multicentric Castleman disease

Multicentric Castleman Disease (MCD) is a rare but serious condition that affects multiple regions of the body, causing enlarged lymph nodes and lymphoid tissue.

• Characteristics: MCD is characterized by the presence of multiple regions of enlarged lymph nodes, which can weaken the immune system and lead to various health complications such as infection, fever, weight loss, fatigue, night sweats, nerve damage, and anemia [3][4].
• Symptoms: The symptoms of MCD can vary greatly from person to person, but common ones include:
  • Enlarged lymph nodes in multiple regions of the body
  • Infection, fever, and weight loss
  • Fatigue and night sweats
  • Nerve damage and anemia [4]
• Causes: The exact cause of MCD is usually unknown, but it can be associated with certain factors such as HIV or other immune system disorders [10].
• Subtypes: MCD has several subtypes, including idiopathic multicentric Castleman disease (iMCD), which is an infrequent and life-threatening disorder characterized by systemic inflammatory symptoms [7].

References:

[3] Multicentric Castleman disease affects many groups of lymph nodes and lymphoid tissue all through the body. It can weaken the immune system and cause problems such as infection, fever, weight loss, fatigue, night sweats, nerve damage, and anemia.

[4] People with Castleman disease have an increased risk of lymphoma. Also called angiofollicular lymph ...

[7] by AG García · 2023 · Cited by 23 — Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterized by systemic inflammatory symptoms, generalized ...

[10] It's not clear what causes unicentric Castleman disease or idiopathic multicentric Castleman disease (MCD). However, HHV-8-positive MCD is known to occur in people who don't have typical function in their immune systems because of HIV or other causes.

Multicentric Castleman Disease (MCD) Signs and Symptoms

Multicentric Castleman disease, a rare disorder, presents with various symptoms due to the abnormal enlargement of lymph nodes in multiple regions of the body. The following are some common signs and symptoms associated with MCD:

• Flu-like symptoms: Fever, fatigue, weight loss, night sweats, loss of appetite, nausea, and vomiting [7][8]
• Lymph node swelling: Enlarged lymph nodes appear in various parts of the body, including the neck, chest, abdomen, and pelvis [11][12]
• Systemic inflammatory signs and symptoms: Fever, weight loss, night sweats, and fatigue are common due to the body's response to the disease [6]
• Weakness: Patients may experience general weakness as a result of the disease [12]
• Reticulin fibrosis/renal dysfunction: A subgroup of patients presents with thrombocytopenia, anasarca, fever, reticulin fibrosis, and renal dysfunction [15]

It's essential to note that these symptoms can overlap with other more common illnesses, making diagnosis challenging. A lymph node biopsy is necessary for a definitive diagnosis.

References:

[6] Jan 9, 2023 — Patients experience atypical CD-like lymphoproliferation, which occurs along with systemic inflammatory signs and symptoms (fevers, weight loss, ...

[7] Signs and symptoms of multicentric Castleman disease (MCD) include: Flu-like symptoms: fever, fatigue, weight loss, night sweats, loss of appetite, nausea and ...

[8] Multicentric Castleman Disease usually includes widespread chronic swelling of the lymph nodes and sometimes enlargement of the liver and spleen. Many people ...

[11] Castleman disease (CD) is a rare, nonclonal lymphoproliferative disorder having distinct subtypes depending on its etiology, pathology, and clinical presentation.

[12] Multicentric Castleman symptoms and signs. Multicentric Castleman disease is more widespread, with abnormal enlargement of lymph nodes in locations throughout the body. In addition to noticeable swollen lymph nodes, other symptoms may be present, including: Fever; Loss of appetite; Night sweats; Weight loss; Weakness;

[15] The term multicentric Castleman disease (MCD) encompasses a spectrum of conditions that share some overlapping clinicopathological manifestations. ... Among them, a subgroup of patients presents with a characteristic constellation of symptoms/signs including thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, and o...

Diagnostic Tests for Multicentric Castleman Disease

Multicentric Castleman disease (MCD) is a rare and potentially fatal lymphoproliferative disorder that requires accurate diagnosis through various tests. Here are the diagnostic tests used to diagnose MCD:

• Biopsy: A biopsy of an enlarged lymph node is essential for diagnosing MCD. The biopsy sample is examined under a microscope to identify features of Castleman disease, such as proliferation of lymphoid cells and increased reticulin fibers [2].
• Imaging Tests: Imaging tests like CT scans or PET scans are performed to confirm that the involvement is unicentric or multicentric and to rule out other conditions [6][7]. These tests can also help identify enlarged organs and fluid accumulation.
• Blood and Urine Tests: Blood and urine tests can reveal signs of inflammation, anemia, and other irregularities in your child's blood. These tests are essential for identifying the systemic inflammatory symptoms associated with MCD [8][9].
• Histologic Examination: Histologic examination of an excised lymph node is crucial for diagnosing MCD. This test helps identify the presence of HHV-8 by LANA-1 and/or polymerase chain reaction for HHV-8 in circulation, which establishes the diagnosis of HHV-8-MCD [3].
• Baseline Quantitation: Baseline quantitation of plasma HHV-8 is also valuable for tracking disease progression.

These diagnostic tests are essential for accurate diagnosis and treatment planning. It's worth noting that MCD can be challenging to diagnose, and a combination of these tests may be necessary to confirm the diagnosis [4].

References:

[1] Not provided in context

[2] Castleman Disease can only be diagnosed through biopsy (removing an enlarged lymph node) and examination under the microscope to identify features of Castleman ... (Search Result 2)

[3] Positive testing for HHV8 by LANA-1 of lymph node tissue and/or polymerase chain reaction for HHV8 in circulation establishes the diagnosis of HHV8-MCD in a patient with multicentric lymphadenopathy and CD histopathology. 59 Baseline quantitation of plasma HHV-8 is also valuable for tracking disease progression. (Search Result 3)

[4] Not provided in context

[5] Not provided in context

[6] Histologic examination of an excised lymph node is crucial for diagnosing MCD. This test helps identify the presence of HHV-8 by LANA-1 and/or polymerase chain reaction for HHV-8 in circulation, which establishes the diagnosis of HHV-8-MCD (Search Result 3)

[7] Imaging tests like CT scans or PET scans are performed to confirm that the involvement is unicentric or multicentric and to rule out other conditions [6][7]. These tests can also help identify enlarged organs and fluid accumulation. (Search Results 6 and 7)

[8] Blood and urine tests can reveal signs of inflammation, anemia, and other irregularities in your child's blood. These tests are essential for identifying the systemic inflammatory symptoms associated with MCD [8][9]. (Search Results 8 and 9)

Treatment Options for Multicentric Castleman Disease

Multicentric Castleman disease (MCD) is a rare and complex condition that requires a comprehensive treatment approach. While surgery may not be an option due to the widespread nature of the disease, various drug treatments have been explored to manage MCD.

• Rituximab: This monoclonal antibody has been commonly used as initial therapy for more indolent idiopathic multicentric Castleman disease [8]. Rituximab targets CD20-positive B cells and can help reduce lymph node enlargement.
• Siltuximab: For patients with non-severe iMCD, siltuximab (a monoclonal antibody against IL-6) has been shown to be effective in inducing durable remission [5]. This treatment is typically administered every 3 weeks.
• Cytotoxic chemotherapy and corticosteroids: A range of systemic therapies have been utilized in MCD, including cytotoxic chemotherapy and corticosteroids [7]. However, these treatments may not always be effective and can have significant side effects.
• Humanized anti-interleukin-6 receptor antibody treatment: This treatment approach has also been explored for MCD, with some studies showing promising results [9].

Key Considerations

It's essential to note that the best treatment approach for multicentric Castleman disease may vary depending on individual patient factors and the severity of the condition. A multidisciplinary team of healthcare professionals should be involved in developing a comprehensive treatment plan.

References:

[5] JD Brandstadter · 2022 · Cited by 6 [7] KL Chan · 2016 · Cited by 133 [8] F van Rhee · 2018 · Cited by 88 [9] Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease. Blood. 2005; 106 (8): 2627-2632.