abb-entrelec XLP1 – 6BC – desconectador Fuse 3 Poles

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lymphoproliferation excessive numbers of lymphocytes, causing enlarged lymph nodes, liver and spleen, and sometimes affecting other organs. 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.

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. Natural Killer (NK) cells a type of lymphocyte particularly important in fighting viral infections and protecting against cancer. 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. Please make sure all customers are aware of the suspension and freight is held until advised further.

immunosuppressants medications that suppress or ‘dampen down’ the immune system; used to suppress inflammation and autoimmunity. 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.

biopsy surgical removal of a small sample of tissue for examination under a microscope for diagnostic purposes. 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.

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. 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.