Castleman Disease Causes, Symptoms, Diagnosis And Treatment

What Is Castleman Disease?

Castleman disease is a rare disorder that involves an overgrowth (proliferation) of cells in your body’s disease-fighting network (lymphatic system).

Also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, Castleman disease can occur in a localized (unicentric) or widespread (multicentric) form.

CD is not cancer. Instead, it is called a lymphoproliferative disorder. This means there is an abnormal overgrowth of cells of the lymph system that is similar in many ways to lymphomas (cancers of lymph nodes).

There are two basic types of Castleman disease:

Unicentric Castleman disease.

This localized form of the disease affects only a single gland (lymph node) in your lymphatic system.

Multicentric Castleman disease.

This type affects multiple lymph nodes and lymphatic tissues, and can severely weaken your immune system.

Multicentric Castleman disease can be further classified as:

Multicentric Castleman disease without POEMS syndrome
Multicentric Castleman disease with POEMS syndrome that involves areas of abnormal bone (osteosclerotic lesions)
Multicentric Castleman disease with POEMS syndrome without osteosclerotic lesions

Causes Of Castleman Disease:

The underlying cause of castleman disease is yet unknown.

However, infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease.

The precise role of HHV-8 is unclear. But it appears to cause malfunctioning immune system cells to reproduce rapidly.

The immune system cells produce a protein called interleukin-6 (IL-6) that contributes to the overgrowth of lymphatic cells.

The only known risk factor for Castleman disease appears to be having HIV/AIDS

Symptoms Of Castleman Disease:

Symptoms of castleman disease vary according to the type of the disease one has.

Unicentric castleman disease
A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating
An enlarged lump under the skin in the neck, groin or armpit
Unintended weight loss
Less commonly, fever, night sweats and weakness
Multicentric castleman disease:
Fever
Night sweats
Fatigue and weakness
Loss of appetite
Unintended weight loss
Enlarged lymph nodes, usually around the neck, collarbone, underarm and groin areas
Enlarged liver or spleen
Nerve damage in the hands and feet that leads to numbness (peripheral neuropathy)
Skin rash

Diagnosis Of Castleman Disease:

Castleman disease is diagnosed via the following tests and exams:

Physical examination

Blood and urine tests, to help rule out other infections or diseases. These tests can also reveal anemia and abnormalities in blood proteins that are sometimes characteristic of Castleman disease.

Imaging tests
MRI
PET
CT scan
Lymph node biopsy,to differentiate Castleman disease from other types of lymphatic tissue disorders, such as lymphoma.

Treatment Of Castleman Disease:

Treatment depends upon the type of castleman disease one has.

Unicentric castleman disease
Surgery to remove the diseased lymph nodes
Medications to shrink the diseased lymph nodes if surgery is not possible
Multicentric castleman disease
Monoclonoal antibodies, to block the action of the IL-6 protein that contributes to cell overgrowth.
Chemotherapy, to slow overgrowth of lymphatic cells.
Corticosteroids, to control inflammation.
Antiviral drugs,to block the activity of HHV-8 or HIV
Thalidomide (Thalomid),to block the action of the IL-6 protein.