Plasmapheresis Health Dictionary

A MALIGNANT disorder of PLASMA cells, derived from B-lymphocytes (see LYMPHOCYTE). In most patients the BONE MARROW is heavily in?ltrated with atypical, monoclonal plasma cells, which gradually replace the normal cell lines, inducing ANAEMIA, LEUCOPENIA, and THROMBOCYTOPENIA. Bone absorption occurs, producing di?use osteoporosis (see under BONE, DISORDERS OF). In some cases only part of the immunoglobulin molecule is produced by the tumour cells, appearing in the urine as Bence Jones PROTEINURIA.

The disease is rare under the age of 30, frequency increasing with age to peak between 60 and 70 years. There may be a long preclinical phase, sometimes as long as 25 years. When symptoms do occur, they tend to re?ect bone involvement, reduced immune function, renal failure, anaemia or hyperviscosity of the blood. Vertebral collapse is common, with nerve root pressure and reduced stature. The disease is eventually fatal, infection being a common cause of death. Local skeletal problems should be treated with RADIOTHERAPY, and the general disease with CHEMOTHERAPY

– chie?y the ALKYLATING AGENTS melphalan or cyclophosphamide. Red-blood-cell TRANSFUSION is usually required, together with plasmapheresis (see PLASMA EXCHANGE), and orthopaedic surgery may be necessary following fractures.... myelomatosis

Also known as plasmapheresis. The removal of the circulating PLASMA from the patient. It is done by removing blood from a patient and returning the red cells with a plasma expander. The plasma exchange is carried out through an in-dwelling CANNULA in the femoral vein, and the red cells and plasma are separated by a hemonetics separator. Usually a sequence of three or four sessions is undertaken, at each of which 2–3 litres of plasma are removed. The lost plasma can either be replaced by human serum albumin (see ALBUMINS) or a plasma expander.

In autoimmune disorders, disease is due to damage wrought by circulating ANTIBODIES or sensitised lymphocytes (see LYMPHOCYTE). If the disease is due to circulating humoral antibodies, removal of these antibodies from the body should theoretically relieve the disorder. This is the principle on which plasma exchange was used in the management of autoimmune diseases due to circulating antibodies. Such disorders include Goodpasture’s syndrome, SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) and MYASTHENIA GRAVIS. One of the problems in the use of plasma exchange in the treatment of such diseases is that the body responds to the removal of an antibody from the circulation by enhanced production of that antibody by the immune system. It is therefore necessary to suppress this homeostatic response with cytotoxic drugs such as AZATHIOPRINE. Nevertheless, remissions can be achieved in autoimmune diseases due to circulating antibodies by the process of plasma exchange.... plasma exchange

A procedure in which blood is withdrawn from a donor and is reinfused after one or more selected components have been separated and removed. In plasmapheresis, antibodies that are causing a disease are removed; and in leukapheresis, white blood cells are removed.... apheresis

A rare autoimmune disorder causing inflammation of the glomeruli in the kidney (see glomerulus) and the alveoli in the lungs, and anaemia. It is a serious disease; unless treated early it may lead to lifethreatening bleeding into the lungs and progressive kidney failure. The disease is most common in young men, but can develop at any age and in women. Sometimes, it responds to treatment with immunosuppressant drugs and plasmapheresis. People who have severe or repeated attacks require dialysis and, eventually, a kidney transplant.... goodpasture’s syndrome

A rare condition affecting the peripheral nerves (see peripheral nervous system) that causes weakness, usually in the limbs. The cause is believed to be an allergic reaction to an infection, usually viral; the nerves are damaged by antibodies produced by the body to eliminate the infection. In most cases, the disease develops 2 or 3 weeks after the onset of infection. Weakness, often accompanied by numbness and tingling, usually starts in the legs and spreads to the arms. The weakness may become progressively worse, resulting in paralysis. The muscles of the face and those controlling speech, swallowing, and breathing may also be affected.

Diagnosis of Guillain–Barré syndrome is confirmed by electrical tests to measure how fast nerve impulses are being conducted, or by a lumbar puncture. Most people recover fully with only supportive treatment. However, in severe cases, treatment with plasmapheresis or immunoglobulin may be given. Mechanical ventilation may be needed to aid breathing if the respiratory muscles and diaphragm are severely affected. Some people are left with permanent weakness in affected areas and/or suffer from further attacks of the disease.... guillain–barré syndrome

(TTP) a rare disorder of coagulation caused by deficiency or inhibition of *ADAMTS13, a protein that is responsible for breaking down von Willebrand factor (see von Willebrand’s disease). This results in haemolytic *anaemia, *thrombocytopenia, and fluctuating neurological abnormalities. It is treated by *plasmapheresis.... thrombotic thrombocytopenic purpura