Materiały źródłowe

• #1 Treatment – CDCN

  https://cdcn.org/treatment/

  The treatment options for Castleman disease are specific to the subtype diagnosed by your physician. […] Unicentric Castleman disease (UCD): Surgery is considered by experts to be the first-line treatment for almost all cases of UCD. A patient is considered cured of UCD if their enlarged lymph node or nodes are removed completely and any previous clinical/laboratory abnormalities return to normal. […] 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.

• #1 Castleman disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/symptoms-causes/syc-20543017

  Treatment and outlook vary depending on the type of Castleman disease you have. Unicentric Castleman disease, which is the type that involves only one enlarged lymph node, can usually be successfully treated with surgery. […] The best treatment for oligocentric Castleman disease, which involves a few enlarged lymph nodes and has limited symptoms, is not known but is thought to be similar to the treatment for unicentric Castleman disease. […] While not all people with MCD respond to the first treatment, there are medicines that work to treat HHV-8-associated MCD and idiopathic MCD.

• #1 Castleman disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/diagnosis-treatment/drc-20543055

  Treatment depends on the type of Castleman disease you have. […] Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease (UCD). If the lymph node is in the chest or abdomen, major surgery may be needed. […] Surgery to remove the enlarged lymph node usually cures UCD. However, UCD sometimes comes back. If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. If medicines don’t work, radiation therapy may be an option. […] Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. Rituximab is highly effective, but sometimes medicines called antivirals and chemotherapies are needed. Antiviral medicines can block the activity of HHV-8 or HIV, and chemotherapies can get rid of extra immune cells.

• #1 Update and new approaches in the treatment of Castleman disease | JBM

  https://www.dovepress.com/update-and-new-approaches-in-the-treatment-of-castleman-disease-peer-reviewed-fulltext-article-JBM

  Complete surgical resection is curative for UCD, leading to excellent long-term outcomes with 10-year overall survival rates in excess of 95%. […] If this is not feasible, the optimal treatment strategy is not well-defined. […] A range of systemic therapies have been utilized in MCD, including cytotoxic chemotherapy, antibodies directed against CD20 as well as IL-6 and its receptor, immunomodulators, bortezomib, and antiviral agents. […] Rituximab, a humanized monoclonal antibody to CD20 (which is typically found on CD plasmablasts), has shown significant activity in HIV-positive and idiopathic MCD patients, when used either as monotherapy or in combination with chemotherapy. […] The advent of monoclonal antibody therapy targeting the IL-6 pathway has significantly impacted the treatment of patients with idiopathic MCD.

• #1 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. […] 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.

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

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

  Optimal treatment for siltuximab non-responders is not known. Studied options include other immunomodulatory therapies, rituximab, and cytotoxic chemotherapies. Single-agent and combination chemotherapy yields symptomatic relief and a partial response in many patients. However, symptoms recur when treatment is stopped, necessitating intermittent maintenance therapy, often lifelong. […] Surgery is usually curative in unicentric Castleman disease. Even in patients whose lesions cannot be completely resected, outcomes remain favorable. […] 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.

• #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. […] In the meantime, treatment involves customizing the right therapies based on your unique diagnosis. […] 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. […] Your provider may use: Corticosteroids. These medications reduce inflammation and help provide symptom relief.

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

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

  In HHV-8associated MCD, treatment with rituximab is highly effective. If relapse occurs, treatment with rituximab should be repeated. Rituximab may be combined with prednisone and/or liposomal doxorubicin. […] Siltuximab (Sylvant) is the only drug approved by the US Food and Drug Administration (FDA) for HHV-8negative MCD. Siltuximab is a monoclonal antibody that binds IL-6. Where available, siltuximab is the preferred therapy, based on its benefit in the only randomized trial and its approval by the FDA; National Comprehensive Cancer Network (NCCN) and Castleman Disease Collaborative Network (CDCN) guidelines recommend siltuximab as the preferred primary treatment. […] AntiIL-6 treatment is continued until progression of disease in order to maintain the response and prevent an early relapse.

• #1 Castleman disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/diagnosis-treatment/drc-20543055

  Siltuximab (Sylvant) is usually the first treatment for idiopathic MCD. In the U.S., siltuximab is the only medicine approved by the Food and Drug Administration (FDA) for the treatment of idiopathic MCD. People who get better after taking siltuximab tend to have successful long-term treatment. This drug blocks the action of a protein called interleukin-6. The bodies of people who have idiopathic MCD produce too much of this protein. […] People who are critically ill with idiopathic MCD often receive treatment with medicines called corticosteroids. They also may need chemotherapy. Corticosteroids such as prednisone can help control inflammation. Chemotherapy can get rid of immune cells that are causing problems. […] When siltuximab doesn’t work, other treatments such as rituximab (Rituxan) and sirolimus (Rapamune) may be used.

• #1 Treatment – CDCN

  https://cdcn.org/treatment/

  Anti-IL-6 therapy is effective in about 34-50% of patients, but some patients do not respond to it. […] 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. […] 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. […] A clinical trial for the medication sirolimus for iMCD patients who did not improve on siltuximab or tocilizumab was recently conducted.

• #1 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. […] We recommend consulting with a physician experienced in treating CD when a second-line therapy is selected. […] Importantly, all patients should be instructed to connect with the CDCN upon diagnosis, 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.

• #1 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 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. […] For a patient with severe disease that is causing neutropenia or thrombocytopenia, we would not delay siltuximab treatment and would provide support with transfusions and antibiotics as necessary.

• #1 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. […] 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.

• #1 Castleman Disease: Types, Symptoms & Treatment

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

  Chemotherapy drugs. Anticancer medications slow the overgrowth of cells in your lymphatic system. Rituximab is a commonly prescribed medication used to treat HHV-8-associated MCD. […] Immunotherapy. This treatment includes monoclonal antibodies that help calm your bodys immune response. Siltuximab (Sylvant) is the only iMCD therapy approved for use by the U.S. Food and Drug Administration (FDA). […] Antiviral drugs. You may need antiviral drugs to treat HIV or HHV-8 if you have HHV-8-associated MCD. […] Cleveland Clinic provides expert, personalized treatment for Castleman disease using the most advanced therapies, from medications to surgery.

• #1 Successful treatment of multicentric Castleman’s disease with combined immunochemotherapy in an AIDS patient with multiorgan failure | Leukemia

  https://www.nature.com/articles/leu200854

  Here we describe the complicated course of a 33-year-old African male with AIDS, with multiorgan failure due to multicentric Castleman’s disease (MCD). […] Use of rituximab as a salvage therapy for HIV-associated multicentric Castleman disease. […] Remission of HHV-8 and HIV-associated multicentric Castleman disease with ganciclovir treatment.

• #1 Castleman Disease Medication: Immunomodulators

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

  Siltuximab is the only agent approved by the US Food and Drug Administration for use in Castleman disease specifically, for idiopathic multicentric Castleman disease. It is also listed as the preferred primary treatment in National Comprehensive Cancer Network guidelines. […] Rituximab and tocilizumab have been used off-label for multicentric Castleman disease. Rituximab is highly effective in human herpesvirus-8 (HHV-8) associated multicentric Castleman disease. […] Siltuximab is a monoclonal antibody that binds IL-6 and prevents the binding of IL-6 to both soluble and membrane-bound IL-6 receptors. It is approved by the FDA with an indication for multicentric Castleman disease in patients who are negative for HIV and human herpesvirus-8. […] Tocilizumab is an IL-6 receptor antagonist. It has been used off-label for multicentric Castleman disease. […] Rituximab is a humanized monoclonal antibody that binds to CD20 antigen, which results in decreased IL-6 production. It has been used off-label for multicentric Castleman disease.

• #1 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  Treatment with tocilizumab (an anti-interleukin 6 receptor antibody) should be considered in cases with severe inflammatory symptoms or organ damage in the kidney, the lung and the others (moderate to severe disease in the severity classification system). Even cases with mild disease, administration of tocilizumab may be considered when the progression of clinical symptoms or organ disorders cannot be sufficiently controlled with steroid alone. Before starting administration of tocilizumab, physicians should explain the possibility of lifelong needs of its administration to patients. Usually, 8 mg/kg of tocilizumab is administered intravenously every two weeks (the dosing interval can be shortened up to one week depending on symptoms). Tocilizumab may be used as an initial treatment in combination with corticosteroids, or alone when corticosteroid treatment is inappropriate due to comorbidities or other reasons. In many cases, once treatment with tocilizumab is started, various systemic inflammatory symptoms and abnormal laboratory values promptly improve. It is often possible to reduce/discontinue the dose of steroids that are used in combination. Also, the enlarged spleen and lymph nodes may gradually shrink. Side effects include headache, upper respiratory tract inflammation, itching, rash, and allergies. Many of these are minor, but serious infections such as pneumonia and septicemia have also been reported. During the treatment with tocilizumab, CRP levels will not increase even in serious infection; therefore, care must be taken not to overlook infectious diseases. Anaphylaxis has been observed in over 1% of cases. Generally, treatment with tocilizumab should not be discontinued once started, but if it is inevitable to halt, temporarily administer or increase steroid to prevent the rebound of inflammatory symptoms. When the treatment with tocilizumab is started, the interleukin 6 concentration in the serum (insurance-inapplicable examination in Japan) jumps up because the interleukin 6 cannot bind to the receptor and is not removed from the plasma.

• #1 Update and new approaches in the treatment of Castleman disease | JBM

  https://www.dovepress.com/update-and-new-approaches-in-the-treatment-of-castleman-disease-peer-reviewed-fulltext-article-JBM

  Siltuximab has regulatory approval in the USA and Europe for the treatment of HIV-negative and HHV-8-negative MCD, while tocilizumab has approval for use in Japan. […] Tocilizumab therapy resulted in significant reduction in lymphadenopathy (30% reduction in the mean short axis of involved lymph nodes) for 52% of patients after 1 year of treatment. […] The prognosis of CD is variable and depends predominantly on disease subtype.

• #1 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  Treatment for CD […] Unicentric (localized) Castleman disease (UCD) […] In most cases, cure can be expected by local therapy (such as surgical resection). It is important to completely remove the lesion. If a recurrence occurs after surgical resection, or if it is difficult to resect the entire lesion and systemic inflammatory symptoms are present, treatment strategies for idiopathic MCD may be conducted. […] Idiopathic multicentric Castleman disease (iMCD) […] Patients with no or modest symptoms may be observed without treatments; but in many cases, therapeutic intervention is necessary to alleviate symptoms such as fatigue. When the symptoms of systemic inflammation are mild, the symptoms may be alleviated with low to intermediate doses of corticosteroids (about 0.3 mg/kg prednisolone in the absence of signs of organ damage, about 0.5 to 1 mg/kg when organ damages are observed). After the symptoms are improved, the dose of corticosteroids should be gradually tapered. In the cases with prolonged administration of corticosteroids, close attention should be paid to the onset of diabetes, osteoporosis, and infections caused by herpes virus and fungi.

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

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

  Regularly prescribed medications like glucocorticoids (often called steroids), like prednisone. This will help control inflammation in your child’s body. […] Chemotherapy describes powerful medicines that can help slow the overgrowth of cells in your child’s lymph nodes. This may be needed if your child’s symptoms do not respond to immunotherapy. […] If your child has HHV-8-associated MCD, antiviral drugs can be used to block the activity of one or both viruses.

• #1 Castleman Disease Treatment, Diagnosis, Prognosis & Symptoms

  https://www.medicinenet.com/castleman_disease/article.htm

  Corticosteroid drugs suppress the immune response. […] These chemotherapy drugs are most often used in Castleman disease: carmustine (BiCNU), cladribine (Leustatin), chlorambucil (Leukeran), cyclophosphamide (Cytoxan), doxorubicin (Adriamycin, Rubex), etoposide (Vepesid), melphalan (Alkeran), vinblastine, and vincristine (Oncovin). […] Chemotherapy and corticosteroids may also be combined with radiation therapy to destroy the abnormal tissue. […] Immunotherapy involves administering drugs that boost or strengthen the body’s natural immune response. […] Immunomodulating drugs such as thalidomide (Thalomid) and lenalidomide (Revlimid) are used to treat multiple myeloma and some lymphomas, and also can be helpful for some people with Castleman disease. […] Interferon-alfa (a man-made version of an immune-boosting substance produced by the body) is occasionally used to treat Castleman disease.

• #1 New guidelines frame treatment options for Castleman disease | MDedge

  https://ma1.mdedge.com/content/new-guidelines-frame-treatment-options-castleman-disease

  Second-line therapy should include rituximab, and immunomodulatory/immunosuppressive agents or steroids may be added, according to the guidelines. […] Third line therapy is less well defined, according to the guidelines, and experts generally recommended immunomodulatory/immunosuppressive agents such as cyclosporine A, sirolimus, thalidomide, and lenalidomide. […] Cytotoxic chemotherapy has a high response rate but also a high rate of relapse and significant toxicities, according to the data analysis conducted as part of the guideline development process. […] Patients who are literally dying in the intensive care unit, given the right combination chemotherapy, can improve within days to weeks and can even leave the hospital, Dr. Fajgenbaum said. […] So we recommended a really quite aggressive approach for these patients. […] Investigators in the Castleman Disease Collaborative Network (CDCN) set up an international registry to collect treatment and outcome data for 500 patients. […] Some of the drugs are demonstrating efficacy in small numbers, he said.

• #1 Castleman Disease | MDedge

  https://www.mdedge.com/content/castleman-disease

  Rituximab is a monoclonal chimeric antibody that targets CD20 on B cells, thus leading to B-cell lymphodepletion via activating complement-dependent cytotoxicity and antibody-dependent cell-mediated cytotoxicity. […] Siltuximab is a chimeric human-mouse monoclonal antibody to IL-6 that has been approved for treatment of patients with MCD who are both HIV negative and HHV-8 negative. […] Tocilizumab targets the IL-6R. The antibody has shown improvement in a study in HIV seronegative adults with MCD. […] Bortezomib is a proteasome inhibitor that inhibits the NF-kappa B pathway, which induces the expression of numerous proinflammatory proteins, including IL-6. It is recommended for relapsed or refractory disease. […] Anakinra is a recombinant IL-1R antagonist that blocks IL-1 effects and controls disease by decreasing IL-6 production. […] Successful treatment has been achieved through targeting HHV-8 replication, CD20, and IL-6 and anti–IL-6R antibodies. Although surgical resection continues to be the standard of therapy for UCD, the management of MCD and relapsed or refractory disease continues to evolve.

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

  https://www.cancertherapyadvisor.com/features/diagnosis-and-management-of-castleman-disease/7/

  Castleman disease is an uncommon lymphoproliferative disorder that continues to pose clinical challenges. […] Although surgical resection remains the standard therapy for unicentric disease, the landscape for the management of multicentric disease continues to evolve. […] Rituximab monotherapy is the current mainstay of therapy, and novel agents targeting interleukin 6 represent exciting new additions to the treatment armamentarium. […] Single-agent and combination chemotherapies as well as antiviral therapy provide adjunctive support, particularly in the setting of relapsed or refractory disease. […] The ongoing exploration of antiviral and novel strategies, such as proteasome inhibition, is warranted. […] The management of Castleman disease also requires careful attention to potential concomitant infections, malignancies, and associated syndromes.

• #1 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. This will help stop your child’s body from making too much of the protein found in people who have MCD.

• #1 Castleman Disease | MDedge

  https://www.mdedge.com/content/castleman-disease

  An understanding of the disease pathogenesis has led to the discovery of therapeutic agents that target human herpesvirus-8 replication, CD20, and IL-6 and IL-6R antibodies. […] Treatment options range from local surgical excision to systemic treatments. Newer therapies include monoclonal antibodies against both IL-6 and CD20 and a few other targets in the inflammatory cascade. […] For UCD, surgery is the mainstay of treatment. In surgically unresectable cases, radiation therapy is helpful for local disease control. Alternatively, neoadjuvant chemotherapy and rituximab are used. Corticosteroids are generally used to treat acute exacerbations and as adjuncts to chemotherapy. […] For MCD, the treatment approach depends on the HIV and HHV-8 status of the patient. For patients with HHV-8 infection, both with and without HIV co-infection, antiviral agents, such as ganciclovir, foscarnet, or cidofovir, have shown in vitro activity against HHV-8 but with limited clinical success. In patients infected with HIV, the aim of treating with HAART is to control the disease, prevent opportunistic infections, and improve tolerance to chemotherapy. Rituximab with or without chemotherapy is the standard treatment approach.

• #1 Castleman Disease | MDedge

  https://www.mdedge.com/content/castleman-disease

  Treatment with HAART does not decrease the risk of relapse in HIV MCD; therefore, the role of rituximab and antiherpesvirus agents as maintenance therapy has been explored. […] The cytotoxic chemotherapy with single agents, such as etoposide, vinblastine, cyclophosphamide, cladribine, chlorambucil, and liposomal doxorubicin, has been used with limited success. The combination chemotherapy with cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) or cyclophosphamide/vincristine/prednisone (CVP) without rituximab has been shown to achieve durable remissions. […] For unresectable UCD or MCD with organ failure or relapse, the use of alternative single-agent or combination chemotherapies with or without rituximab is recommended. Thalidomide has shown some success, probably secondary to disruption of IL-6 production.

• #1 What are the treatment options for Castleman disease? – Carenity

  https://www.carenity.us/condition-information/magazine/news/what-are-the-treatments-for-castleman-disease-1168

  Steroids are to be avoided due to the risk of apparition or aggravation of Kaposi disease, even though they are capable of improving the situation for several days. […] In case of treatment failure, other chemotherapy drugs can be suggested, possibly associated with rituximab in the absence of progressive Kaposi disease. […] The treatment is based on the same scheme, but the need to control viral replication and the severity of the immune deficiency in patients affected with HIV must be taken into account. Etoposide is the standard treatment in case of emergency or initiation.

• #1 Castleman Disease: Types, Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/hiv-aids/castleman-disease

  Corticosteroids can reduce inflammation. Prednisone, which is taken as a pill, is often used for Castleman disease. […] Even though Castleman disease isn’t cancer, chemotherapy can sometimes help treat MCD. Some chemo drugs are injected into your vein, but others you can take by mouth. Sometimes, you’ll need to take a mix of drugs. […] Once you’ve had treatment, it’s important to have regular checkups. Your doctor can keep tabs on your health and watch for signs that the disease might be coming back.

• #1 What are the treatment options for Castleman disease? – Carenity

  https://www.carenity.us/condition-information/magazine/news/what-are-the-treatments-for-castleman-disease-1168

  No matter what treatment plan has been adopted, post treatment monitoring is always carried out via blood tests and medical imaging. […] As this form of Castleman disease affects multiple lymph nodes, surgery is not suitable, and it is all the more difficult to find appropriate treatment. […] Various other therapeutic approaches are possible. Depending on the target for the adopted therapeutic approach, the aim will be to reduce the lymph node mass, treat the symptoms and try to control the disease as much as possible. […] Steroids are also indicated for controlling inflammatory syndrome, in case of associated complication or in combination with another treatment. The usual dose is 1mg/kg, but higher doses are possible. […] Kaposi sarcoma is a tumor combined with human herpes virus type 8 infection (HHV8).

• #1 Treatment Guidelines for Idiopathic Multicentric Castleman Diseaselogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na47480/2018/09/12/treatment-guidelines-idiopathic-multicentric-castleman

  Responses usually occur after 3 or 4 doses of anti-IL-6 mAb, but median time to resolution of lymphadenopathy is 5 months; IL-6 levels remain spuriously elevated for 18 to 24 months. […] Continue anti-IL-6 mAb therapy indefinitely to avoid relapse. […] These new treatment guidelines for iMCD are based on disease severity. The anti-IL-6 mAb, siltuximab, or a short course of rituximab is suggested for patients with mild to moderately severe disease. For those with more severe disease, high-dose steroids and immunomodulating agents are added to anti-IL-6 mAb therapy, and consider cytotoxic chemotherapy to control cytokine storm. A limitation of the guidelines is that they have been formulated from case-series and clinical experience because randomized, controlled trials directly comparing the available agents are unavailable.

• #1 Get Castleman Disease Treatment | Cleveland Clinic

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

  If you have UCD, you’ll have surgery to remove any affected lymph nodes. Depending on their size and location, you may have immunotherapy or radiation therapy before surgery. […] MCD is harder to treat because it’s widespread throughout your body. We don’t usually recommend radiation or surgery. We plan treatment based on how severe your MCD is and if it’s connected to HHV-8. We may recommend corticosteroids to relieve symptoms and reduce inflammation, chemotherapy to slow cell overgrowth in your lymph nodes, immunotherapy: Siltuximab (Sylvant) to block proteins that fuel abnormal cell growth, and antiviral drugs to treat HIV or HHV-8 infections. […] 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.

• #1 A 50-year-old patient battling life-threatening Castleman disease was provided adalimumab as a treatment of last resort following its identification by AI-guided discovery. Every Cure and its partners are expanding upon this discovery to harness a novel

  https://everycure.org/first-life-saved-with-ai-discovered-repurposed-medicine-for-castleman-disease-with-significant-potential-applications-to-treat-other-illnesses/

  A 50-year-old patient battling life-threatening Castleman disease was provided adalimumab as a treatment of last resort following its identification by AI-guided discovery. […] A patient’s life was saved after researchers at the University of Pennsylvania, Castleman Disease Collaborative Network, Medidata, a Dassault Systèmes company, and Every Cure, led by Dr. David Fajgenbaum, utilized AI to uncover a previously unknown use for an existing drug, adalimumab, to treat idiopathic multicentric Castleman disease (iMCD). […] The only FDA-approved treatment works in a portion of those diagnosed with iMCD, leaving the majority of patients with limited options. […] Alan, a 50-year-old iMCD patient who exhausted all known treatments, was prescribed adalimumab by Dr. Luke Chen from the University of British Columbia after consultation with Dr. Fajgenbaum and his team from Every Cure, who had identified the potential benefit of this medication using an AI-guided proteomics approach.

• #1 A 50-year-old patient battling life-threatening Castleman disease was provided adalimumab as a treatment of last resort following its identification by AI-guided discovery. Every Cure and its partners are expanding upon this discovery to harness a novel

  https://everycure.org/first-life-saved-with-ai-discovered-repurposed-medicine-for-castleman-disease-with-significant-potential-applications-to-treat-other-illnesses/

  Within a few days of taking adalimumab, his organs regained function, his symptoms subsided, and he went into remission. The use of adalimumab to treat iMCD would be a novel application. […] Treating iMCD with adalimumab is not a currently approved use. With the groundbreaking discovery, Every Cure and its research partners are beginning to lay the groundwork for conducting clinical trials to further validate its use for iMCD. […] Every Cure is currently analyzing the top results from its initial findings in its Every Cure LinkMap, in addition to consulting industry experts, to identify the most promising candidates for repurposing, including opportunities such as the use of metreleptin for anorexia, folinic acid for autism spectrum disorder, anakinra for sepsis, and bosutinib for ALS.

• #1 Update and new approaches in the treatment of Castleman disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4976903/

  Complete surgical resection is curative for UCD, leading to excellent long-term outcomes with 10-year overall survival rates in excess of 95%. […] A range of systemic therapies have been utilized in MCD, including cytotoxic chemotherapy, antibodies directed against CD20 as well as IL-6 and its receptor, immunomodulators, bortezomib, and antiviral agents. […] Rituximab, a humanized monoclonal antibody to CD20 (which is typically found on CD plasmablasts), has shown significant activity in HIV-positive and idiopathic MCD patients, when used either as monotherapy or in combination with chemotherapy. […] The advent of monoclonal antibody therapy targeting the IL-6 pathway has significantly impacted the treatment of patients with idiopathic MCD. Agents that have been studied include siltuximab, a chimeric monoclonal antibody to IL-6, and tocilizumab, a recombinant humanized monoclonal antibody that blocks the IL-6 receptor. […] Siltuximab has regulatory approval in the USA and Europe for the treatment of HIV-negative and HHV-8-negative MCD, while tocilizumab has approval for use in Japan.

• #1

  https://haematologica.org/article/view/5799

  Highly active antiretroviral therapy alone may be an effective treatment for HIV-associated multi-centric Castlemans disease. […] There is no standard treatment. Interventions include rituximab, lymphoma-type treatment with chemotherapy and splenectomy. […] Our findings suggest HAART alone may represent an effective treatment for MCD patients, avoiding unnecessary splenectomy or chemotherapy with attendant toxicity and impeding immune reconstitution/CD4 recovery. […] These 4 patients demonstrate that HAART may be an effective treatment for HIV-associated MCD. Currently there is a lack of data to offer clear guidance to clinicians on treatment strategy. Multi-center clinical trials are needed to examine the role of HAART for first-line treatment of MCD.

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

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

  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. […] 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. Antiviral therapy comprising of ganciclovir, foscarnet, and cidofovir is proven effective against HHV-8 replication pathways. Novel immunomodulatory agents like rituximab, thalidomide, bortezomib, IL-1 antagonist anakinra, and interferon-alpha have somehow shown promising results as well with minimum side effects. […] Chemotherapy and immunomodulatory drugs are best reserved for the relapse scenarios. However, it should be kept in mind that not all CD cases respond to therapy and get cured.

• #1 Successful Treatment of Idiopathic Multicentric Castleman Disease With Rash as the Initial Symptom Using a Rituximab-Based Regimen | Zhu | Journal of Hematology

  https://www.thejh.org/index.php/jh/article/view/1313/880

  For iMCD patients, regardless of disease severity, the only US Food and Drug Administration (FDA)-approved treatment tested in a randomized trial for iMCD is siltuximab, an IL-6 antagonist. […] In our previous research, we have innovatively applied RVD regimen (rituximab, bortezomib and dexamethasone) in iMCD patients and achieved satisfying response and great tolerability.

• #2 Update and new approaches in the treatment of Castleman disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4976903/

  First described 60 years ago, Castleman disease comprises a rare and heterogeneous cluster of disorders, characterized by lymphadenopathy with unique histological features and associated with cytokine-driven constitutional symptoms and biochemical disturbances. […] Although unicentric Castleman disease is curable with complete surgical excision, its multicentric counterpart is a considerable therapeutic challenge. The recent development of biological agents, particularly monoclonal antibodies to interleukin-6 and its receptor, allow for more targeted disease-specific intervention that promises improved response rates and more durable disease control; however, further work is required to fill knowledge gaps in terms of underlying pathophysiology and to facilitate alternative treatment options for refractory cases.

• #2 Castleman disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/diagnosis-treatment/drc-20543055

  Treatment depends on the type of Castleman disease you have. […] Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease (UCD). If the lymph node is in the chest or abdomen, major surgery may be needed. […] Surgery to remove the enlarged lymph node usually cures UCD. However, UCD sometimes comes back. If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. If medicines don’t work, radiation therapy may be an option. […] Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. Rituximab is highly effective, but sometimes medicines called antivirals and chemotherapies are needed. Antiviral medicines can block the activity of HHV-8 or HIV, and chemotherapies can get rid of extra immune cells.

• #2 Castleman disease // Middlesex Health

  https://middlesexhealth.org/6D45E88A-F751-989D-073B9A57D2251060

  Treatment and outlook vary depending on the type of Castleman disease you have. Unicentric Castleman disease, which is the type that involves only one enlarged lymph node, can usually be successfully treated with surgery. […] The best treatment for oligocentric Castleman disease, which involves a few enlarged lymph nodes and has limited symptoms, is not known but is thought to be similar to the treatment for unicentric Castleman disease. […] While not all people with MCD respond to the first treatment, there are medicines that work to treat HHV-8-associated MCD and idiopathic MCD. […] Surgery to remove the affected lymph node is the usual treatment for unicentric Castleman disease (UCD). If the lymph node is in the chest or abdomen, major surgery may be needed. […] Surgery to remove the enlarged lymph node usually cures UCD. However, UCD sometimes comes back. If surgery is not possible, you may need medicines typically used for multicentric Castleman disease. If medicines don’t work, radiation therapy may be an option.

• #2 Update and new approaches in the treatment of Castleman disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4976903/

  Complete surgical resection is curative for UCD, leading to excellent long-term outcomes with 10-year overall survival rates in excess of 95%. […] A range of systemic therapies have been utilized in MCD, including cytotoxic chemotherapy, antibodies directed against CD20 as well as IL-6 and its receptor, immunomodulators, bortezomib, and antiviral agents. […] Rituximab, a humanized monoclonal antibody to CD20 (which is typically found on CD plasmablasts), has shown significant activity in HIV-positive and idiopathic MCD patients, when used either as monotherapy or in combination with chemotherapy. […] The advent of monoclonal antibody therapy targeting the IL-6 pathway has significantly impacted the treatment of patients with idiopathic MCD. Agents that have been studied include siltuximab, a chimeric monoclonal antibody to IL-6, and tocilizumab, a recombinant humanized monoclonal antibody that blocks the IL-6 receptor. […] Siltuximab has regulatory approval in the USA and Europe for the treatment of HIV-negative and HHV-8-negative MCD, while tocilizumab has approval for use in Japan.

• #2 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. […] 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.

• #2 Treatment – CDCN

  https://cdcn.org/treatment/

  People with unicentric Castleman disease usually do well once the affected lymph node is removed and life expectancy is usually not changed. […] However, people with unicentric Castleman disease are at increased risk of developing paraneoplastic pemphigus (PNP), which is a rare autoimmune condition that can be deadly. […] There are no reported cases of UCD transforming into MCD. […] HHV-8 associated multicentric Castleman disease (HHV-8+MCD): Rituximab is recommended as first-line therapy for HHV-8+ MCD. […] First-line treatment for idiopathic multicentric Castleman disease is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] Siltuximab (Sylvant) is the only FDA-approved treatment for iMCD and patients who responded to siltuximab tend to have long-term responses.

• #2 Castleman disease – Wikipedia

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

  First-line treatment of HHV-8-associated MCD is rituximab, a drug used to eliminate a type of immune cell called the B lymphocyte. […] First line treatment for iMCD is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] In critically ill patients, chemotherapy and corticosteroids are recommended if the patient is demonstrating disease progression while on siltuximab. […] In patients where siltuximab is not effective, other treatments such as rituximab and sirolimus can be used.

• #2 Castleman disease // Middlesex Health

  https://middlesexhealth.org/6D45E88A-F751-989D-073B9A57D2251060

  More research into the treatment of oligocentric Castleman disease is needed, but treatment is usually similar to that of UCD. […] Rituximab (Rituxan) is usually the first treatment for HHV-8-positive MCD. Rituximab is highly effective, but sometimes medicines called antivirals and chemotherapies are needed. Antiviral medicines can block the activity of HHV-8 or HIV, and chemotherapies can get rid of extra immune cells. […] Siltuximab (Sylvant) is usually the first treatment for idiopathic MCD. In the U.S., siltuximab is the only medicine approved by the Food and Drug Administration (FDA) for the treatment of idiopathic MCD. People who get better after taking siltuximab tend to have successful long-term treatment. This drug blocks the action of a protein called interleukin-6. The bodies of people who have idiopathic MCD produce too much of this protein.

• #2 Castleman disease: Types, symptoms, treatment, and more

  https://www.medicalnewstoday.com/articles/castleman-disease

  Treatment is very effective for the unicentric type. However, researchers have not established optimal treatments for the multicentric type, so it has a less favorable outlook. […] The first-line treatment involves surgically removing the affected lymph nodes. Although this usually results in a cure, health experts have reported that the condition may recur in some cases. […] If symptoms stem from an inflammatory syndrome, the treatment may entail anti-interleukin-6 therapy with siltuximab (Sylvant), which targets a protein that plays a role in inflammation. […] The treatment of choice for idiopathic multicentric is siltuximab (Sylvant), which is effective in 34-50% of people. […] First-line treatment is rituximab (Rixathon). It is very effective, but sometimes cytotoxic chemotherapy, antiviral agents, or both are necessary. […] Treatment is very effective for the unicentric type but much less so for the multicentric form of the condition. Consequently, unicentric is often curable, and the outlook for multicentric is less favorable.

• #2 Castleman disease // Middlesex Health

  https://middlesexhealth.org/6D45E88A-F751-989D-073B9A57D2251060

  People who are critically ill with idiopathic MCD often receive treatment with medicines called corticosteroids. They also may need chemotherapy. Corticosteroids such as prednisone can help control inflammation. Chemotherapy can get rid of immune cells that are causing problems. […] When siltuximab doesn’t work, other treatments such as rituximab (Rituxan) and sirolimus (Rapamune) may be used.

• #2 Treatment – CDCN

  https://cdcn.org/treatment/

  Anti-IL-6 therapy is effective in about 34-50% of patients, but some patients do not respond to it. […] 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. […] 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. […] A clinical trial for the medication sirolimus for iMCD patients who did not improve on siltuximab or tocilizumab was recently conducted.

• #2 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

  UCD usually presents as solitary lymphoid tissue overgrowth or lymphadenopathy that is curable with surgical extirpation. […] In case of an unresectable mass or a dangerous localization, remission can be achieved or the size can be reduced with administration of corticosteroids and/or rituximab, and a delayed surgical intervention may be offered afterwards. […] Treatment with rituximab is effective because it depletes the viral reservoir and reduces the risk of lymphoma. […] According to novel recommendations of CDCN, therapy for iMCD should be preceded by stratification to a non-severe or severe clinical manifestation. […] Therapy for non-severe iMCD should target IL-6. Siltuximab, a monoclonal antibody to IL-6, is a recommended front-line option with a 34% response rate demonstrated in clinical trials and already approved by the US Food and Drug Administration and European Medicines Agency.

• #2 Castleman Disease: Types, Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/hiv-aids/castleman-disease

  Corticosteroids can reduce inflammation. Prednisone, which is taken as a pill, is often used for Castleman disease. […] Even though Castleman disease isn’t cancer, chemotherapy can sometimes help treat MCD. Some chemo drugs are injected into your vein, but others you can take by mouth. Sometimes, you’ll need to take a mix of drugs. […] Once you’ve had treatment, it’s important to have regular checkups. Your doctor can keep tabs on your health and watch for signs that the disease might be coming back.

• #2 Castleman Disease: Types, Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/hiv-aids/castleman-disease

  Your treatment will depend on many things, including the type of Castleman disease you have. Talk to your doctor about a plan that’s right for you. […] If you have the unicentric type, you may need surgery to remove your swollen lymph node. When the node is in an easy-to-reach place, like your armpit, the procedure is simple. You can often go home the same day. […] If you have MCD, you’ll need treatment that works throughout your body, because the disease has spread to many lymph nodes. […] Your doctor might give you drugs that help stop the inflammation in cells that cause Castleman disease. They might prescribe immunotherapy medicines like: Siltuximab (Sylvant) and Tocilizumab (Actemra). […] Lenalidomide (Revlimid) or thalidomide (Thalomid) can also help lower inflammation and make you feel better.

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

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

  Regularly prescribed medications like glucocorticoids (often called steroids), like prednisone. This will help control inflammation in your child’s body. […] Chemotherapy describes powerful medicines that can help slow the overgrowth of cells in your child’s lymph nodes. This may be needed if your child’s symptoms do not respond to immunotherapy. […] If your child has HHV-8-associated MCD, antiviral drugs can be used to block the activity of one or both viruses.

• #2 Diagnosis – Castleman Disease Collaborative Network | CDCN

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

  There are various types of Castleman disease and they have very important differences in symptoms, disease course, and treatment. […] A clinical trial for the medication sirolimus for iMCD patients who did not improve on siltuximab or tocilizumab is open and enrolling patients. […] Patients can learn more about this trial at cdcn.org/trial.

• #2 Using AI to Identify Treatment Options for Castleman Disease – The ASCO Post

  https://ascopost.com/news/february-2025/using-ai-to-identify-treatment-options-for-castleman-disease

  They suggested that the patient involved in the study could be one of the first to receive life-saving treatment with the help of an AI prediction system, which could potentially be applied to other rare conditions. […] They plan to launch a clinical trial in 2025 to explore the effectiveness of another repurposed drug – a Janus kinase 1/2 inhibitor – in patients with idiopathic multicentric Castleman disease. […] More research is needed, but I’m hopeful that many of them could benefit from this new treatment, he concluded.

• #2 Treatment and outcome of Castleman Disease | TCRM

  https://www.dovepress.com/treatment-and-outcome-of-castleman-disease-a-retrospective-report-of-3-peer-reviewed-fulltext-article-TCRM

  Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with a spectrum of characteristic pathological abnormalities of lymph node. […] This study aimed to investigate the clinical presentations, treatment and prognosis of CD, thereby improving the understanding and diagnosis of CD. […] Patients with UCD were mainly treated with surgical resection alone, with a five-year survival rate of 95.65%. When siltuximab is not an option, steroid plus rituximab-based chemotherapy and specific supportive care are common options for MCD. […] In the absence of IL-6 antagonists, anti-inflammatory and immunosuppressive therapeutic strategies, and cytotoxic clearance of cells responsible for hypercytokinemia could be adopted. […] Treatment of CD included surgery, siltuximab, rituximab, steroids, immunosuppressive agents, radiotherapy and chemotherapy.

• #2 Successful treatment of multicentric Castleman’s disease with combined immunochemotherapy in an AIDS patient with multiorgan failure | Leukemia

  https://www.nature.com/articles/leu200854

  Here we describe the complicated course of a 33-year-old African male with AIDS, with multiorgan failure due to multicentric Castleman’s disease (MCD). […] Use of rituximab as a salvage therapy for HIV-associated multicentric Castleman disease. […] Remission of HHV-8 and HIV-associated multicentric Castleman disease with ganciclovir treatment.

• #3 Castleman disease – Wikipedia

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

  Castleman disease includes at least three distinct subtypes: unicentric Castleman disease (UCD), human herpesvirus 8 associated multicentric Castleman disease (HHV-8-associated MCD), and idiopathic multicentric Castleman disease (iMCD). […] Correctly classifying the Castleman disease subtype is important, as the three subtypes vary significantly in symptoms, clinical findings, disease mechanism, treatment approach, and prognosis. […] Surgery is considered by experts to be the first-line treatment option for all cases of UCD. […] 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, then radiation may be needed.

• #3 Castleman disease – Wikipedia

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

  First-line treatment of HHV-8-associated MCD is rituximab, a drug used to eliminate a type of immune cell called the B lymphocyte. […] First line treatment for iMCD is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] In critically ill patients, chemotherapy and corticosteroids are recommended if the patient is demonstrating disease progression while on siltuximab. […] In patients where siltuximab is not effective, other treatments such as rituximab and sirolimus can be used.

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