iMCD

Overview:

Castleman disease is a rare disease that affects the lymphatic system. The most common form of this disease is called unicentric Castleman disease, which affects a single lymph node or region of lymph nodes. Multicentric Castleman Disease (MCD) is a form of Castleman disease that has spread to multiple regions of lymph nodes rather than being isolated to a single location or node which makes it more aggressive in nature. ¹

There are two main types of MCD. One type is known as HHV-8-associated or HIV-associated MCD, which is triggered by a human herpesvirus (the same family that causes cold sores) or human immunodeficiency virus, respectively1. The other type is known as idiopathic MCD (iMCD). Being ‘idiopathic’ means that the cause of your MCD is not known1. Only between 3 and 4 people among every million in the general population are diagnosed with iMCD each year. ¹ It can affect anyone – males, females, adults and children, although most people with iMCD are above the age of 45.¹

Diagnosis: 

It is important to diagnose iMCD as soon as possible in order to get the most out of potential treatments. As soon as you have been diagnosed with iMCD, your doctor physician will be able to is able to assess your condition and determine the most suitable management plan for you. ²

Symptoms: 

Before you were diagnosed, you may have experienced a variety of symptoms that could have been mistaken for a variety of other diseases. iMCD varies from person to person and can have a mild to severe clinical presentation, with symptoms including:

Weakness and Fatigue; Nausea and vomiting; Shortness of breath; Loss of appetite and weight loss; Enlarged lymph nodes; Night sweats; Fever; Skin problems such as rashes; Enlarged liver or spleen; Renal dysfunction; Peripheral neuropathy; Anaemia²

References:
1. Mukherjee S et al. Epidemiology and treatment patterns of HHV-8-negative/idiopathic multicentric Castleman disease in the era of Interleukin-6 directed therapy. Blood Adv. 2022;6(2):359–367.
2. van Rhee F et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood 2018;132(20):2115–2124.