What is hemophagocytic lymphohistiocytosis?
Hemophagocytic lymphohistiocytosis is a very rare disorder that can quickly become fatal, sometimes within weeks or days of onset. It is characterized by an extremely aggressive activity of the immune system in which certain types of white blood cells attack and destroy healthy tissues in the body. This disease can be genetic or acquired and is usually seen in infant or early childhood. Treatment options include drugs suppressing the immune system and chemotherapy to block the action of abnormal blood cells. In severe cases, patients may need to receive emergency bone marrow transplants.
The exact causes of haemofagocytic lymphohistiocytosis are not fully understood, but doctors have identified some risk factors. The genetic form of disorder is the result of mutations in one or more specific genes that control the production and effect of two types of blood cells: lymphocytes and histiocytes. The disease is autosomal recessive, which means that both parents must bear abnormal copies of genes to inherit their child. NicheA scanned form of hemophagocytic lymphohistiocytosis usually occurs after severe viral or bacterial infection. The immune system is unable to return to the normal level of activity once infectious pathogens are removed from the body.
lymphocytes and histiocytes usually help fight infections. In the case of hemophagocytic lymphohistiocytosis, however, cells respond to the tissues of a healthy body in the same way as pathogens. The result is organ inflammation, tissue death and destruction of important blood and bone marrow cells. Patients with disorder usually experience high fever, skin rashes and jaundice. Sleen, liver and other organs can increase and stop working properly. The brain can also be influenced, leading to irritability, mental confusion, seizures and perhaps comment or sudden.
doctors can diagnose hemopheric lymphistiocytosis by careful review of symptoms and perform a number of tests. Blood samples jSOU analyzed to control the abnormal number of white and red blood cells and for screening the presence of infections. Physical tests and imaging tests are useful in determining which authorities have been damaged and to what extent. Patients showing symptoms of hemopheric lymphoHistiocytosis are hospitalized and immediately treated.
The aim of treatment is to stop excessive activity of lymphocytes and histiocytes. Chemotherapy drugs and other immunosuppressants are often effective at the temporary closure of most of the immune system, giving organs and body tissues time to recover. The obtained form of disorder usually responds to medical care, but most patients who have genetic conditions eventually need bone marrow transplantation to prevent problems. By removing abnormal cells from the bone marrow and exchange of healthy stem cells, a large number of patients can recover permanently.