Castleman Disease Treatment

What is Castleman Disease?

Castleman disease (CD) is a very rare disease that is characterized by the proliferation of lymphoid tissue. It is defined by the proliferation of tissues in the lymph node, which revolves around the disease’s immune system. There are two main types of Castleman disease:

1. Unicentric Castleman Disease (UCD):

• It is localized in one single lymph node or several lymph nodes in a group.
• Smaller percentage and generally termed as less aggressive in nature.
• Can frequently require surgical removal of the nodes affected.

2. Multicentric Castleman Disease (MCD):

• May affect several lymph nodes and other organs including the liver, spleen, and bone marrow.
• May be more severe and cause such symptoms as fever, fatigue, weight loss, and that affects different organs and systems of the human body.
• Compared with non-smokers, former smokers’ risks of infections and some forms of cancer (including lymphoma) are heightened.
• Management may include drugs such as immunosuppressants, or chemotherapy.

Castleman Disease Virus

Castleman disease may be classified following its relation to human herpes virus 8 (HHV-8), though it may be detected in some instances and is common in patients with HIV. Therapy is most often condition specific, and may vary according to the type, degree of intensity, or existence of other, preponderant disorders.

Need for Castleman Disease Treatment

Castleman disease (CD) requires treatment to address several key factors:

• Preventing Disease Progression
• Controlling Symptoms
• Reducing Risk of Complications
• Improving Prognosis and Long-Term Survival
• Improving Quality of Life

Castleman Disease Symptoms

The symtoms of Castleman disease (CD) depends with the type, whether is unicentric or multicentric and the organs involved. Below are the typical symptoms associated with each type:

• Enlarged Lymph Nodes
• Pressure or Pain
• Cough or Difficulty Breathing
• Fever
• Fatigue
• Weight Loss
• Night Sweats
• Anemia
• Swelling
• Liver and Spleen Enlargement
• Abdominal Pain or Discomfort
• Shortness of Breath
• Kidney Involvement
• Skin Changes

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Risk Factors of Castleman Disease Treatment

The available treatment of Castleman disease (CD) can be administered through different approaches and this treatment can be very effective as they have their pros but also cons as they are associated with some severe side effects. The risk associated with treatment may also be influenced by the type of Castleman disease whether it is unicentric or multicentric, the treatment offered and the general health of the patient. Below are the main risk factors associated with treatments for Castleman disease:

• Infection
• Bleeding
• Damage to Surrounding Tissues
• Nausea and Vomiting
• Hair Loss
• Fatigue
• Blood Cell Count Drop

Causes of Castleman Disease

The factors which can lead to Castleman disease depend on the classification of the particular disease as unicentric or multicentric; in most cases Castleman disease seems to be closely associated with immune system failure, viral infections, and heredity factors. Here’s a detailed breakdown of the known and potential causes:

• Immune System Dysregulation
• Viral Infections
• Genetic Factors
• Autoimmune and Inflammatory Diseases
• Chronic Inflammation
• Environmental Factors

Castleman Disease Pathology

Unicentric Castleman Disease Pathology

It usually causes a single, enlarged lymph node and pathologic changes are seen only in that lymph node. They have studied that the disease is not systemic and does not spread around the entire body. The pathology of UCD is marked by the following features:

• Hypertrophy of Follicular Structures
• Hypervascularity
• Atrophic Medulla
• Absence of Systemic Involvement

Multicentric Castleman Disease Pathology

MCD is more complex and also typically has systems involvement presenting with features such as fever, weight reduction, fatigue and organ involvement. MCD pathology presents more diffuse and severe findings in multiple LN, and more commonly associate with more advanced immune dysfunction.

• Plasma cell and B-cell Hyperplasia
• Paracorticol Expansion
• Vascular Proliferation and Inflammation
• Lymphoid Follicles and Sclerotic Areas
• Systemic Involvement
• HIV and HHV-8 Infection

Castleman Disease Radiology

Imaging studies aid in the diagnostic work-up, disease staging, and evaluation for response to therapy in Castleman disease (CD). Traditionally utilized to map the area of involvement, measure the size and position of the enlarged lymph nodes and demarcate CD from other conditions with resembling symptoms. Here’s an overview of how radiology is used in the evaluation of Castleman disease:

• CT Scan
• MRI
• PET Scan
• Ultrasound

Castleman Disease Treatment

The method of managing Castleman disease (CD) varies depending on the type and stage of the disease as well as the other diseases that are associated with the disease, for instance HIV or autoimmune diseases. The aim of the treatment is to deal with symptoms and slow the progress of lymphoid tissue growths and reduce risk of complications. Here's a breakdown of the common treatment options:

Unicentric Castleman Disease

Surgical Removal of Affected Lymph Node(s):

• Primary Treatment: The same surgical excision of the affected regional lymph node is the most typical and sometime enough for the treatment of unicentric Castleman disease. This procedure may be performed under general anaesthesia and its success may require the use of steroids.
• Outcome: Surgery is nearly always curative and the vast majority of patients never require any additional therapy after the node is excised. It is believed after surgery that there are minimal chances of the disease reoccurring.
• Risks: As with any surgery, there are some possibilities of infection afterwards, occasionally some bleeding, but there is a chance of harming other tissues and organs depending on the location of the lymph nodes.

Radiation Therapy (for Recurrence or Non-Surgical Cases):

However, if surgery cannot be performed or if lymph nodes are still affected in the course of the disease, radiation therapy can be used to destroy the rest of the affected area.

• Side Effects: Possible side effects are: exhaustion, dermatitis, and higher propensity of other types of cancer in the future.

Multicentric Castleman Disease

Multicentric Castleman disease is more severe and frequently calls for systematic treatment. The treatment options include:

Monoclonal Antibodies:

• Siltuximab (Sylvant): MCD is an illness that affects the kidneys and cause inflammation, siltuximab is a monoclonal antibody that works by inhibiting interleukin-6 (IL-6), which is a cytokine. It controls the interleukin-6 which leads to constraining the growth of lymphoid tissue and strengthens the response of the body against the symptoms such as fever, fatigue, weight loss of MCD.
• Tocilizumab (Actemra): Another monoclonal antibody that targeting with IL-6. Tocilizumab is utilised for MCD if siltuximab cannot be used or is unavailable for use. Besides, it has a capacity to decrease inflammation processes as well as ease the manifestations of the disease.
• Effectiveness: Those reports have indicated that both of them were effective when used for the treatment of MCD leading to a decreased size of the affected lymph node and CSM (fever and fatigue).
• Side Effects: They include fever which is often low grade, headache, rash and some gastrointestinal symptoms. Other concerns that are effects of immune suppression include infections.

Chemotherapy (for Severe or Refractory MCD):

Chemotherapy is recommended if MCD is progressive, rapidly advancing, refractory to treatment with anti-monoclonal antibodies. In particular, other medications used in the treatment of MCD are rituximab, an anti-B cell agent, and cyclophosphamide.

• Effectiveness: Chemotherapy assists in the management of the disease by decreasing the amount of irregular immune cells in circulation.
• Side Effects: Side effects of chemotherapy include: vomiting, nausea, fatigue and hair loss; immunosuppression, that leads to an increased risk of infections; anemia, leukopenia, thrombocytopenia.

Corticosteroids:

Prednisone or other corticosteroids may be taken to lessen inflammation and the severity of the symptoms of MCD, if there are any episodes.

• Effectiveness: Steroids may decrease fever and swelling, but should not be taken for an extended period of time because of the possible consequences.
• Side Effects: It also has risks and bad side effects such as increasing steroid use and may lead to weight gain and fluid retention and may cause bone to become thinner or osteoporosis, high blood pressure and more risks to get infected easily.

Antiviral Treatment (for HHV-8-associated MCD):

If for one reason or another the MCD is related to the human herpesvirus- 8, antiviral drugs including ganciclovir or valganciclovir could be used to control the virus. This is well illustrated in HIV/AIDS patients especially those with HHV-8 co infection.

• Effectiveness: When used in combo with other interventions such as monoclonal antibodies, antivirals can help to decrease viral quantities and outcomes such as headache.
• Side Effects: Common side effects of antivirals include vomiting and rash while there are serious side effects such as liver toxicity which is why there is need for frequent monitoring.

Stem Cell Transplantation (in Severe Cases of MCD):

However, in rare conditions provoking severe or non-responsive MCD (the condition that does not react to other therapies) stem cell transplantation (hematopoietic stem cell transplantation) is possible.

• Effectiveness: It is scram treatment which is the process of replacing the new stem cells for the diseased ones, as a way of restoring the functions of a bad Immune system.

• Risks: Stem cell transplants are very complicated procedures and are associated with numerous hazards such as the graft versus host disease, infection, and organ toxicity arising from immune suppression treatments.

Best Hospitals for Castleman Disease Treatment

• Artemis Hospital, Gurgaon
• Medanta-The Medicity, Gurgaon
• Fortis Memorial Research Institute, Gurgaon
• Max Hospital, Saket
• BLK-Max Super Speciality Hospital, New Delhi

Best Doctors for Castleman Disease Treatment

• Dr. Gaurav Dixit
• Dr. Ashok Kumar Vaid
• Dr. Rahul Bhargava
• Dr. Harit Chaturvedi
• Dr. Dharma Choudhary

Why Choose GetWellGo for Castleman Disease Treatment?

GetWellGo might be the best choice for Castleman Disease for reasons because of the following reasons: If they offer specialized treatment for rare diseases or have a team that focuses mainly on complicated illnesses like Castleman disease or offer extension services which cover all aspects as a patient may require. Here are some reasons why one might choose GetWellGo for Castleman Disease treatment:

• Medical Expertise
• Advanced Diagnostics and Imaging
• Visa Assistance
• Language Support
• Travel and Accommodation Arrangements

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