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Articles / Journals

Dispenzieri, Angela, and David C. Fajgenbaum. “Overview of Castleman disease.” Blood, The Journal of the American Society of Hematology 135, no. 16 (2020): 1353-1364. Accessed 11 July 2024.

mayoclinic.org

“Castleman Disease.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 3 May 2024. Accessed 11 July 2024.

my.clevelandclinic.org

Professional, C. C. M. Cleveland Clinic medical. “Castleman Disease: Types, Symptoms & Treatment.” Cleveland Clinic.  Accessed 11 July 2024

What is Castleman Disease (CD)?

Castleman disease is a rare disease that affects lymph nodes in any region of the body and normally results in high rates of cell division. This causes an overgrowth of cells in a specific region – and can be either benign or malignant. The Castleman disease directly weakens the immune system as the enlarged lymph nodes remain in a chronic hyper-inflammatory state, in turn damaging multiple organs. CD can also increase chances of getting cancer. Under Castleman disease, there are two main categories that are determined by the number of regions in the body with enlarged lymph nodes; unicentric and multicentric. Castleman Disease can affect anyone. UCD is more prevalent in both sexes in their 30s or 40s while MCD is more prevalent in the male sex in their 50s or 60s. “CD is a rare disease with about 4,300 to 5,200 cases each year in the USA.”

1. Unicentric Castleman Disease (UCD): affects a single lymph node or multiple lymph nodes in the region of a single lymph node (more commonly in the chest/abdomen). There are no exact causes but patients with UCD are either asymptomatic or have symptoms (common to both UCD and MCD) such as: 

    1. Fever 
    2. Night sweats 
    3. Fatigue 
    4. Unintended weight loss 
    5. Enlarged liver or spleen 
    6. Enlarged lymph nodes
    7. Nausea and vomiting 
    8. Swelling in abdomen, feet, neck, or groin 
    9. Anemia 

 

2. Multicentric Castleman Disease (MCD): affects multiple lymph nodes in different regions of the body. MCD is divided into three subtypes: 

  1.  
  • POEMS-associated MCD: POEMS (“polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell proliferative disorder, and skin changes”) is a rare blood disease that affects the nerves and other parts of the body. It occurs simultaneously with MCD. 
  • HHV-8-associated MCD: HHV-8 or Human Herpes Virus 8 is a common disease that accompanies MCD. People tested positive for HIV are more susceptible to this type of MCD, but it is not chronic and does not get one sick. 
  • Idiopathic MCD (iMCD): This is the most common form of MCD and is also known as HHV-8-negative MCD. Its causes are unknown hence ‘idiopathic’ in nature. There are three types of iMCD: 
      1. iMCD associated with TAFRO: TAFRO syndrome accompanying iMCD. TAFRO includes low platelet count, swelling from fluid build up, fever, kidney dysfunction, and enlarged spleen/liver. 
      2. iMCD with idiopathic plasmacytic lymphadenopathy (iMCD-IPL): This includes too much platelet and antibody production and presence in the blood 
      3. iMCD, not otherwise specified (iMCD-NOS): iMCD with unknown causes; not typically associated with TAFRO or POEMS.
    1.  

Since CD has a few common symptoms with the flu; healthcare advisors would run the tests mentioned below to diagnose one for CD, they are: 

  1. Biopsies 
  2. Blood and urine tests 
  3. HIV tests 
  4. Imaging tests

The treatment of a CD depends on whether it is a UCD or a MCD. 

In the case of a UCD, the primary treatment involves direct removal of the enlarged lymph node through surgery. In cases where the lymph node is too big, the patient would need to go through immunotherapy or radiation therapy in order to shrink the lymph node in order to proceed with the surgery.  

In the case of a MCD, it is more difficult to treat due to its widespread nature. Healthcare advisors would initially try to see if one has HHV-8 and the severity. Hence they would use the methods below to treat MCD: 

  1. Medications that immediately reduce inflammation in an extreme case of MCD in order to effectively subside swelling and enlargement 
  2. Chemotherapy drugs that prevent inflammation of affected regions to limit further division of cells in the lymph nodes 
  3. Immunotherapy to release antibodies and boost the immune system 
  4. Antiviral drugs to specifically treat HHV-8 or HIV

Patients with UCD have a good outlook after surgery as it normally cures the root cause after removing the single affected lymph node. Life expectancy isn’t altered. The prognosis for people with MCD depends on the type of MCD and the severity. If it is too severe, then the MCD can be life threatening especially cases with HHV-8-MCD as the HIV affects the immune system and increases susceptibility to diseases. “About 65% to 75% of people diagnosed with MCD are still alive five years later” – and future developments hope to increase the chances.