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Question

Please may I have some information about Evans' syndrome and lymphoproliferative disease? Why would someone prescribe steroids to treat these conditions?

Answer

Evans' syndrome is the term used to describe a situation in which there is immune mediated destruction of both red blood cells and platelets. By immune mediated we mean that the body effectively starts to behave as though its own cells are foreign - in this case red blood cells and platelets - and produces antibodies to these. Antibodies are substances which are produced in response to antigens - which are usually foreign substances regarded as dangerous by the body. In the case of Evans' syndrome, there are separate antibodies produced to each type of blood cell.

The results of this are that the body is breaking down essential components of the blood which causes serious complications and in its most severe form can lead to death.

The symptoms and signs are fever and abdominal pain in some people. Thrombosis, or clots in the veins can occur, usually in the deep veins of the legs. Thrombosis of the arteries can also occur. Some people have an enlarged liver.

The cause of this syndrome is generally not known, although some drugs such as methyldopa, penicillin type drugs and those like quinidine, can cause a similar picture.

Steroids are used to treat this because they damp down the action of the immune system and it is the immune system which is causing the problem.

Lymphoproliferative disorders are any in which the lymphocyte portion of the blood and bone marrow is produced in excess, such as lymphocytic leukaemia and the lymphomas (see Lymphoma and Leukaemia in the Cancer section). There is also something called X-linked lymphoproliferative syndrome in which people cannot eliminate the Epstein-Barr virus, which causes glandular fever. In men with this condition, inherited through the mother, glandular fever can lead to serious complications and even death.

MORE BLOOD DISEASE Q&A'S

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