Materiały źródłowe

• #1 Castleman Disease: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17920-castleman-disease

  Castleman disease is a group of disorders that involve a hyperactive immune system. Typically, healthy immune systems activate to fight germs invading your body. Then, they settle down once the threat is gone. But if you have Castleman disease, your immune system remains activated. This leads to long-lasting inflammation that can damage organs. […] Treatment for Castleman disease varies according to the type. […] Surgery to remove the affected lymph nodes is the primary treatment for UCD. You may need radiation therapy or immunotherapy before surgery. These therapies shrink the growths on your lymph nodes, making them easier to remove. […] MCD is more difficult to treat than UCD. As its widespread, providers dont typically use surgery or radiation to treat it. Instead, treatment depends on whether you have HHV-8 and how severe the disease is.

• #2 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. […] This activity explains the evaluation and treatment of Castleman disease and highlights the role of the interprofessional team in evaluating and treating patients with this condition. […] Castleman disease is a rare disease, with minimum availability of therapeutic options. As a unicentric Castleman disease lesion is localized, complete surgical resection of the tumor is the best treatment available. […] Many treatment options are available for treating multicentric CD, including surgery, cytotoxic chemotherapy with or without corticosteroids, and autologous stem cell transplantation (ASCT) with varying outcomes. […] Better results have been observed by targeting CD20 and IL-6 pathways and HHV-8 replication.

• #3 Treatment – CDCN

  https://cdcn.org/treatment/

  The treatment options for Castleman disease are specific to the subtype diagnosed by your physician. […] Surgery is considered by experts to be the first-line treatment for almost all cases of UCD. […] If surgical excision is not possible, treatment is recommended for symptomatic patients. […] If symptoms are due to compression, then rituximab may be administered to attempt to shrink the enlarged lymph node. […] Some UCD patients may experience inflammatory symptoms (fever, fatigue) and elevated inflammatory laboratory markers after lymph node excision. […] These patients are sometimes treated with treatments frequently used to treat MCD, such as anti-IL-6 therapy with siltuximab. […] If these treatments are not effective, then radiation is sometimes tried. […] Your medical team will discuss the options and risk for surgery depending on the location of the enlarged lymph nodes.

• #4 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  Treatment varies depending on the subtype of Castleman disease. […] For unicentric Castleman disease (UCD), surgical removal of the enlarged lymph node is considered the first-line treatment option and can be curative. […] If surgical excision is not possible, treatment is recommended for symptomatic patients. […] If symptoms are due to compression, then rituximab is recommended. […] If symptoms are due to an inflammatory syndrome, then anti-interleukin-6 (IL-6) therapy is recommended. […] If these treatments are not effective, radiation therapy may be needed. […] In HHV-8associated MCD, treatment with rituximab is highly effective. […] For patients with concomitant HIV infection and a low CD4 count and/or higher HIV load, antiretroviral therapy (ART) should be included with the rituximab.

• #5 Diagnosis and Management of Castleman Disease in: Journal of the National Comprehensive Cancer Network Volume 17 Issue 11.5 (2019)

  https://jnccn.org/view/journals/jnccn/17/11.5/article-p1417.xml

  Castleman disease is a heterogeneous nonmalignant lymphoproliferative disorder. Major distinctions include unicentric versus multicentric presentation; hyaline vascular, plasmacytic, or mixed pathology; and HHV8-associated (typically HIV-positive) versus idiopathic disease. At the NCCN 2019 Annual Congress: Hematologic Malignancies, Dr. Jeremy S. Abramson stated that rituximab is preferred as initial therapy for HHV8-positive disease, and chemotherapy can be added for patients with fulminant disease (antiretrovirals should always be used as well for those who are HIV-positive). Siltuximab is the preferred frontline therapy for idiopathic disease. […] Castleman disease is a nonmalignant lymphoproliferative disorder that, although uncommon, is a relatively complex disease to diagnose and manage, according to Jeremy S. Abramson, MD, MMSc, Associate Professor of Medicine, Harvard Medical School, and Director of the Lymphoma Center, Massachusetts General Hospital Cancer Center.

• #6 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For HIV-negative patients, there may also be a role for concomitant antiviral therapy with ganciclovir. […] In HHV-8negative (idiopathic) MCD, the IL-6 inhibitor siltuximab (Sylvant) is the preferred therapy; it is the only drug approved by the US Food and Drug Administration (FDA) for this indication. […] If siltuximab is not available, the IL-6 inhibitor tocilizumab may be used in its place. […] AntiIL-6 treatment is continued until progression of disease in order to maintain the response and prevent an early relapse. […] Optimal treatment for siltuximab non-responders is not known. […] Systemic corticosteroids can provide symptomatic relief but do not predictably reduce tumor size. […] Radiation therapy with 30-45 Gy can result in complete and partial remission rates of 40% and 10%, respectively, but can cause radiation-induced fibrosis that makes subsequent surgical intervention more difficult.

• #7 Castleman Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/castleman-disease

  Castleman disease is a rare immune dysregulation disorder where an overgrowth of cells in your child’s lymph nodes causes them to be enlarged and sometimes also causes fevers or other signs of systemic inflammation (when the immune system is constantly defending the body). […] Enlarged lymph nodes due to Castleman disease are most often found in the neck, collarbone, underarm and groin areas. […] If your child has Castleman disease, you may notice symptoms like: Fever, Unintended weight loss, Fatigue, Night sweats, Raised lumps under the skin, especially in the neck, underarm, collarbone or groin area. […] If you notice lumps in your child’s neck, collarbone, underarm or groin area that are persistent, large, painful, or otherwise concerning, your child should see a doctor right away.

• #8 Castleman’s Disease Presents Challenges in Diagnosis, Treatment | Duke Health Referring Physicians

  https://physicians.dukehealth.org/articles/castlemans-disease-presents-challenges-diagnosis-treatment

  Castlemans disease requires care from multiple subspecialists as a result of overlapping disease characteristics. […] Although Castlemans disease can be strongly suggested by biopsy of an enlarged lymph node, like many rheumatological conditions, it cannot be identified with a single lab result. […] Specialists reach the diagnosis of Castlemans disease after a thorough review of clinical presentation, laboratory data, and differential diagnoses. […] This review includes a multidisciplinary discussion with hematologists, infectious disease specialists, and pathologists. […] Castlemans disease presents with two forms: Unicentric and Multicentric. […] Surgery may be required to remove a diseased lymph node, but medication or radiation therapy will be considered. […] Specific treatment depends on the presence of HIV or human herpesvirus 8 infection. Medications and other therapies are usually employed to control excessive cytokine release.

• #9 What Is Multicentric Castleman Disease?

  https://www.healthline.com/health/multicentric-castleman-disease

  Multicentric Castleman disease is an immune system disease that affects the lymph nodes. Its one of two main types of Castleman disease. The other is unicentric Castleman disease. […] Treatment depends on the type and severity of the disease, so getting a timely diagnosis is vital to improving your odds of a healthy outcome. […] Diagnosing multicentric Castleman disease usually starts with a review of symptoms and your medical history. Lab tests and imaging are also necessary to make an accurate diagnosis. […] The only FDA-approved medication to treat multicentric Castleman disease is the anti-IL-6-targeted monoclonal antibody siltuximab (Sylvant). Treatment guidelines suggest this medication as the first-line treatment in people with multicentric Castleman disease who dont have HIV and HHV-8.

• #10 Castleman disease: Experience from a single institution

  https://www.spandidos-publications.com/10.3892/mi.2023.116

  Castleman disease (CD) describes a group of rare heterogeneous lymphoproliferative disorders characterized by enlarged hyperplastic lymph nodes. […] Complete resection of the involved lesion is the gold standard of treatment for UCD. […] The standard of care for UCD is surgical excision and for multicentric CD, systemic therapy is the mainstay of treatment. […] Among the 7 patients with UCD, 6 patients underwent surgery, including 5 patients who underwent complete excision, 1 patient with debulking, and 1 patient (patient no. 11) received rituximab. […] The patients with MCD received systemic treatment with rituximab and/or chemotherapy with rituximab, cyclophosphamide, adriamycin, vincristine and prednisolone (R-CHOP). […] Systemic therapy is the mainstay of treatment for patients with MCD. […] In conclusion, CD is a rare disease occurring in immunodeficient patients. UCD is more common and is associated with improved outcomes. Surgery is the mainstay of management in unicentric disease, whereas MCD requires combination chemotherapy.

• #11 Diagnosis and Management of Castleman Disease in: Journal of the National Comprehensive Cancer Network Volume 17 Issue 11.5 (2019)

  https://jnccn.org/view/journals/jnccn/17/11.5/article-p1417.xml

  The treatment of choice for UCD is surgical resection, which is associated with 90% relapse-free survival. Unresectable disease can be treated with local radiation, or rituximab and/or steroids can be used to reduce the mass with the goal of converting it to be surgically resectable. […] HHV8-positive MCD is generally associated with an IL-6-mediated inflammatory syndrome, and is most commonly seen in the setting of HIV (but may rarely occur in other immunocompromised patients). It is most commonly plasmacytic or mixed histology, with a male predominance. Patients with MCD are often quite ill and typically present with fevers, diffuse lymphadenopathy, hepatosplenomegaly, elevated inflammatory markers, cytopenias, and hypoalbuminemia. […] Prognosis for HHV8-positive MCD has improved dramatically over time due to highly active antiretroviral therapy for HIV and modern biologic therapy for Castleman disease (most notably rituximab).

• #12 Diagnosis and Management of Castleman Disease- Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/features/diagnosis-and-management-of-castleman-disease/3/

  The optimal therapy for UCD is surgical resection, which is usually curative if the disease is amenable to complete resection. For disease that cannot be completely excised, radiation therapy is an option due to its high rates of objective response, including complete responses in nearly one-half of reported cases. […] For select patients who are not candidates for surgical excision, but who are also not candidates for radiation therapy based on the location of the disease, partial resection followed by clinical observation alone may result in lengthy remissions; however, such treatment warrants careful attention to local progression. […] Select patients who are asymptomatic with a low disease burden who cannot be treated with either surgery or radiation may be closely followed, given the often indolent nature of the disease. Systemic options for MCD, as necessary, should be considered for patients with symptomatic local disease who cannot be treated with surgery or radiation or for those whose disease fails to respond to such treatment.

• #13 Castleman Disease

  https://elsevier.health/en-US/preview/castleman-disease

  Treatment depends on the type of CD you have. […] UCD is usually treated with surgery to remove the swollen lymph nodes. It’s rare for UCD to come back after surgery. […] HHV-8-associated MCD is usually treated with medicine to boost your immune system. This is called immunotherapy. You may also be given steroids. […] If the disease comes back after treatment, you may need another round of immunotherapy. […] Many people with HHV-8-associated MCD also have HIV or AIDS. Treating HIV or AIDS also helps to control HHV-8-associated MCD. […] Idiopathic MCD is treated with immunotherapy. If immunotherapy doesn’t work, you may be given different medicines that lower the activity of the immune system. These are called immunosuppressants. […] If you have POEMS-associated MCD, treatment will target the POEMS syndrome.

• #14 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For HIV-negative patients, there may also be a role for concomitant antiviral therapy with ganciclovir. […] In HHV-8negative (idiopathic) MCD, the IL-6 inhibitor siltuximab (Sylvant) is the preferred therapy; it is the only drug approved by the US Food and Drug Administration (FDA) for this indication. […] If siltuximab is not available, the IL-6 inhibitor tocilizumab may be used in its place. […] AntiIL-6 treatment is continued until progression of disease in order to maintain the response and prevent an early relapse. […] Optimal treatment for siltuximab non-responders is not known. […] Systemic corticosteroids can provide symptomatic relief but do not predictably reduce tumor size. […] Radiation therapy with 30-45 Gy can result in complete and partial remission rates of 40% and 10%, respectively, but can cause radiation-induced fibrosis that makes subsequent surgical intervention more difficult.

• #15 Treatment – CDCN

  https://cdcn.org/treatment/

  The treatment options for Castleman disease are specific to the subtype diagnosed by your physician. […] Surgery is considered by experts to be the first-line treatment for almost all cases of UCD. […] If surgical excision is not possible, treatment is recommended for symptomatic patients. […] If symptoms are due to compression, then rituximab may be administered to attempt to shrink the enlarged lymph node. […] Some UCD patients may experience inflammatory symptoms (fever, fatigue) and elevated inflammatory laboratory markers after lymph node excision. […] These patients are sometimes treated with treatments frequently used to treat MCD, such as anti-IL-6 therapy with siltuximab. […] If these treatments are not effective, then radiation is sometimes tried. […] Your medical team will discuss the options and risk for surgery depending on the location of the enlarged lymph nodes.

• #16 Treatment – CDCN

  https://cdcn.org/treatment/

  The treatment options for Castleman disease are specific to the subtype diagnosed by your physician. […] Surgery is considered by experts to be the first-line treatment for almost all cases of UCD. […] If surgical excision is not possible, treatment is recommended for symptomatic patients. […] If symptoms are due to compression, then rituximab may be administered to attempt to shrink the enlarged lymph node. […] Some UCD patients may experience inflammatory symptoms (fever, fatigue) and elevated inflammatory laboratory markers after lymph node excision. […] These patients are sometimes treated with treatments frequently used to treat MCD, such as anti-IL-6 therapy with siltuximab. […] If these treatments are not effective, then radiation is sometimes tried. […] Your medical team will discuss the options and risk for surgery depending on the location of the enlarged lymph nodes.

• #17 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For HIV-negative patients, there may also be a role for concomitant antiviral therapy with ganciclovir. […] In HHV-8negative (idiopathic) MCD, the IL-6 inhibitor siltuximab (Sylvant) is the preferred therapy; it is the only drug approved by the US Food and Drug Administration (FDA) for this indication. […] If siltuximab is not available, the IL-6 inhibitor tocilizumab may be used in its place. […] AntiIL-6 treatment is continued until progression of disease in order to maintain the response and prevent an early relapse. […] Optimal treatment for siltuximab non-responders is not known. […] Systemic corticosteroids can provide symptomatic relief but do not predictably reduce tumor size. […] Radiation therapy with 30-45 Gy can result in complete and partial remission rates of 40% and 10%, respectively, but can cause radiation-induced fibrosis that makes subsequent surgical intervention more difficult.

• #18 Diagnosis and Management of Castleman Disease- Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/features/diagnosis-and-management-of-castleman-disease/3/

  The optimal therapy for UCD is surgical resection, which is usually curative if the disease is amenable to complete resection. For disease that cannot be completely excised, radiation therapy is an option due to its high rates of objective response, including complete responses in nearly one-half of reported cases. […] For select patients who are not candidates for surgical excision, but who are also not candidates for radiation therapy based on the location of the disease, partial resection followed by clinical observation alone may result in lengthy remissions; however, such treatment warrants careful attention to local progression. […] Select patients who are asymptomatic with a low disease burden who cannot be treated with either surgery or radiation may be closely followed, given the often indolent nature of the disease. Systemic options for MCD, as necessary, should be considered for patients with symptomatic local disease who cannot be treated with surgery or radiation or for those whose disease fails to respond to such treatment.

• #19 Castleman disease | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/castleman-disease?content_id=CON-20540888

  Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease (UCD). […] If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. […] You’ll likely need follow-up exams, including imaging and laboratory tests, to check that the disease hasn’t come back. […] Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. […] Siltuximab (Sylvant) is usually the first treatment for idiopathic MCD. […] People who are critically ill with idiopathic MCD often receive treatment with medicines called corticosteroids. […] When siltuximab doesn’t work, other treatments such as rituximab (Rituxan) and sirolimus (Rapamune) may be used.

• #20 Castleman Disease – StatPearls – NCBI Bookshelf

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

  Monoclonal antibodies interrupting the IL-6 signaling cascade, including the antiIL-6R antibody Tocilizumab and Siltuximab, an antiIL-6 antibody, tend to be highly effective and are considered to be the first-line therapy, especially for highly symptomatic patients without being HIV or HHV-8 positive. […] The pharmacist will ensure that the patient is on the appropriate analgesics, antiemetics, and appropriate antibiotics for pain and wound infection in the postoperative period. […] The outcomes and prognosis of CD depend on its cause and pathogenicity. However, prompt consultation with an interprofessional group of specialists is recommended to improve outcomes.

• #21 Treatment – CDCN

  https://cdcn.org/treatment/

  The treatment options for Castleman disease are specific to the subtype diagnosed by your physician. […] Surgery is considered by experts to be the first-line treatment for almost all cases of UCD. […] If surgical excision is not possible, treatment is recommended for symptomatic patients. […] If symptoms are due to compression, then rituximab may be administered to attempt to shrink the enlarged lymph node. […] Some UCD patients may experience inflammatory symptoms (fever, fatigue) and elevated inflammatory laboratory markers after lymph node excision. […] These patients are sometimes treated with treatments frequently used to treat MCD, such as anti-IL-6 therapy with siltuximab. […] If these treatments are not effective, then radiation is sometimes tried. […] Your medical team will discuss the options and risk for surgery depending on the location of the enlarged lymph nodes.

• #22 Get Castleman Disease Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/castleman-disease-treatment

  During this visit, you’ll also have a physical exam so your provider can check for swollen lymph nodes and other signs of Castleman disease, like an enlarged spleen or liver, or swelling in your feet, ankles or belly (abdomen). […] A lymph node biopsy is required to diagnose Castleman disease. We’ll remove a tissue sample from one of your lymph nodes, or the entire lymph node. […] Once your care team confirms your diagnosis, they’ll walk you through each step of your personalized treatment plan, going over what you can expect. […] If you have UCD, you’ll have surgery to remove any affected lymph nodes. […] MCD is harder to treat because it’s widespread throughout your body. We don’t usually recommend radiation or surgery. […] Having a rare condition like Castleman disease might be challenging, but that doesn’t mean it’s not treatable. Especially when you have a healthcare team that’s highly skilled and experienced in caring for people with this uncommon condition.

• #23 Castleman Disease: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17920-castleman-disease

  Castleman disease is a group of disorders that involve a hyperactive immune system. Typically, healthy immune systems activate to fight germs invading your body. Then, they settle down once the threat is gone. But if you have Castleman disease, your immune system remains activated. This leads to long-lasting inflammation that can damage organs. […] Treatment for Castleman disease varies according to the type. […] Surgery to remove the affected lymph nodes is the primary treatment for UCD. You may need radiation therapy or immunotherapy before surgery. These therapies shrink the growths on your lymph nodes, making them easier to remove. […] MCD is more difficult to treat than UCD. As its widespread, providers dont typically use surgery or radiation to treat it. Instead, treatment depends on whether you have HHV-8 and how severe the disease is.

• #24 Castleman disease | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/castleman-disease?content_id=CON-20540888

  Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease (UCD). […] If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. […] You’ll likely need follow-up exams, including imaging and laboratory tests, to check that the disease hasn’t come back. […] Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. […] Siltuximab (Sylvant) is usually the first treatment for idiopathic MCD. […] People who are critically ill with idiopathic MCD often receive treatment with medicines called corticosteroids. […] When siltuximab doesn’t work, other treatments such as rituximab (Rituxan) and sirolimus (Rapamune) may be used.

• #25 Treatment – CDCN

  https://cdcn.org/treatment/

  UCD patients should be evaluated for lymph node resectability, with surgery being the preferred method of treatment. […] Rituximab is recommended as first-line therapy for HHV-8+ MCD. […] The degree of immunosuppression is also milder than traditional chemotherapy drugs. […] First-line treatment for idiopathic multicentric Castleman disease is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] The amount of immunosuppression will be dependent on whether other drugs are used simultaneously. […] In critically ill patients, corticosteroids are recommended and adjuvant chemotherapy is needed if the patient is demonstrating disease progression while on siltuximab. […] In patients where siltuximab is not effective, other treatments such as rituximab and sirolimus (Rapamune) can be used.

• #26 Treatment – CDCN

  https://cdcn.org/treatment/

  UCD patients should be evaluated for lymph node resectability, with surgery being the preferred method of treatment. […] Rituximab is recommended as first-line therapy for HHV-8+ MCD. […] The degree of immunosuppression is also milder than traditional chemotherapy drugs. […] First-line treatment for idiopathic multicentric Castleman disease is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] The amount of immunosuppression will be dependent on whether other drugs are used simultaneously. […] In critically ill patients, corticosteroids are recommended and adjuvant chemotherapy is needed if the patient is demonstrating disease progression while on siltuximab. […] In patients where siltuximab is not effective, other treatments such as rituximab and sirolimus (Rapamune) can be used.

• #27 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  Treatment varies depending on the subtype of Castleman disease. […] For unicentric Castleman disease (UCD), surgical removal of the enlarged lymph node is considered the first-line treatment option and can be curative. […] If surgical excision is not possible, treatment is recommended for symptomatic patients. […] If symptoms are due to compression, then rituximab is recommended. […] If symptoms are due to an inflammatory syndrome, then anti-interleukin-6 (IL-6) therapy is recommended. […] If these treatments are not effective, radiation therapy may be needed. […] In HHV-8associated MCD, treatment with rituximab is highly effective. […] For patients with concomitant HIV infection and a low CD4 count and/or higher HIV load, antiretroviral therapy (ART) should be included with the rituximab.

• #28 Diagnosis and Management of Castleman Disease in: Journal of the National Comprehensive Cancer Network Volume 17 Issue 11.5 (2019)

  https://jnccn.org/view/journals/jnccn/17/11.5/article-p1417.xml

  Certainly, all patients with HIV should be started on antiretroviral therapy, he said. But unlike some viral diseases with associated lymphomas, you will not see a regression of Castleman disease with antiretroviral therapy directed at HIV alone. So patients will need Castleman disease-directed therapy. […] For HHV8-negative disease (iMCD), go down the siltuximab route for most patients, advised Dr. Abramson. […] Currently the data predominantly support siltuximab as initial therapy for iMCD.

• #29 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For HIV-negative patients, there may also be a role for concomitant antiviral therapy with ganciclovir. […] In HHV-8negative (idiopathic) MCD, the IL-6 inhibitor siltuximab (Sylvant) is the preferred therapy; it is the only drug approved by the US Food and Drug Administration (FDA) for this indication. […] If siltuximab is not available, the IL-6 inhibitor tocilizumab may be used in its place. […] AntiIL-6 treatment is continued until progression of disease in order to maintain the response and prevent an early relapse. […] Optimal treatment for siltuximab non-responders is not known. […] Systemic corticosteroids can provide symptomatic relief but do not predictably reduce tumor size. […] Radiation therapy with 30-45 Gy can result in complete and partial remission rates of 40% and 10%, respectively, but can cause radiation-induced fibrosis that makes subsequent surgical intervention more difficult.

• #30 Diagnosis and Management of Castleman Disease in: Journal of the National Comprehensive Cancer Network Volume 17 Issue 11.5 (2019)

  https://jnccn.org/view/journals/jnccn/17/11.5/article-p1417.xml

  The treatment of choice for UCD is surgical resection, which is associated with 90% relapse-free survival. Unresectable disease can be treated with local radiation, or rituximab and/or steroids can be used to reduce the mass with the goal of converting it to be surgically resectable. […] HHV8-positive MCD is generally associated with an IL-6-mediated inflammatory syndrome, and is most commonly seen in the setting of HIV (but may rarely occur in other immunocompromised patients). It is most commonly plasmacytic or mixed histology, with a male predominance. Patients with MCD are often quite ill and typically present with fevers, diffuse lymphadenopathy, hepatosplenomegaly, elevated inflammatory markers, cytopenias, and hypoalbuminemia. […] Prognosis for HHV8-positive MCD has improved dramatically over time due to highly active antiretroviral therapy for HIV and modern biologic therapy for Castleman disease (most notably rituximab).

• #31 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For HIV-negative patients, there may also be a role for concomitant antiviral therapy with ganciclovir. […] In HHV-8negative (idiopathic) MCD, the IL-6 inhibitor siltuximab (Sylvant) is the preferred therapy; it is the only drug approved by the US Food and Drug Administration (FDA) for this indication. […] If siltuximab is not available, the IL-6 inhibitor tocilizumab may be used in its place. […] AntiIL-6 treatment is continued until progression of disease in order to maintain the response and prevent an early relapse. […] Optimal treatment for siltuximab non-responders is not known. […] Systemic corticosteroids can provide symptomatic relief but do not predictably reduce tumor size. […] Radiation therapy with 30-45 Gy can result in complete and partial remission rates of 40% and 10%, respectively, but can cause radiation-induced fibrosis that makes subsequent surgical intervention more difficult.

• #32 What Is Multicentric Castleman Disease?

  https://www.healthline.com/health/multicentric-castleman-disease

  Multicentric Castleman disease is an immune system disease that affects the lymph nodes. Its one of two main types of Castleman disease. The other is unicentric Castleman disease. […] Treatment depends on the type and severity of the disease, so getting a timely diagnosis is vital to improving your odds of a healthy outcome. […] Diagnosing multicentric Castleman disease usually starts with a review of symptoms and your medical history. Lab tests and imaging are also necessary to make an accurate diagnosis. […] The only FDA-approved medication to treat multicentric Castleman disease is the anti-IL-6-targeted monoclonal antibody siltuximab (Sylvant). Treatment guidelines suggest this medication as the first-line treatment in people with multicentric Castleman disease who dont have HIV and HHV-8.

• #33 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For HIV-negative patients, there may also be a role for concomitant antiviral therapy with ganciclovir. […] In HHV-8negative (idiopathic) MCD, the IL-6 inhibitor siltuximab (Sylvant) is the preferred therapy; it is the only drug approved by the US Food and Drug Administration (FDA) for this indication. […] If siltuximab is not available, the IL-6 inhibitor tocilizumab may be used in its place. […] AntiIL-6 treatment is continued until progression of disease in order to maintain the response and prevent an early relapse. […] Optimal treatment for siltuximab non-responders is not known. […] Systemic corticosteroids can provide symptomatic relief but do not predictably reduce tumor size. […] Radiation therapy with 30-45 Gy can result in complete and partial remission rates of 40% and 10%, respectively, but can cause radiation-induced fibrosis that makes subsequent surgical intervention more difficult.

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

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

  • We recommend first-line treatment with anti–interleukin 6 (anti–IL-6) for all patients with idiopathic multicentric Castleman disease. […] • For patients with severe disease, we recommend the accelerated weekly dosing of anti–IL-6 therapy in combination with high-dose corticosteroids with close monitoring and consideration of cytotoxic chemotherapy. […] • In mild or moderate cases, we follow standard anti–IL-6 dosing schedules and consider clinical trial enrollment, rituximab, or other immune modulators for nonresponders. […] The treatment of iMCD is complicated by a limited understanding of the etiology and pathogenesis. […] 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.

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

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

  To treat patients with iMCD effectively, we recommend a careful confirmation of the diagnosis with the exclusion of alternative diagnoses and other forms of CD. […] When time allows, pathologic confirmation should be obtained at an experienced center, given the high number of discordant interpretations. […] Although siltuximab is the first-line therapy for all patients with iMCD, we recommend that the severity of disease be determined at diagnosis and the therapeutic approach guided accordingly. […] We recommend starting with 4 weekly doses of siltuximab and high-dose corticosteroids for severe iMCD. […] Furthermore, patients with severe iMCD should be treated with multiagent chemotherapy at the first signs of disease progression or refractory cytokine storm after siltuximab has been initiated.

• #36 Treatment – CDCN

  https://cdcn.org/treatment/

  UCD patients should be evaluated for lymph node resectability, with surgery being the preferred method of treatment. […] Rituximab is recommended as first-line therapy for HHV-8+ MCD. […] The degree of immunosuppression is also milder than traditional chemotherapy drugs. […] First-line treatment for idiopathic multicentric Castleman disease is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] The amount of immunosuppression will be dependent on whether other drugs are used simultaneously. […] In critically ill patients, corticosteroids are recommended and adjuvant chemotherapy is needed if the patient is demonstrating disease progression while on siltuximab. […] In patients where siltuximab is not effective, other treatments such as rituximab and sirolimus (Rapamune) can be used.

• #37 Treatment – CDCN

  https://cdcn.org/treatment/

  UCD patients should be evaluated for lymph node resectability, with surgery being the preferred method of treatment. […] Rituximab is recommended as first-line therapy for HHV-8+ MCD. […] The degree of immunosuppression is also milder than traditional chemotherapy drugs. […] First-line treatment for idiopathic multicentric Castleman disease is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] The amount of immunosuppression will be dependent on whether other drugs are used simultaneously. […] In critically ill patients, corticosteroids are recommended and adjuvant chemotherapy is needed if the patient is demonstrating disease progression while on siltuximab. […] In patients where siltuximab is not effective, other treatments such as rituximab and sirolimus (Rapamune) can be used.

• #38 Castleman disease | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/castleman-disease?content_id=CON-20540888

  Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease (UCD). […] If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. […] You’ll likely need follow-up exams, including imaging and laboratory tests, to check that the disease hasn’t come back. […] Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. […] Siltuximab (Sylvant) is usually the first treatment for idiopathic MCD. […] People who are critically ill with idiopathic MCD often receive treatment with medicines called corticosteroids. […] When siltuximab doesn’t work, other treatments such as rituximab (Rituxan) and sirolimus (Rapamune) may be used.

• #39 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For patients with unresectable disease with compressive symptoms can be treated as described for HIV-negative MCD. […] Disease response is assessed by evaluating lymph node size via imaging, as well as biochemical values. […] The frequency of monitoring depends on the severity of presentation. […] For severe presentation, initially monitoring should be daily. […] For less severe presentation, weekly assessment of clinical symptoms and laboratory values is necessary, then biweekly until maximum benefit has occurred. […] Radiologic evaluation of lymph nodes with computed tomography is as follows: Begin at 6 weeks after treatment initiation. […] Repeat every 3 months until maximum response is achieved, then reduce to every 6 to 12 months.

• #40 Castleman Disease Treatment & Management: Approach Considerations, Unicentric Castleman Disease, Multicentric Castleman Disease

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

  For patients with unresectable disease with compressive symptoms can be treated as described for HIV-negative MCD. […] Disease response is assessed by evaluating lymph node size via imaging, as well as biochemical values. […] The frequency of monitoring depends on the severity of presentation. […] For severe presentation, initially monitoring should be daily. […] For less severe presentation, weekly assessment of clinical symptoms and laboratory values is necessary, then biweekly until maximum benefit has occurred. […] Radiologic evaluation of lymph nodes with computed tomography is as follows: Begin at 6 weeks after treatment initiation. […] Repeat every 3 months until maximum response is achieved, then reduce to every 6 to 12 months.

• #41 Get Castleman Disease Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/castleman-disease-treatment

  Castleman disease is complex, so you’ll have a care team of providers from many specialties. They all work closely together to give you highly personalized care with the best results. […] Castleman disease is rare, but our team is one of the few in the country that treats it regularly. We have years of experience treating this condition, including unicentric Castleman disease (UCD) and all subtypes of multicentric Castleman disease (MCD). […] We have expertise in managing all types of Castleman disease including complications and treatment side effects. […] We’re here to listen to yours so we can make sure treatment fits your diagnosis, lifestyle and needs. This includes caring for you mentally, emotionally and physically. […] Your first visit is a time for you and your provider to get to know each other. We’re here to help ease your fears and guide you through every step of confirming your diagnosis.

• #42 Castleman Disease

  https://www.ascp.org/education/topics/castleman-disease?srsltid=AfmBOooi96BLQYyRXZ93_a2fjVJdNQ0WYTvYUPR9M9D-UrJ3DdYJ4qg5

  Multicentric Castleman disease is a rare, complex disease that requires close collaboration between the pathologist, laboratory team, and clinicians to diagnose and direct therapy for optimal patient outcomes. […] ASCP is committed to helping you be equipped with the knowledge, skills, and competencies needed to ensure patients with Castleman disease are accurately diagnosed so they can receive timely, appropriate therapies to manage their disease. […] This microlearning series includes the following topics: […] Clinical practice guidelines for diagnosis and treatment […] Current/emerging therapies in the management of multicentric Castleman disease […] The importance of a multidisciplinary team-based approach to the diagnosis and management of multicentric Castleman disease. […] ASCP developed a CME/CMLE-accredited Tweetorial designed to highlight key concepts and clinical practice guidelines regarding the multidisciplinary diagnosis and treatment of patients with multicentric Castleman disease.

• #43 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. […] This activity explains the evaluation and treatment of Castleman disease and highlights the role of the interprofessional team in evaluating and treating patients with this condition. […] Castleman disease is a rare disease, with minimum availability of therapeutic options. As a unicentric Castleman disease lesion is localized, complete surgical resection of the tumor is the best treatment available. […] Many treatment options are available for treating multicentric CD, including surgery, cytotoxic chemotherapy with or without corticosteroids, and autologous stem cell transplantation (ASCT) with varying outcomes. […] Better results have been observed by targeting CD20 and IL-6 pathways and HHV-8 replication.

• #44 Castleman Disease

  https://www.ascp.org/education/topics/castleman-disease?srsltid=AfmBOooi96BLQYyRXZ93_a2fjVJdNQ0WYTvYUPR9M9D-UrJ3DdYJ4qg5

  Multicentric Castleman disease is a rare, complex disease that requires close collaboration between the pathologist, laboratory team, and clinicians to diagnose and direct therapy for optimal patient outcomes. […] ASCP is committed to helping you be equipped with the knowledge, skills, and competencies needed to ensure patients with Castleman disease are accurately diagnosed so they can receive timely, appropriate therapies to manage their disease. […] This microlearning series includes the following topics: […] Clinical practice guidelines for diagnosis and treatment […] Current/emerging therapies in the management of multicentric Castleman disease […] The importance of a multidisciplinary team-based approach to the diagnosis and management of multicentric Castleman disease. […] ASCP developed a CME/CMLE-accredited Tweetorial designed to highlight key concepts and clinical practice guidelines regarding the multidisciplinary diagnosis and treatment of patients with multicentric Castleman disease.

• #45 Get Castleman Disease Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/castleman-disease-treatment

  Castleman disease is complex, so you’ll have a care team of providers from many specialties. They all work closely together to give you highly personalized care with the best results. […] Castleman disease is rare, but our team is one of the few in the country that treats it regularly. We have years of experience treating this condition, including unicentric Castleman disease (UCD) and all subtypes of multicentric Castleman disease (MCD). […] We have expertise in managing all types of Castleman disease including complications and treatment side effects. […] We’re here to listen to yours so we can make sure treatment fits your diagnosis, lifestyle and needs. This includes caring for you mentally, emotionally and physically. […] Your first visit is a time for you and your provider to get to know each other. We’re here to help ease your fears and guide you through every step of confirming your diagnosis.

• #46 Castleman Disease

  https://elsevier.health/en-US/preview/castleman-disease

  Follow these instructions at home: Take over-the-counter and prescription medicines only as told by your health care provider. […] If you take medicine or are having radiation for CD, make sure you talk with your provider about possible side effects. […] Return to your normal activities as told by your provider. Ask your provider what activities are safe for you. […] Keep all follow-up and checkup visits. You’ll need regular checkups for about 5 years to make sure CD does not come back. Checkups may include a physical exam, lab tests, and imaging tests. […] Contact a health care provider if: You have chills or a fever. […] Your arms or legs are swollen. […] You get any new symptoms. […] Symptoms of CD come back after treatment. […] Get help right away if: You have trouble breathing.

• #47 Castleman Disease

  https://elsevier.health/en-US/preview/castleman-disease

  Follow these instructions at home: Take over-the-counter and prescription medicines only as told by your health care provider. […] If you take medicine or are having radiation for CD, make sure you talk with your provider about possible side effects. […] Return to your normal activities as told by your provider. Ask your provider what activities are safe for you. […] Keep all follow-up and checkup visits. You’ll need regular checkups for about 5 years to make sure CD does not come back. Checkups may include a physical exam, lab tests, and imaging tests. […] Contact a health care provider if: You have chills or a fever. […] Your arms or legs are swollen. […] You get any new symptoms. […] Symptoms of CD come back after treatment. […] Get help right away if: You have trouble breathing.

• #48 Castleman Disease – StatPearls – NCBI Bookshelf

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

  Monoclonal antibodies interrupting the IL-6 signaling cascade, including the antiIL-6R antibody Tocilizumab and Siltuximab, an antiIL-6 antibody, tend to be highly effective and are considered to be the first-line therapy, especially for highly symptomatic patients without being HIV or HHV-8 positive. […] The pharmacist will ensure that the patient is on the appropriate analgesics, antiemetics, and appropriate antibiotics for pain and wound infection in the postoperative period. […] The outcomes and prognosis of CD depend on its cause and pathogenicity. However, prompt consultation with an interprofessional group of specialists is recommended to improve outcomes.

• #49 Castleman Disease

  https://elsevier.health/en-US/preview/castleman-disease

  Follow these instructions at home: Take over-the-counter and prescription medicines only as told by your health care provider. […] If you take medicine or are having radiation for CD, make sure you talk with your provider about possible side effects. […] Return to your normal activities as told by your provider. Ask your provider what activities are safe for you. […] Keep all follow-up and checkup visits. You’ll need regular checkups for about 5 years to make sure CD does not come back. Checkups may include a physical exam, lab tests, and imaging tests. […] Contact a health care provider if: You have chills or a fever. […] Your arms or legs are swollen. […] You get any new symptoms. […] Symptoms of CD come back after treatment. […] Get help right away if: You have trouble breathing.

• #50 Get Castleman Disease Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/castleman-disease-treatment

  Castleman disease is complex, so you’ll have a care team of providers from many specialties. They all work closely together to give you highly personalized care with the best results. […] Castleman disease is rare, but our team is one of the few in the country that treats it regularly. We have years of experience treating this condition, including unicentric Castleman disease (UCD) and all subtypes of multicentric Castleman disease (MCD). […] We have expertise in managing all types of Castleman disease including complications and treatment side effects. […] We’re here to listen to yours so we can make sure treatment fits your diagnosis, lifestyle and needs. This includes caring for you mentally, emotionally and physically. […] Your first visit is a time for you and your provider to get to know each other. We’re here to help ease your fears and guide you through every step of confirming your diagnosis.

• #51

  https://penntoday.upenn.edu/news/penn-medicine-first-new-subtype-castleman-disease-discovered-45-years

  A newly identified subtype of Castleman disease will help diagnose and properly treat thousands of patients who have been caught between existing classification systems, marking the first major discovery of its kind in 45 years. […] This discovery is a game-changer for Castleman disease patients, says David Fajgenbaum, an associate professor of translational medicine and human genetics and co-founder of the Castleman Disease Collaborative Network (CDCN). […] Now we can match these patients about 15% of all Castleman cases with the right treatments for their specific condition. […] The study finds that OligoCD patients exhibit fewer and less severe symptoms than those with iMCD, suggesting that surgical removal of affected lymph nodes effective for the milder UCD may be more appropriate than the intense treatments used for idiopathic multicentric Castleman disease (iMCD). […] However, the team emphasizes the need for further research to refine treatment guidelines and a further understanding of how OligoCD develops, with ACCELERATE poised to provide ongoing insights.

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

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

  For patients who have iMCD with mild or moderate disease activity, siltuximab monotherapy administered every 3 weeks is the recommended first-line therapy. […] For patients with mild or moderate disease that does not respond to siltuximab, we suggest enrollment in an open clinical trial, given the limited data on second-line therapies. […] Importantly, all patients should be instructed to connect with the CDCN upon diagnosis (www.CDCN.org, [email protected]), so that they can learn about their illness, connect with a CD-experienced specialist in a timely manner should an urgent consultation later become necessary, connect with the larger CD patient community, and be informed about opportunities to contribute medical data to the ACCELERATE Castleman disease registry (www.CDCN.org/ACCELERATE) and contribute blood and/or lymph node tissue to research.

• #53 Treatment – CDCN

  https://cdcn.org/treatment/

  Treatment should be directed at the POEMS syndrome. […] The side effects of treatment will be very specific to the treatment chosen by the medical team. […] The good news is that in the last decade, an explosion in Castleman disease research has identified new targets and there is hope new treatments will emerge soon.

• #54 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. […] This activity explains the evaluation and treatment of Castleman disease and highlights the role of the interprofessional team in evaluating and treating patients with this condition. […] Castleman disease is a rare disease, with minimum availability of therapeutic options. As a unicentric Castleman disease lesion is localized, complete surgical resection of the tumor is the best treatment available. […] Many treatment options are available for treating multicentric CD, including surgery, cytotoxic chemotherapy with or without corticosteroids, and autologous stem cell transplantation (ASCT) with varying outcomes. […] Better results have been observed by targeting CD20 and IL-6 pathways and HHV-8 replication.

• #55 Diagnosis and Management of Castleman Disease in: Journal of the National Comprehensive Cancer Network Volume 17 Issue 11.5 (2019)

  https://jnccn.org/view/journals/jnccn/17/11.5/article-p1417.xml

  The treatment of choice for UCD is surgical resection, which is associated with 90% relapse-free survival. Unresectable disease can be treated with local radiation, or rituximab and/or steroids can be used to reduce the mass with the goal of converting it to be surgically resectable. […] HHV8-positive MCD is generally associated with an IL-6-mediated inflammatory syndrome, and is most commonly seen in the setting of HIV (but may rarely occur in other immunocompromised patients). It is most commonly plasmacytic or mixed histology, with a male predominance. Patients with MCD are often quite ill and typically present with fevers, diffuse lymphadenopathy, hepatosplenomegaly, elevated inflammatory markers, cytopenias, and hypoalbuminemia. […] Prognosis for HHV8-positive MCD has improved dramatically over time due to highly active antiretroviral therapy for HIV and modern biologic therapy for Castleman disease (most notably rituximab).

• #56 Castleman Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/castleman-disease

  Our program includes experts in many medical specialties throughout CHOP. We can work with them as necessary to treat your child. […] After using state-of-the-art testing to diagnose your child, we will work with you to create a personalized treatment plan. Treatment will vary depending on which type of Castleman disease your child has. […] If your child is diagnosed with unicentric Castleman disease (UCD), we can treat them by surgically removing the lymph node that is affected or using medicine to shrink the lymph node. […] If your child is diagnosed with multicentric Castleman disease (MCD), treatment can include: Regularly prescribed medications, such as immunotherapy drugs like siltuximab, rituximab, or rapamycin. […] Children with unicentric Castleman disease usually do well once the affected lymph node is removed. […] Our team at CHOP will work together to determine the treatment that will result in your child’s best possible health.

• #57 Diagnosis and Management of Castleman Disease in: Journal of the National Comprehensive Cancer Network Volume 17 Issue 11.5 (2019)

  https://jnccn.org/view/journals/jnccn/17/11.5/article-p1417.xml

  The treatment of choice for UCD is surgical resection, which is associated with 90% relapse-free survival. Unresectable disease can be treated with local radiation, or rituximab and/or steroids can be used to reduce the mass with the goal of converting it to be surgically resectable. […] HHV8-positive MCD is generally associated with an IL-6-mediated inflammatory syndrome, and is most commonly seen in the setting of HIV (but may rarely occur in other immunocompromised patients). It is most commonly plasmacytic or mixed histology, with a male predominance. Patients with MCD are often quite ill and typically present with fevers, diffuse lymphadenopathy, hepatosplenomegaly, elevated inflammatory markers, cytopenias, and hypoalbuminemia. […] Prognosis for HHV8-positive MCD has improved dramatically over time due to highly active antiretroviral therapy for HIV and modern biologic therapy for Castleman disease (most notably rituximab).

• #58 What Is Multicentric Castleman Disease?

  https://www.healthline.com/health/multicentric-castleman-disease

  If treatment begins early enough, the outlook for someone with multicentric Castleman disease is generally encouraging. […] Castleman disease can be a life threatening disease without prompt and effective treatment. Potential complications of the disease include severe damage to multiple organs and cancer. […] Because the cause is usually unknown, theres little you can do to lower your risk of developing it. However, responding quickly to symptoms can greatly improve your odds of having a positive outcome.

• #59 Castleman disease of plasma cell type accompanied with bronchiolitis obliterans: a case report and review of the literature | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-023-04285-2

  Castleman disease is a rare lymphoproliferative disorder with a variety of symptoms. […] At present, the treatment of Castleman disease accompanied with bronchiolitis obliterans is mostly based on experiences or previous case reports, and there is no standard treatment. […] To control the progression of BO, the effective adjuvant therapy should be given after resection of the tumor, such as glucocorticoid or immunosuppressive medication. […] Following surgery, the patients symptoms were partially resolved, and he is now fully capable of caring for himself. […] The prognosis of CD accompanied with BO is often poor, even after surgical resection of the tumor, and it is also difficult to prevent the progression of BO. […] Therefore, the immunosuppressive medication was administered postoperatively and followed up regularly after discharge. […] Early detection of the disease, timely resection of the tumor, and effective adjuvant therapy may prevent the progression of BO. […] It is important to administer treatment promptly to enhance patients quality of life, their prognosis, and lower the death rate.

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