemiology of human herpesvirus-8-negative/idiopathic multicentric Castleman disease (iMCD) remains incompletely understood. […] Limited data exist regarding the epidemiology and treatment patterns of iMCD, and CD more generally, in the United States, particularly among patients receiving care in nonacademic settings. […] Our study using a novel administrative claims-based methodology provides the most up-to-date population estimates of the incidence and prevalence of CD and iMCD in the United States and sheds light on the treatment landscape of this rare disease.

• #27 SEER Inquiry System – Question 20021014 Details

  https://seer.cancer.gov/seer-inquiry/inquiry-detail/20021014/

  Castleman’s Disease is a rare disorder characterized by non-cancerous growths that may develop in the lymph node tissue throughout the body. […] For cases diagnosed prior to 1/1/2010: Castleman’s Disease is not reportable to SEER. […] For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question.

• #28 SEER Hematopoietic and Lymphoid Neoplasm Database

  https://seer.cancer.gov/seertools/hemelymph/532b3e3fe4b0626b1926ec92/

  This neoplasm is not reportable. […] Multicentric Castleman disease affects many groups of lymph nodes and lymphoid tissue all through the body. […] People with Castleman disease have an increased risk of lymphoma.

• #29

  https://haematologica.org/article/view/haematol.2023.283603

  The ACCELERATE natural history registry is ideally suited to better understand the burden of disease as the most extensive source of longitudinal clinical data (median 3.4 years of follow-up after diagnosis) for these patients. […] We found that patients with iMCD require extensive use of the healthcare system and experience long-term effects in terms of iMCD-related morbidities and comorbidities, time in disease flare, and QOL. […] Patients with TAFRO required significantly longer stays in hospital and disproportionately required interventions such as dialysis, mechanical ventilation, and transfusions, illustrating the severe, life-threatening nature of this disease. […] Our observation that patients required extensive hospital care in the year surrounding diagnosis is consistent with the findings of a recent claims-based study.

• #30 Comprehensive analysis of 65 patients with Castleman disease in a single center in China | Scientific Reports

  https://www.nature.com/articles/s41598-022-12797-y

  Therefore, we suggest that a multi-disciplinary team (MDT) for rare diseases, a working group made up of experts from departments of surgery, oncology, radiology, pathology, radiotherapy, etc., should be applied to propose the best treatment plan suitable for patients through a regular consultation.

• #31 Comprehensive analysis of 65 patients with Castleman disease in a single center in China | Scientific Reports

  https://www.nature.com/articles/s41598-022-12797-y

  In this study, we retrospectively analyzed 65 patients in Henan Provincial Peoples Hospital in central China for the better understanding of this disease. […] A single-center data specificity with a fair number of patients was demonstrated. […] In general, more evidence and insights were given for further investigations of this disease. […] The diagnosis of CD includes clinical examinations (specific clinical signs and complications), imaging study (systemic imaging with PET/computed tomography), laboratory investigations, pathological evaluation with immunostaining as well as molecular virology. […] The diagnosis delay of MCD patients (3.00 months) in our study was longer than UCD patients (1.25 months). […] The treatment of CD in a single department is generally summarized experience, plus the rarity characteristic and clinical heterogeneity it owns, so there are diverse perceptions of this disease among doctors from different departments.

• #32 An unusual presentation of Castleman’s Disease:a case report | BMC Infectious Diseases | Full Text

  https://bmcinfectdis.biomedcentral.com/articles/10.1186/1471-2334-3-20

  Castleman’s disease (CD), a rare condition of uncertain etiology, involves a massive proliferation of lymphoid tissues and typically presents as mediastinal masses. […] Because patients with similar clinical histories are at high risk for the development of non-Hodgkin’s lymphoma and Kaposi sarcoma, regular medical surveillance is recommended. […] The first case of MCD, which presented as generalized lymphadenopathy with systemic manifestations of fever, night sweats, weight loss and fatigue, was reported in 1978. […] HIV seropositive individuals appear to be at an increased risk for MCD, and it can often arise concurrently with Kaposi sarcoma (KS). […] It should be noted that because of this medical history, this patient is considered to be at high risk for the development of non-Hodgkin’s lymphoma and Kaposi sarcoma and regular medical follow up is indicated.

• #33

  https://haematologica.org/article/view/haematol.2023.283603

  Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. […] Due to its rarity and heterogeneity, the natural history and long-term burden of iMCD are poorly understood. […] A recent epidemiological study based on USA insurance claims found that approximately 1,000 patients were diagnosed with iMCD each year. […] Making a diagnosis of iMCD is challenging because it is based on non-specific clinical features and characteristic lymph node histopathology. […] Data from the USA before 2012 suggested 5- and 10-year mortality rates of 35% and 60% for iMCD patients whereas more recent data from electronic medical records suggest 25% mortality at 5 years.

• #34

  https://www.jci.org/articles/view/131332

  Idiopathic multicentric Castleman disease (iMCD) is a rare, systemic hematologic illness that involves widespread cytokine-induced inflammation, polyclonal lymphoproliferation, cytopenias, and multi-organ dysfunction. It has an incidence of 1000-1500 cases in the United States and a 5-year overall survival of only 55%-77%. […] The most severe cases of iMCD are marked by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly, collectively categorized as the TAFRO subtype. […] The etiologic trigger for iMCD remains unknown. However, excessive signaling from the proinflammatory cytokine IL-6 appears to play a role in the pathogenesis and symptomatology of the disease. […] While IL-6 blockade represented a significant breakthrough in iMCD treatment, a substantial portion of patients with iMCD remain refractory to therapy with siltuximab or tocilizumab. This suggests that additional pathways may underlie iMCD pathogenesis and be important targets for future iMCD therapies. […] Fajgenbaum et al. successfully employ a precision medicine approach to identify and target the mTOR pathway in 3 IL-6 blockaderefractory patients with iMCD. Their methodology was innovative and presents a potential roadmap for similar investigations in other rare diseases.

• #35 How We Manage Idiopathic Multicentric Castleman Disease – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/september-2022/how-we-manage-idiopathic-multicentric-castleman-disease/

  The publication of evidence-based consensus diagnostic criteria for iMCD has successfully standardized previously disparate paths to establishing the diagnosis. […] The CDCN consensus guidelines recommend first-line anti–IL-6 therapy with siltuximab for all patients with iMCD. […] Patients with severe iMCD should receive high-dose corticosteroids concurrently with siltuximab. […] Patients with mild or moderate disease should start with siltuximab every 3 weeks, and if their disease proves to be refractory, they should either enroll in a clinical trial in the second line or receive rituximab or other immunomodulators. […] All patients should be encouraged to join the CDCN at diagnosis (or earlier if CD is strongly suspected), and an additional immunomodulatory agent should be selected in consultation with a CD-experienced physician for patients with siltuximab-refractory disease.

• #36 Siltuximab in Idiopathic Multicentric Castleman Disease: Real-World Experience | Jitaru | Journal of Hematology

  https://thejh.org/index.php/jh/article/view/1343/874

  Castleman disease (CD) is an extremely rare, non-malignant, lymphoproliferative disorder. Its estimated worldwide incidence is 5 – 16 per million people a year, depending on region, although accurate incidence rates in low- and middle-income countries are lacking. […] Due to the rarity of iMCD, it is important to report real-world data for patients treated with siltuximab in regions where modern therapies are available. Here, we report disease response and survival outcomes of patients with iMCD following therapy with siltuximab in a retrospective, real-world study conducted in Greece and Romania. […] MCD is very rare and real-world data for patients with iMCD treated with siltuximab are currently scarce, with only two publications related to European patients (from Italy and Poland) published so far.

• #37 Siltuximab in Idiopathic Multicentric Castleman Disease: Real-World Experience | Jitaru | Journal of Hematology

  https://thejh.org/index.php/jh/article/view/1343/874

  Despite limitations such as the relatively small sample size, the retrospective nature of the study, and the potential inclusion of bias and confounding factors, this study provides valuable insights into the effectiveness and safety/tolerability of siltuximab when used in clinical practice. Further data are required to optimize therapy and improve therapeutic outcomes in patients with iMCD.

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