Castleman disease, also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia, is a rare disorder that affects the lymph nodes and related tissues. It was first described by Dr. Benjamin Castleman in 1954. Castleman disease is characterized by the abnormal growth of lymphoid tissue, which can cause the enlargement of one or more lymph nodes in the body.
The disease can be classified into two main types: unicentric Castleman disease (UCD), which affects a single lymph node or a group of lymph nodes in one region of the body, and multicentric Castleman disease (MCD), which affects multiple lymph nodes in different regions of the body. The Castleman disease can occur in people of any age and gender, but it is more commonly diagnosed in adults.
The cause of Castleman disease is not fully understood, but it is thought to be related to an abnormal immune system response. Symptoms of Castleman disease may vary depending on the type and location of the disease and can include enlarged lymph nodes, fever, night sweats, fatigue, weight loss, and other symptoms. Treatment options may include surgery, radiation therapy, chemotherapy, or targeted therapy, depending on the type and severity of the disease.
Epidemiology
Castleman disease is a rare disorder, with an estimated incidence of 1 in 100,000 individuals per year. It can occur in people of any age and gender, but it is more commonly diagnosed in adults. Multicentric Castleman disease (MCD) accounts for approximately 10-20% of all cases of Castleman disease. MCD is more common in men than in women, with a male-to-female ratio of approximately 2:1.
It is also more common in individuals over the age of 50. Unicentric Castleman disease (UCD) is more common in women than in men and typically affects younger individuals, with a peak incidence in the third decade of life. The prevalence of Castleman disease may be underestimated due to its rarity and the need for more awareness among healthcare providers. Further research is needed to better understand the epidemiology and risk factors for Castleman disease.
Anatomy
Pathophysiology
The pathophysiology of Castleman disease is not completely understood, but it is believed to involve an abnormal immune system response, particularly involving the production of cytokines. Cytokines are signaling molecules that are produced by immune cells, and they play a role in regulating the immune response. In Castleman disease, abnormal cytokine production leads to the growth of lymphoid tissue, which can cause the enlargement of one or more lymph nodes in the body.
In unicentric Castleman disease (UCD), the abnormal growth of lymphoid tissue occurs in a single lymph node or a group of lymph nodes in one region of the body. UCD is typically a localized and benign condition that may be cured with surgical removal of the affected lymph node. In multicentric Castleman disease (MCD), the abnormal growth of lymphoid tissue occurs in multiple lymph nodes in different regions of the body and may also involve other tissues and organs, such as the liver, spleen, and bone marrow.
MCD is characterized by systemic symptoms such as fever, night sweats, fatigue, and weight loss, which are thought to be related to the production of abnormal cytokines. MCD can be further divided into two subtypes: HHV-8-associated MCD, which is caused by the human herpesvirus 8 (HHV-8) and is more common in people with HIV/AIDS or other immunocompromised conditions, and idiopathic MCD, which has no known cause.
The abnormal cytokine production in Castleman disease is thought to be related to the activation of a type of immune cell called the plasma cell. Abnormal plasma cell activation may be triggered by various factors, including infections, autoimmune disorders, or other underlying medical conditions. Further research is needed to better understand the pathophysiology of Castleman’s disease and to develop more effective treatments.
Etiology
The exact cause of Castleman disease is not known, but it is believed to be related to an abnormal immune system response. In some cases, Castleman disease is associated with infections, such as the human herpesvirus 8 (HHV-8), which is known to cause multicentric Castleman disease (MCD) in people with HIV/AIDS or other immunocompromised conditions. HHV-8-associated MCD is more common in people with HIV/AIDS but can also occur in people who are not infected with HIV.
Other underlying medical conditions or factors may also contribute to the development of Castleman disease, including autoimmune disorders, such as rheumatoid arthritis or lupus, or other types of cancer, such as lymphoma. In some cases, Castleman disease may be idiopathic, meaning that there is no known cause. The Castleman disease can be classified into two main types based on the location and pattern of lymph node involvement.
Unicentric Castleman disease (UCD) involves a single lymph node or a group of lymph nodes in one region of the body, while multicentric Castleman disease (MCD) involves multiple lymph nodes in different regions of the body. MCD is more commonly associated with infections or underlying medical conditions, while UCD is often idiopathic. Further research is needed to better understand the underlying causes of Castleman disease and to develop more effective treatments for this rare disorder.
Genetics
Prognostic Factors
The prognosis of Castleman disease depends on several factors, such as the subtype and extent of the disease, the presence of underlying medical conditions or complications, and the response to treatment. Unicentric Castleman disease (UCD) has an excellent prognosis, and most patients can be cured with surgical removal of the affected lymph node or tissue. The recurrence rate is low, and long-term survival is expected. Multicentric Castleman disease (MCD), on the other hand, has a more variable prognosis and may be associated with significant morbidity and mortality.
The overall survival of MCD varies depending on the subtype of the disease and the presence of coexisting medical conditions. The human herpesvirus 8 (HHV-8)-associated MCD subtype has a better prognosis compared to the idiopathic or HIV-associated subtypes. The use of targeted therapies, such as anti-IL-6 antibodies, has improved the prognosis of MCD, particularly in patients who do not respond to standard therapies.
In some cases, MCD may be associated with the development of other types of cancer, such as lymphoma, and may require more aggressive treatment, such as high-dose chemotherapy with stem cell transplantation. Close monitoring and follow-up are essential to detect any potential complications or recurrence of the disease. Overall, the prognosis of Castleman disease can vary widely depending on the subtype and individual patient factors, and close collaboration between the patient, medical team, and other healthcare professionals is critical to achieving the best possible outcome.
Clinical History
Clinical History
The clinical history of Castleman disease may vary depending on the subtype and location of the disease. Unicentric Castleman disease (UCD) is usually asymptomatic or may cause mild symptoms, such as swelling or tenderness of the affected lymph node or tissue. In some cases, UCD may be discovered incidentally during imaging studies or surgical procedures. Multicentric Castleman disease (MCD) is usually more symptomatic and may cause fever, fatigue, night sweats, weight loss, and generalized lymphadenopathy (enlarged lymph nodes throughout the body).
MCD may also involve other tissues and organs, such as the liver, spleen, or lungs, and can cause a variety of symptoms depending on the location of the involvement. MCD may be associated with an increased risk of infections, such as pneumonia or sepsis, and may also increase the risk of other types of cancer, such as lymphoma. In some cases, MCD may be associated with a specific infection, such as the human herpesvirus 8 (HHV-8), which is known to cause HHV-8-associated MCD in people with HIV/AIDS or other immunocompromised conditions.
HHV-8-associated MCD may be more severe and progressive than other subtypes of MCD and may require more aggressive treatment. Since the symptoms of Castleman disease can be nonspecific and overlap with other conditions, a thorough medical history, physical examination, and diagnostic tests are important to differentiate it from other possible conditions.
Physical Examination
Physical examination
The physical examination findings in Castleman disease may depend on the subtype and location of the disease. Unicentric Castleman disease (UCD) may present as a localized mass or swelling that is usually non-tender and non-mobile. In some cases, UCD may be discovered incidentally during imaging studies or surgical procedures.
Multicentric Castleman disease (MCD) is usually associated with generalized lymphadenopathy (enlarged lymph nodes throughout the body) and may also involve other tissues and organs, such as the liver, spleen, or lungs. MCD may present with enlarged lymph nodes in the neck, armpits, or groin, as well as abdominal pain, hepatosplenomegaly (enlarged liver and spleen), skin rash, or lung infiltrates. In some cases, MCD may be associated with systemic symptoms, such as fever, fatigue, night sweats, and weight loss.
The physical examination may also reveal signs of other underlying medical conditions or complications, such as infections, autoimmune disorders, or other types of cancer. Depending on the location and severity of the disease, additional diagnostic tests, such as imaging studies or a biopsy of the affected tissue, may be necessary to confirm the diagnosis and differentiate it from other possible conditions.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential diagnosis
The differential diagnosis of Castleman disease may depend on the location and subtype of the disease. Unicentric Castleman disease (UCD) is usually localized to a single lymph node or a group of lymph nodes in one region of the body, while multicentric Castleman disease (MCD) involves multiple lymph nodes in different regions of the body and may also involve other tissues and organs. Differential diagnoses of UCD may include other benign lymphoid or soft tissue tumors, such as lymphoma, reactive lymphadenopathy, or other types of benign tumors.
Differential diagnosis of MCD may include other lymphoproliferative disorders, such as lymphoma or leukemia, or other conditions that involve lymph node enlargements and systemic symptoms, such as infections (e.g., tuberculosis or HIV), autoimmune disorders (e.g., lupus or rheumatoid arthritis), or other types of cancer.
Since the symptoms of Castleman disease can vary depending on the subtype and location of the disease, it is important to conduct a thorough medical history, physical examination, and diagnostic tests to differentiate it from other possible conditions. Further testing may include blood tests to evaluate levels of cytokines and other markers, imaging studies (e.g., CT scan, MRI, or PET scan), and a biopsy of the affected tissue for pathological examination.
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
The treatment of Castleman disease depends on the subtype and extent of the disease, as well as the presence of underlying medical conditions or complications. Unicentric Castleman disease (UCD) is usually treated with surgical removal of the affected lymph node or tissue. In some cases, radiation therapy may be used as an alternative or adjunct to surgery. The prognosis for UCD is generally favorable, and most patients can be cured with local treatment.
Multicentric Castleman disease (MCD) is usually more difficult to treat and may require systemic therapy. The treatment of MCD depends on the subtype and severity of the disease, as well as the presence of underlying medical conditions or complications. In some cases, the use of anti-viral medications or immunosuppressive therapy may be effective in treating certain subtypes of MCD, such as human herpesvirus 8 (HHV-8)-associated MCD. In other cases, chemotherapy or targeted therapy may be used to reduce the size of the affected lymph nodes and improve symptoms.
Corticosteroids may also be used to reduce inflammation and improve symptoms, although they may have significant side effects with long-term use. In some cases, MCD may be associated with the development of other types of cancer, such as lymphoma, and may require more aggressive treatment, such as high-dose chemotherapy with stem cell transplantation. The management of Castleman disease should be individualized and based on the patient’s specific condition and response to treatment. Close monitoring and follow-up are important to assess the response to treatment and detect any potential complications or recurrence of the disease.
Castleman disease, also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia, is a rare disorder that affects the lymph nodes and related tissues. It was first described by Dr. Benjamin Castleman in 1954. Castleman disease is characterized by the abnormal growth of lymphoid tissue, which can cause the enlargement of one or more lymph nodes in the body.
The disease can be classified into two main types: unicentric Castleman disease (UCD), which affects a single lymph node or a group of lymph nodes in one region of the body, and multicentric Castleman disease (MCD), which affects multiple lymph nodes in different regions of the body. The Castleman disease can occur in people of any age and gender, but it is more commonly diagnosed in adults.
The cause of Castleman disease is not fully understood, but it is thought to be related to an abnormal immune system response. Symptoms of Castleman disease may vary depending on the type and location of the disease and can include enlarged lymph nodes, fever, night sweats, fatigue, weight loss, and other symptoms. Treatment options may include surgery, radiation therapy, chemotherapy, or targeted therapy, depending on the type and severity of the disease.
Castleman disease is a rare disorder, with an estimated incidence of 1 in 100,000 individuals per year. It can occur in people of any age and gender, but it is more commonly diagnosed in adults. Multicentric Castleman disease (MCD) accounts for approximately 10-20% of all cases of Castleman disease. MCD is more common in men than in women, with a male-to-female ratio of approximately 2:1.
It is also more common in individuals over the age of 50. Unicentric Castleman disease (UCD) is more common in women than in men and typically affects younger individuals, with a peak incidence in the third decade of life. The prevalence of Castleman disease may be underestimated due to its rarity and the need for more awareness among healthcare providers. Further research is needed to better understand the epidemiology and risk factors for Castleman disease.
The pathophysiology of Castleman disease is not completely understood, but it is believed to involve an abnormal immune system response, particularly involving the production of cytokines. Cytokines are signaling molecules that are produced by immune cells, and they play a role in regulating the immune response. In Castleman disease, abnormal cytokine production leads to the growth of lymphoid tissue, which can cause the enlargement of one or more lymph nodes in the body.
In unicentric Castleman disease (UCD), the abnormal growth of lymphoid tissue occurs in a single lymph node or a group of lymph nodes in one region of the body. UCD is typically a localized and benign condition that may be cured with surgical removal of the affected lymph node. In multicentric Castleman disease (MCD), the abnormal growth of lymphoid tissue occurs in multiple lymph nodes in different regions of the body and may also involve other tissues and organs, such as the liver, spleen, and bone marrow.
MCD is characterized by systemic symptoms such as fever, night sweats, fatigue, and weight loss, which are thought to be related to the production of abnormal cytokines. MCD can be further divided into two subtypes: HHV-8-associated MCD, which is caused by the human herpesvirus 8 (HHV-8) and is more common in people with HIV/AIDS or other immunocompromised conditions, and idiopathic MCD, which has no known cause.
The abnormal cytokine production in Castleman disease is thought to be related to the activation of a type of immune cell called the plasma cell. Abnormal plasma cell activation may be triggered by various factors, including infections, autoimmune disorders, or other underlying medical conditions. Further research is needed to better understand the pathophysiology of Castleman’s disease and to develop more effective treatments.
The exact cause of Castleman disease is not known, but it is believed to be related to an abnormal immune system response. In some cases, Castleman disease is associated with infections, such as the human herpesvirus 8 (HHV-8), which is known to cause multicentric Castleman disease (MCD) in people with HIV/AIDS or other immunocompromised conditions. HHV-8-associated MCD is more common in people with HIV/AIDS but can also occur in people who are not infected with HIV.
Other underlying medical conditions or factors may also contribute to the development of Castleman disease, including autoimmune disorders, such as rheumatoid arthritis or lupus, or other types of cancer, such as lymphoma. In some cases, Castleman disease may be idiopathic, meaning that there is no known cause. The Castleman disease can be classified into two main types based on the location and pattern of lymph node involvement.
Unicentric Castleman disease (UCD) involves a single lymph node or a group of lymph nodes in one region of the body, while multicentric Castleman disease (MCD) involves multiple lymph nodes in different regions of the body. MCD is more commonly associated with infections or underlying medical conditions, while UCD is often idiopathic. Further research is needed to better understand the underlying causes of Castleman disease and to develop more effective treatments for this rare disorder.
The prognosis of Castleman disease depends on several factors, such as the subtype and extent of the disease, the presence of underlying medical conditions or complications, and the response to treatment. Unicentric Castleman disease (UCD) has an excellent prognosis, and most patients can be cured with surgical removal of the affected lymph node or tissue. The recurrence rate is low, and long-term survival is expected. Multicentric Castleman disease (MCD), on the other hand, has a more variable prognosis and may be associated with significant morbidity and mortality.
The overall survival of MCD varies depending on the subtype of the disease and the presence of coexisting medical conditions. The human herpesvirus 8 (HHV-8)-associated MCD subtype has a better prognosis compared to the idiopathic or HIV-associated subtypes. The use of targeted therapies, such as anti-IL-6 antibodies, has improved the prognosis of MCD, particularly in patients who do not respond to standard therapies.
In some cases, MCD may be associated with the development of other types of cancer, such as lymphoma, and may require more aggressive treatment, such as high-dose chemotherapy with stem cell transplantation. Close monitoring and follow-up are essential to detect any potential complications or recurrence of the disease. Overall, the prognosis of Castleman disease can vary widely depending on the subtype and individual patient factors, and close collaboration between the patient, medical team, and other healthcare professionals is critical to achieving the best possible outcome.
Clinical History
The clinical history of Castleman disease may vary depending on the subtype and location of the disease. Unicentric Castleman disease (UCD) is usually asymptomatic or may cause mild symptoms, such as swelling or tenderness of the affected lymph node or tissue. In some cases, UCD may be discovered incidentally during imaging studies or surgical procedures. Multicentric Castleman disease (MCD) is usually more symptomatic and may cause fever, fatigue, night sweats, weight loss, and generalized lymphadenopathy (enlarged lymph nodes throughout the body).
MCD may also involve other tissues and organs, such as the liver, spleen, or lungs, and can cause a variety of symptoms depending on the location of the involvement. MCD may be associated with an increased risk of infections, such as pneumonia or sepsis, and may also increase the risk of other types of cancer, such as lymphoma. In some cases, MCD may be associated with a specific infection, such as the human herpesvirus 8 (HHV-8), which is known to cause HHV-8-associated MCD in people with HIV/AIDS or other immunocompromised conditions.
HHV-8-associated MCD may be more severe and progressive than other subtypes of MCD and may require more aggressive treatment. Since the symptoms of Castleman disease can be nonspecific and overlap with other conditions, a thorough medical history, physical examination, and diagnostic tests are important to differentiate it from other possible conditions.
Physical examination
The physical examination findings in Castleman disease may depend on the subtype and location of the disease. Unicentric Castleman disease (UCD) may present as a localized mass or swelling that is usually non-tender and non-mobile. In some cases, UCD may be discovered incidentally during imaging studies or surgical procedures.
Multicentric Castleman disease (MCD) is usually associated with generalized lymphadenopathy (enlarged lymph nodes throughout the body) and may also involve other tissues and organs, such as the liver, spleen, or lungs. MCD may present with enlarged lymph nodes in the neck, armpits, or groin, as well as abdominal pain, hepatosplenomegaly (enlarged liver and spleen), skin rash, or lung infiltrates. In some cases, MCD may be associated with systemic symptoms, such as fever, fatigue, night sweats, and weight loss.
The physical examination may also reveal signs of other underlying medical conditions or complications, such as infections, autoimmune disorders, or other types of cancer. Depending on the location and severity of the disease, additional diagnostic tests, such as imaging studies or a biopsy of the affected tissue, may be necessary to confirm the diagnosis and differentiate it from other possible conditions.
Differential diagnosis
The differential diagnosis of Castleman disease may depend on the location and subtype of the disease. Unicentric Castleman disease (UCD) is usually localized to a single lymph node or a group of lymph nodes in one region of the body, while multicentric Castleman disease (MCD) involves multiple lymph nodes in different regions of the body and may also involve other tissues and organs. Differential diagnoses of UCD may include other benign lymphoid or soft tissue tumors, such as lymphoma, reactive lymphadenopathy, or other types of benign tumors.
Differential diagnosis of MCD may include other lymphoproliferative disorders, such as lymphoma or leukemia, or other conditions that involve lymph node enlargements and systemic symptoms, such as infections (e.g., tuberculosis or HIV), autoimmune disorders (e.g., lupus or rheumatoid arthritis), or other types of cancer.
Since the symptoms of Castleman disease can vary depending on the subtype and location of the disease, it is important to conduct a thorough medical history, physical examination, and diagnostic tests to differentiate it from other possible conditions. Further testing may include blood tests to evaluate levels of cytokines and other markers, imaging studies (e.g., CT scan, MRI, or PET scan), and a biopsy of the affected tissue for pathological examination.
The treatment of Castleman disease depends on the subtype and extent of the disease, as well as the presence of underlying medical conditions or complications. Unicentric Castleman disease (UCD) is usually treated with surgical removal of the affected lymph node or tissue. In some cases, radiation therapy may be used as an alternative or adjunct to surgery. The prognosis for UCD is generally favorable, and most patients can be cured with local treatment.
Multicentric Castleman disease (MCD) is usually more difficult to treat and may require systemic therapy. The treatment of MCD depends on the subtype and severity of the disease, as well as the presence of underlying medical conditions or complications. In some cases, the use of anti-viral medications or immunosuppressive therapy may be effective in treating certain subtypes of MCD, such as human herpesvirus 8 (HHV-8)-associated MCD. In other cases, chemotherapy or targeted therapy may be used to reduce the size of the affected lymph nodes and improve symptoms.
Corticosteroids may also be used to reduce inflammation and improve symptoms, although they may have significant side effects with long-term use. In some cases, MCD may be associated with the development of other types of cancer, such as lymphoma, and may require more aggressive treatment, such as high-dose chemotherapy with stem cell transplantation. The management of Castleman disease should be individualized and based on the patient’s specific condition and response to treatment. Close monitoring and follow-up are important to assess the response to treatment and detect any potential complications or recurrence of the disease.
11 mg per kg intravenously every three weeks
Ensure the infusion is conducted over a duration of one hour
resume till treatment failure
https://www.ncbi.nlm.nih.gov/books/NBK576394/
medtigo
Castleman Disease
Updated :
April 7, 2023
Castleman disease, also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia, is a rare disorder that affects the lymph nodes and related tissues. It was first described by Dr. Benjamin Castleman in 1954. Castleman disease is characterized by the abnormal growth of lymphoid tissue, which can cause the enlargement of one or more lymph nodes in the body.
The disease can be classified into two main types: unicentric Castleman disease (UCD), which affects a single lymph node or a group of lymph nodes in one region of the body, and multicentric Castleman disease (MCD), which affects multiple lymph nodes in different regions of the body. The Castleman disease can occur in people of any age and gender, but it is more commonly diagnosed in adults.
The cause of Castleman disease is not fully understood, but it is thought to be related to an abnormal immune system response. Symptoms of Castleman disease may vary depending on the type and location of the disease and can include enlarged lymph nodes, fever, night sweats, fatigue, weight loss, and other symptoms. Treatment options may include surgery, radiation therapy, chemotherapy, or targeted therapy, depending on the type and severity of the disease.
Castleman disease is a rare disorder, with an estimated incidence of 1 in 100,000 individuals per year. It can occur in people of any age and gender, but it is more commonly diagnosed in adults. Multicentric Castleman disease (MCD) accounts for approximately 10-20% of all cases of Castleman disease. MCD is more common in men than in women, with a male-to-female ratio of approximately 2:1.
It is also more common in individuals over the age of 50. Unicentric Castleman disease (UCD) is more common in women than in men and typically affects younger individuals, with a peak incidence in the third decade of life. The prevalence of Castleman disease may be underestimated due to its rarity and the need for more awareness among healthcare providers. Further research is needed to better understand the epidemiology and risk factors for Castleman disease.
The pathophysiology of Castleman disease is not completely understood, but it is believed to involve an abnormal immune system response, particularly involving the production of cytokines. Cytokines are signaling molecules that are produced by immune cells, and they play a role in regulating the immune response. In Castleman disease, abnormal cytokine production leads to the growth of lymphoid tissue, which can cause the enlargement of one or more lymph nodes in the body.
In unicentric Castleman disease (UCD), the abnormal growth of lymphoid tissue occurs in a single lymph node or a group of lymph nodes in one region of the body. UCD is typically a localized and benign condition that may be cured with surgical removal of the affected lymph node. In multicentric Castleman disease (MCD), the abnormal growth of lymphoid tissue occurs in multiple lymph nodes in different regions of the body and may also involve other tissues and organs, such as the liver, spleen, and bone marrow.
MCD is characterized by systemic symptoms such as fever, night sweats, fatigue, and weight loss, which are thought to be related to the production of abnormal cytokines. MCD can be further divided into two subtypes: HHV-8-associated MCD, which is caused by the human herpesvirus 8 (HHV-8) and is more common in people with HIV/AIDS or other immunocompromised conditions, and idiopathic MCD, which has no known cause.
The abnormal cytokine production in Castleman disease is thought to be related to the activation of a type of immune cell called the plasma cell. Abnormal plasma cell activation may be triggered by various factors, including infections, autoimmune disorders, or other underlying medical conditions. Further research is needed to better understand the pathophysiology of Castleman’s disease and to develop more effective treatments.
The exact cause of Castleman disease is not known, but it is believed to be related to an abnormal immune system response. In some cases, Castleman disease is associated with infections, such as the human herpesvirus 8 (HHV-8), which is known to cause multicentric Castleman disease (MCD) in people with HIV/AIDS or other immunocompromised conditions. HHV-8-associated MCD is more common in people with HIV/AIDS but can also occur in people who are not infected with HIV.
Other underlying medical conditions or factors may also contribute to the development of Castleman disease, including autoimmune disorders, such as rheumatoid arthritis or lupus, or other types of cancer, such as lymphoma. In some cases, Castleman disease may be idiopathic, meaning that there is no known cause. The Castleman disease can be classified into two main types based on the location and pattern of lymph node involvement.
Unicentric Castleman disease (UCD) involves a single lymph node or a group of lymph nodes in one region of the body, while multicentric Castleman disease (MCD) involves multiple lymph nodes in different regions of the body. MCD is more commonly associated with infections or underlying medical conditions, while UCD is often idiopathic. Further research is needed to better understand the underlying causes of Castleman disease and to develop more effective treatments for this rare disorder.
The prognosis of Castleman disease depends on several factors, such as the subtype and extent of the disease, the presence of underlying medical conditions or complications, and the response to treatment. Unicentric Castleman disease (UCD) has an excellent prognosis, and most patients can be cured with surgical removal of the affected lymph node or tissue. The recurrence rate is low, and long-term survival is expected. Multicentric Castleman disease (MCD), on the other hand, has a more variable prognosis and may be associated with significant morbidity and mortality.
The overall survival of MCD varies depending on the subtype of the disease and the presence of coexisting medical conditions. The human herpesvirus 8 (HHV-8)-associated MCD subtype has a better prognosis compared to the idiopathic or HIV-associated subtypes. The use of targeted therapies, such as anti-IL-6 antibodies, has improved the prognosis of MCD, particularly in patients who do not respond to standard therapies.
In some cases, MCD may be associated with the development of other types of cancer, such as lymphoma, and may require more aggressive treatment, such as high-dose chemotherapy with stem cell transplantation. Close monitoring and follow-up are essential to detect any potential complications or recurrence of the disease. Overall, the prognosis of Castleman disease can vary widely depending on the subtype and individual patient factors, and close collaboration between the patient, medical team, and other healthcare professionals is critical to achieving the best possible outcome.
Clinical History
The clinical history of Castleman disease may vary depending on the subtype and location of the disease. Unicentric Castleman disease (UCD) is usually asymptomatic or may cause mild symptoms, such as swelling or tenderness of the affected lymph node or tissue. In some cases, UCD may be discovered incidentally during imaging studies or surgical procedures. Multicentric Castleman disease (MCD) is usually more symptomatic and may cause fever, fatigue, night sweats, weight loss, and generalized lymphadenopathy (enlarged lymph nodes throughout the body).
MCD may also involve other tissues and organs, such as the liver, spleen, or lungs, and can cause a variety of symptoms depending on the location of the involvement. MCD may be associated with an increased risk of infections, such as pneumonia or sepsis, and may also increase the risk of other types of cancer, such as lymphoma. In some cases, MCD may be associated with a specific infection, such as the human herpesvirus 8 (HHV-8), which is known to cause HHV-8-associated MCD in people with HIV/AIDS or other immunocompromised conditions.
HHV-8-associated MCD may be more severe and progressive than other subtypes of MCD and may require more aggressive treatment. Since the symptoms of Castleman disease can be nonspecific and overlap with other conditions, a thorough medical history, physical examination, and diagnostic tests are important to differentiate it from other possible conditions.
Physical examination
The physical examination findings in Castleman disease may depend on the subtype and location of the disease. Unicentric Castleman disease (UCD) may present as a localized mass or swelling that is usually non-tender and non-mobile. In some cases, UCD may be discovered incidentally during imaging studies or surgical procedures.
Multicentric Castleman disease (MCD) is usually associated with generalized lymphadenopathy (enlarged lymph nodes throughout the body) and may also involve other tissues and organs, such as the liver, spleen, or lungs. MCD may present with enlarged lymph nodes in the neck, armpits, or groin, as well as abdominal pain, hepatosplenomegaly (enlarged liver and spleen), skin rash, or lung infiltrates. In some cases, MCD may be associated with systemic symptoms, such as fever, fatigue, night sweats, and weight loss.
The physical examination may also reveal signs of other underlying medical conditions or complications, such as infections, autoimmune disorders, or other types of cancer. Depending on the location and severity of the disease, additional diagnostic tests, such as imaging studies or a biopsy of the affected tissue, may be necessary to confirm the diagnosis and differentiate it from other possible conditions.
Differential diagnosis
The differential diagnosis of Castleman disease may depend on the location and subtype of the disease. Unicentric Castleman disease (UCD) is usually localized to a single lymph node or a group of lymph nodes in one region of the body, while multicentric Castleman disease (MCD) involves multiple lymph nodes in different regions of the body and may also involve other tissues and organs. Differential diagnoses of UCD may include other benign lymphoid or soft tissue tumors, such as lymphoma, reactive lymphadenopathy, or other types of benign tumors.
Differential diagnosis of MCD may include other lymphoproliferative disorders, such as lymphoma or leukemia, or other conditions that involve lymph node enlargements and systemic symptoms, such as infections (e.g., tuberculosis or HIV), autoimmune disorders (e.g., lupus or rheumatoid arthritis), or other types of cancer.
Since the symptoms of Castleman disease can vary depending on the subtype and location of the disease, it is important to conduct a thorough medical history, physical examination, and diagnostic tests to differentiate it from other possible conditions. Further testing may include blood tests to evaluate levels of cytokines and other markers, imaging studies (e.g., CT scan, MRI, or PET scan), and a biopsy of the affected tissue for pathological examination.
The treatment of Castleman disease depends on the subtype and extent of the disease, as well as the presence of underlying medical conditions or complications. Unicentric Castleman disease (UCD) is usually treated with surgical removal of the affected lymph node or tissue. In some cases, radiation therapy may be used as an alternative or adjunct to surgery. The prognosis for UCD is generally favorable, and most patients can be cured with local treatment.
Multicentric Castleman disease (MCD) is usually more difficult to treat and may require systemic therapy. The treatment of MCD depends on the subtype and severity of the disease, as well as the presence of underlying medical conditions or complications. In some cases, the use of anti-viral medications or immunosuppressive therapy may be effective in treating certain subtypes of MCD, such as human herpesvirus 8 (HHV-8)-associated MCD. In other cases, chemotherapy or targeted therapy may be used to reduce the size of the affected lymph nodes and improve symptoms.
Corticosteroids may also be used to reduce inflammation and improve symptoms, although they may have significant side effects with long-term use. In some cases, MCD may be associated with the development of other types of cancer, such as lymphoma, and may require more aggressive treatment, such as high-dose chemotherapy with stem cell transplantation. The management of Castleman disease should be individualized and based on the patient’s specific condition and response to treatment. Close monitoring and follow-up are important to assess the response to treatment and detect any potential complications or recurrence of the disease.
https://www.ncbi.nlm.nih.gov/books/NBK576394/
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