Overview
Castleman disease is a rare disorder that involves an overgrowth of cells in your body's lymph nodes. It is characterized by an abnormal proliferation of lymph node tissue, leading to enlarged lymph nodes. The most common form of the disorder, unicentric Castleman disease, affects a single lymph node, usually in the chest or abdomen. Multicentric Castleman disease affects multiple lymph nodes throughout the body, and it is further divided into three types: HHV-8-associated MCD, idiopathic MCD, and POEMS-associated MCD. The type that affects only one lymph node, unicentric Castleman disease, can usually be successfully treated with surgery. Symptoms include fever, unintended weight loss, fatigue, night sweats, and enlarged liver or spleen. The cause of Castleman disease is not clear, but infection with human herpes virus 8 (HHV-8) is associated with the multicentric form of the disease.
Understanding the disease
Symptoms
The symptoms of Castleman disease may include:
- Enlarged lymph nodes: Castleman disease is characterized by the enlargement of lymph nodes, often found in the neck, collarbone, underarm, or groin areas.
- Fever: Some people with Castleman disease may experience fever.
- Unintended weight loss: Patients may experience a loss of weight without trying.
- Fatigue: Persistent feelings of tiredness or exhaustion can be a symptom of Castleman disease.
- Night sweats: Excessive sweating during nighttime that soaks through bedclothes or clothes can occur in some cases.
- Enlarged liver or spleen: These organs may become enlarged in people with Castleman disease. It is important to note that the presence of these symptoms does not necessarily mean a person has Castleman disease. If you experience any of these symptoms or have an enlarged lymph node, it is recommended to consult with a healthcare provider for further evaluation.
Development
Idiopathic MCD is the type of Castleman disease where the cause is unknown. HHV-8-associated MCD is associated with human herpes virus type 8 and human immunodeficiency virus (HIV). POEMS-associated MCD is linked to POEMS syndrome, a rare blood disorder that affects other parts of the body, including the nerves.
Assessment and Diagnosis
Triage
If you notice an enlarged lymph node on the side of your neck or in your underarm, collarbone, or groin area, you should talk to your health care provider. Additionally, you should call your care team if you have a persistent feeling of fullness in your chest or abdomen, fever, fatigue, or unexplained weight loss. These symptoms may indicate the presence of Castleman disease.
Diagnosis
Diagnosis of Castleman disease involves a combination of medical history review, physical examination, and diagnostic tests. To diagnose Castleman disease, your healthcare provider may recommend:
- Blood and urine tests: These tests can help rule out other infections or diseases and reveal anemia and abnormalities in blood proteins that may be typical of Castleman disease.
- Imaging tests: A CT scan of the neck, chest, abdomen, and pelvis may be used to look for enlarged lymph nodes, liver, or spleen. Positron emission tomography (PET) scans may be used to diagnose Castleman disease and assess the effectiveness of treatment.
- Lymph node biopsy: A tissue sample from an enlarged lymph node is removed and examined in the laboratory to distinguish Castleman disease from other types of lymphatic tissue disorders, such as lymphoma. The specific diagnostic approach depends on the type of Castleman disease (unicentric or multicentric) and the presence of other medical conditions. Your healthcare provider may refer you to a hematologist for further evaluation and management.
Management and Treatment
Home Remedies
It's important to consult with a healthcare professional for personalized advice on managing Castleman disease.