Castleman Disease

Castleman Disease
Castleman Disease is a rare disease in which the body's
disease-fighting network (lymphatic system) contains
more than required cells (overgrowth of cells). It is also
known as giant lymph node hyperplasia and
angiofollicular lymph node hyperplasia. Castleman
disease can occur in a unicentric (localized) or
multicentric (widespread) form.
Multicentric Castleman disease can be life-threatening
because sometimes it is linked with HIV infection. It is
also associated with other cell-proliferation disorders,
including cancer of the lymphatic system
(lymphoma), Kaposi's sarcoma and POEMS syndrome.

Symptoms
Most patients suffering from unicentric Castleman
disease don't notice any signs or symptoms. The
affected lymph node is usually located in the chest,
neck or abdomen. Signs and symptoms include:
 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
 Unexpected weight loss
 Less commonly, fever, night sweats and weakness

Symptoms
Symptoms of multicentric Castleman disease
 Fever
 Night sweats
 Fatigue and weakness
 Loss of appetite
 Unexpected weight loss
 Enlarged lymph nodes, usually around the neck, collarbone,
underarm and groin areas
 Enlarged liver or spleen
 Skin rash
 Nerve damage in the hands and feet that leads to numbness
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Diagnosis of Castleman disease
If your doctor suspects unicentric or multicentric
Castleman disease, he is likely to do a physical
examination of your lymph nodes, to determine their size
and consistency. Your doctor may then recommend:
 Blood and urine tests: This is to help rule out the
possibility of other infections or diseases. These tests can
also reveal anemia and abnormalities in blood proteins.
 Imaging tests: They are performed to detect enlarged
lymph nodes, liver or spleen. Along with these, CT scan
or MRI of your neck, chest, abdomen and pelvis may be
used.
 Lymph node biopsy: It is done to differentiate Castleman
disease from other types of lymphatic tissue disorders,
such as lymphoma. A tissue sample from an enlarged
lymph node is removed and taken to a lab for testing.

Treatments
Unicentric Castleman disease: It can be cured by
surgically removing the diseased lymph node. A major operation
may be needed if the lymph node is in your chest or abdomen. If
surgical removal isn't possible, medicines may be used to shrink
the lymph node following a radiation therapy.
Multicentric Castleman disease: Normally, surgery is not the
right treatment for multicentric Castleman disease because of
the number of lymph nodes involved. However, surgery may be
required to remove an enlarged spleen. Treatment generally
involves medications and other therapies to manage cell
overgrowth including:
 Monoclonal 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 swelling.
 Antiviral drugs, to block the activity of HHV-8 or HIV if you have
one or both of those viruses.
 Thalidomide, to block the action of the IL-6 protein.

Treatments
A diagnosis of Castleman disease can be demanding
because the condition is uncommon and often occurs
with other serious illnesses, such as HIV/AIDS
and Kaposi's sarcoma.
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