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.
2. INTRODUCTION TO 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.
3. SYMPTOMS OF CASTLEMAN DISEASE
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
4. 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
SYMPTOMS OF MULTICENTRIC
CASTLEMAN DISEASE
5. DIAGNOSIS OF CASTLEMAN DISEASE
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.
6. 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:
TREATMENT OF CASTLEMAN DISEASE
7. CONTINUE :- TREATMENT OF
CASTLEMAN DISEASE
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.
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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