What is the life expectancy of someone with Castleman disease?

Castleman disease, also known as Castleman syndrome or angiofollicular lymph node hyperplasia, is a rare and complex disorder of the lymphatic system. It is characterized by abnormal growth of lymphatic cells in lymph nodes, leading to the formation of enlarged lymph nodes. Castleman disease can occur in two forms: unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD).

Unicentric Castleman disease (UCD) is the more common form and typically affects a single lymph node or a group of adjacent lymph nodes. It is usually localized and does not spread to other parts of the body. UCD is often asymptomatic, meaning individuals may not experience any noticeable symptoms. However, if symptoms do occur, they may include localized swelling, fatigue, night sweats, and flu-like symptoms. Surgical removal of the affected lymph node(s) is the primary treatment for UCD, and the prognosis is generally excellent. With appropriate treatment, individuals with UCD can expect to live a normal lifespan.

Multicentric Castleman disease (MCD) is a more aggressive and potentially life-threatening form of the disease. It involves multiple lymph nodes and can affect various organs and tissues throughout the body. MCD is often associated with dysregulated immune system function and overproduction of certain proteins, such as interleukin-6 (IL-6). Symptoms of MCD can vary widely and may include enlarged lymph nodes, fever, night sweats, weight loss, fatigue, skin rash, and organ dysfunction.

The life expectancy of someone with multicentric Castleman disease depends on several factors, including the subtype of MCD, the extent of organ involvement, and the effectiveness of treatment. There are two main subtypes of MCD: idiopathic multicentric Castleman disease (iMCD) and human herpesvirus-8 (HHV-8)-associated multicentric Castleman disease (HHV-8-associated MCD).

Idiopathic multicentric Castleman disease (iMCD) is the more common subtype and occurs without a known cause. It is often more challenging to treat and manage compared to UCD or HHV-8-associated MCD. The prognosis for iMCD varies widely, with some individuals experiencing a more indolent course of the disease and others facing a more aggressive progression. The average life expectancy for individuals with iMCD can range from a few years to over a decade, depending on the response to treatment and the presence of complications.

HHV-8-associated multicentric Castleman disease is caused by infection with the human herpesvirus-8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). This subtype is often seen in individuals with weakened immune systems, such as those with HIV/AIDS. The prognosis for HHV-8-associated MCD is generally poorer compared to iMCD. Without appropriate treatment, the average life expectancy for individuals with HHV-8-associated MCD is typically shorter, ranging from a few months to a few years.

It is important to note that Castleman disease is a complex and heterogeneous condition, and individual cases can vary significantly. The information provided here is a general overview and should not be considered as a definitive prognosis for any specific individual. It is crucial for individuals diagnosed with Castleman disease to work closely with their healthcare team to determine the most appropriate treatment plan and to monitor the progression of the disease.