lymphoproliferation excessive numbers of lymphocytes, causing enlarged lymph nodes, liver and spleen, and sometimes affecting other organs. If lymphoproliferation is severe, giving immunosuppressant medications to dampen down (suppress) the immune system may be helpful. These medications reduce the overreaction and lessen the risk of tissue damage. Suppressing the immune system often involves courses of corticosteroids and chemotherapy medicines, usually given into a vein (intravenously) in hospital. T-cells (or T-lymphocytes) lymphocytes that develop in the thymus, an organ in the chest. They are important players in the immune response. macrophage a type of cell that engulfs and digests microbes, damaged or dead cells. They also take part in the anti-inflammatory response to decrease immune reactions. Natural Killer (NK) cells a type of lymphocyte particularly important in fighting viral infections and protecting against cancer.

In many cases, XLP1 is an inherited condition, meaning it is passed on in families in the same way that physical characteristics, such as eye colour, are passed from parent to child. It is caused by a mutation (change) in a child’s genetic make-up. Specialists in genetics and genetic counselling are on hand to talk through the inheritance of XLP1 with you if needed, and PID UK has a separate information leaflet devoted to the genetics of primary immunodeficiency. biopsy surgical removal of a small sample of tissue for examination under a microscope for diagnostic purposes. The aim is to give enough treatment to control the overactive lymphocytes without losing protection against infection, while avoiding other side effects. Some people affected by XLP1 are more likely to develop infections than normal and may benefit from regular (prophylactic) antibiotics and immunoglobulin replacement. ‘Live’ vaccines should be avoided. In many cases, an inactive form of a vaccine is available.

lymphocytes small white blood cells, normally present in the blood and in lymphoid tissue, that carry out the functions of the immune system. There are two major forms of lymphocytes, B-cells and T-cells, which have distinct but related functions in generating an immune response. immunosuppressants medications that suppress or ‘dampen down’ the immune system; used to suppress inflammation and autoimmunity.

gene a section of DNA on a chromosome that codes for a functional RNA molecule and thus a protein. Put another way, a word, rather than a letter, in the genetic code. Genes are the fundamental units of inheritance that carry the instructions for how the body grows and develops. white blood cells (leucocytes) a group of small, colourless blood cells that play a major role in the body’s immune system. There are five basic types of white blood cells: monocytes, lymphocytes, neutrophils, eosinophils and basophils. immunoglobulin replacement therapy administration of immunoglobulin purified from plasma to people with immune deficiency. The immunoglobulin contains antibodies that help protect against infection. This treatment can be given through a vein or under the skin. Please make sure all customers are aware of the suspension and freight is held until advised further. haematopoietic stem cell transplant (HSCT) the transfer of stem cells from a donor – either related or unrelated – to a recipient. Stem cells may be obtained from bone marrow (from the hip bone – this is also known as bone marrow transplantation), peripheral blood (PBSCs), or from stored umbilical cord blood. Haematopoietic means blood-forming. The donor cells are given by intravenous infusion and make their way to the recipient bone marrow to provide a new immune system, curing the immunodeficiency.The most important step is recognising that there is a problem with the immune system and involving the right specialists to investigate and treat further. Diagnosis depends on the recognition of suggestive clinical features, along with blood tests. There are many conditions that can cause similar symptoms and it is important that careful evaluation is performed to exclude any of these.