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What is Castleman Disease?

Nurse Talks Review Notes: Castleman Disease


Castleman Disease (CD) is a disease of the lymph nodes. First described by Dr Benjamin Castleman, CD is characterised by enlargement of the nodes with underlying microscopic changes. The histological changes are due to an overproduction of lymphocytes and pro-inflammatory proteins such as an increase in interlukin-6 (IL-6) which can result to inflammation (Castleman Disease Collaborative Network (CDCN) (2021)). It is important to note that this is not a malignant disease in itself.


Other terms used for CD are angiofolicular lymph node hyperplasia or giant lymph node hyperplasia.


The exact cause of CD is still unknown but there are associative factors like viruses (Human Herpes Virus-8 as an example), genetic mutations and autoimmunity (Markman, 2021).


Anatomy of the Lymph Node

by Medicosis Perfectionalist (2018)



There are several categories of CD:

  • Unicentric CD the CD that involves enlargement in a single node only or in a specific region of the lymph node.

  • Multicentric CD - CD that occurs in various lymph nodes of the body which can happen at the same time.

  • HHV-8 positive Multicentric Castleman disease (HHV8 + MCD) - This type of CD is associated with Human Herpes Virus 8 infection which may be a result of a suppressed immune system such as those experienced in patients with HIV.

  • Idiopathic Multicentric CD (iMCD) (has 2 subtypes) -

    1. iMCD TAFRO – a subtype of iMCD which involves:

      • Thrombocytopenia or low platelet

      • Anasarca or generalized edema

      • Fevers

      • Reticulin myelofibrosis (reticulin is a protein resembling a collagen-like structure which is present in connective tissues especially in muscles and nerve fibers).

      • Abnormal enlargement of organomegaly, and normal or only slightly elevated immunoglobulin levels.

2. Idiopathic Multicentric CD Not Otherwise Specified (iMCD NOS) – This is the type of iMCD which also does not have any identified cause and does not have the TAFRO features.

Signs and symptoms:

The signs and symptoms of CD can be wide ranging depending on the extent of the enlargement and the affected surrounding organs due to the pressure it exerts.


Wheezing or difficulty of breathing may be experienced especially if the affected lymph node is in the chest and/or abdomen.


The signs and symptoms depend on the type of CD and the severity of the lymph node enlargement.


Other signs and symptoms include fatigue, fever, rashes, loss of appetite, weight loss, sweating and anemia (WebMD, 2021).


How is the disease detected?

The diagnosis of this disease in patients can sometimes be difficult since there are other diseases that may also share similar features. Imaging procedures such as CT scanning or MRI may be conducted to assess lymph node growth especially if the enlargement affects other underlying organs. A biopsy of the affected lymph node and analysis through histopathology will also be conducted to rule out diseases that have similar signs and symptoms (CDCN, 2021).

Treatment and Management

Depending on the type of the CD and how severe the patient’s symptoms are, several interventions may be implemented such as surgical removal of the affected lymph node (usually for the unicentric type), radiation therapy, administration of corticosteroids, chemotherapy and immunotherapy (Markman, 2021). Sometimes, a medication called Siltuximab may be used for patients with multicentric CD. This drug neutralizes interlukin-6 (van Rhee, 2014).


 

Disclaimer:


The content in this blog is for informational purposes only and should not be taken as medical advice. It is always best to consult your doctor for medical questions.


If you are a healthcare provider, the content here should not be used to make any diagnosis, give advice or prescribe treatment as this blog is for informational and educational purposes only. Healthcare is an everchanging field and each patient is unique. It is your responsibility as a healthcare provider to always refer to current care standards and practices.

 

References:


Castleman Data Collaborative Network, 2021. Overview - Castleman Disease Collaborative Network | CDCN. [online] CDCN. Available at: <https://cdcn.org/castleman-disease/overview/>


Markman, M., 2021. Castleman disease. [online] Cancer Treatment Centers of America. Available at: <https://www.cancercenter.com/risk-factors/castleman-disease>

Srkalovic, G., Marijanovic, I., Srkalovic, M. B. and Fajgenbaum, D. C. (2017) TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease, Bosnian Journal of Basic Medical Sciences, 17 (2), pp. 81–84. DOI:10.17305/bjbms.2017.1930.


van Rhee, F., Wong, R. S., Munshi, N., Rossi, J.-F., Ke, X.-Y., Fosså, A., et al. (2014) Siltuximab for multicentric Castleman’s disease: a randomised, double-blind, placebo-controlled trial, The Lancet Oncology, 15 (9), pp. 966–974. DOI:10.1016/s1470-2045(14)70319-5.


WebMD, 2021. Castleman Disease. [online] WebMD. Available at: <https://www.webmd.com/hiv-aids/castleman-disease#1> [Accessed 27 August 2021].

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