Description Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin , lymph nodes, spleen , lungs, liver , and bone marrow. In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. Many researchers now consider Langerhans cell histiocytosis to be a form of cancer, but this classification remains controversial.
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Description Langerhans cell histiocytosis is a disorder in which excess immune system cells called Langerhans cells build up in the body. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin , lymph nodes, spleen , lungs, liver , and bone marrow.
In Langerhans cell histiocytosis, excess immature Langerhans cells usually form tumors called granulomas. Many researchers now consider Langerhans cell histiocytosis to be a form of cancer, but this classification remains controversial. In approximately 80 percent of affected individuals, one or more granulomas develop in the bones, causing pain and swelling.
The granulomas, which usually occur in the skull or the long bones of the arms or legs, may cause the bone to fracture. Granulomas also frequently occur in the skin, appearing as blisters, reddish bumps, or rashes which can be mild to severe.
The pituitary gland may also be affected; this gland is located at the base of the brain and produces hormones that control many important body functions. Without hormone supplementation, affected individuals may experience delayed or absent puberty or an inability to have children infertility.
In addition, pituitary gland damage may result in the production of excessive amounts of urine diabetes insipidus and dysfunction of another gland called the thyroid. Thyroid dysfunction can affect the rate of chemical reactions in the body metabolism , body temperature, skin and hair texture, and behavior.
In 15 to 20 percent of cases, Langerhans cell histiocytosis affects the lungs, liver, or blood-forming hematopoietic system; damage to these organs and tissues may be life-threatening. Lung involvement , which appears as swelling of the small airways bronchioles and blood vessels of the lungs, results in stiffening of the lung tissue, breathing problems, and increased risk of infection.
Hematopoietic involvement, which occurs when the Langerhans cells crowd out blood-forming cells in the bone marrow , leads to a general reduction in the number of blood cells pancytopenia. Pancytopenia results in fatigue due to low numbers of red blood cells anemia , frequent infections due to low numbers of white blood cells neutropenia , and clotting problems due to low numbers of platelets thrombocytopenia.
Other signs and symptoms that may occur in Langerhans cell histiocytosis, depending on which organs and tissues have Langerhans cell deposits, include swollen lymph nodes, abdominal pain, yellowing of the skin and whites of the eyes jaundice , delayed puberty, protruding eyes, dizziness, irritability, and seizures.
About 1 in 50 affected individuals experience deterioration of neurological function neurodegeneration. Langerhans cell histiocytosis is often diagnosed in childhood, usually between ages 2 and 3, but can appear at any age.
Most individuals with adult-onset Langerhans cell histiocytosis are current or past smokers; in about two-thirds of adult-onset cases the disorder affects only the lungs. The severity of Langerhans cell histiocytosis, and its signs and symptoms, vary widely among affected individuals. Certain presentations or forms of the disorder were formerly considered to be separate diseases. In many people with Langerhans cell histiocytosis, the disorder eventually goes away with appropriate treatment.
It may even disappear on its own, especially if the disease occurs only in the skin. However, some complications of the condition, such as diabetes insipidus or other effects of tissue and organ damage, may be permanent. Related Information.
Mubei Introduction The preferred name for the condition is Langerhns cell histiocytosis because the cell of origin LC is now known. Histicitosis tried to diagnose Langerhans cell histiocytosis in a boy with a previously diagnosed osteosarcoma. Bone and Lung tumor chapters. Langerhans cell histiocytosis LCH is an inflammatory myeloid neoplasia caused by alterations mutations of several genes in the MAPKinase pathway. Together we are strong. Diagnosis is confirmed histologically by tissue biopsy. This is a privacy protected site that provides up-to-date information for laangerhans interested in the latest scientific news, trials, and treatments related to rare lung diseases.
HISTIOCITOSIS NO LANGERHANS PDF
An even more mature dendritic cell carrying this mutation may go to only skin and bone. The Journal of Clinical Endocrinology and Metabolism. Sign up for our Email Newsletters. If treatment is needed, hydroxyurea alone or with methotrexate is very effective. Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma.
HISTIOCITOSIS NO LANGERHANS PDF
Molrajas If only skin lesions are present and not extensive, treatment may not be necessary. Page views in Journal of Clinical Pathology. Seen mostly in children, multifocal unisystem LCH is characterized by fever, bone lesions and diffuse eruptions, usually on the scalp and in the ear canals. Badalian-Very, Berres, Chakrobarty The cell of origin is a white blood cell known as a dendritic cell, although the historic designation of histiocyte is still used.