Langerhans cell histiocytosis: Description, Causes and Risk Factors:Abbreviation: LCH.Langerhans cell histiocytosis is a rare disease that occurs when normal Langerhans cells lose their ability to fight infection and instead group together and destroy healthy tissue.Normal Langerhans cells are cells that are part of our body's infection fighting defense system. These Langerhans cells are usually found in body tissues such as the skin, lymph nodes, and spleen. These cells act like watchdogs, constantly on the look out for anything abnormal. If the body is invaded by germs (like bacteria or viruses), the Langerhans cells “sniff out” the invader and move through a series of orderly steps to alert other cells in the immune system to begin fighting the infection.Both children and adults can develop Langerhans cell histiocytosis. Approximately 1 in every 200,000 children younger than 15 years old will be diagnosed with Langerhans cell histiocytosis each year. “In children younger than 2 years old, Langerhans cell histiocytosis usually affects multiple parts of the body and causes the child to become very ill. In older children, Langerhans cell histiocytosis is typically milder and may cause only bone or skin lesions.Langerhans cell histiocytosis develops when normal Langerhans cells become overactive, lose control, and do not follow their usual orderly routine. The cells are stimulated to begin their defense work, but instead of going through their normal steps of gathering information and activating other cells in the immune system, these abnormal Langerhans cells cluster together like an unruly mob. This cluster of abnormal cells results in tumor-like lesions that begin to destroy normal tissues in the bones, skin, or other body systems. Scientists are not sure exactly what causes the cells' abnormal behavior, but research shows that infections or problems in the immune system may contribute.Langerhans cell histiocytosis can infiltrate all organs: Liver, spleen, gastrointestinal tract, central nervous systems, bone.Langerhans cell histiocytosis originate in the bone marrow and are derived from CD34-positive stem cells. Experimental argument plays to consider as monocyte as a precursor of Langerhans cell histiocytosis. They expressed certain numbers of surface phenotypic markers, among which the most important are the major histocompatibility complex class II molecules and antigen CD1a. With their dendrites, they cover 25% of the entire cutaneous surface, even though they represent only 5% of the dermal cells.From the skin they migrate via the lymphatics to the paracortical zones of lymph nodes to assure their function.Langerhans cell histiocytosis could arise secondary to a somatic mutation of a gene with, as a consequence, clonal proliferation of the cells of Langerhans cell histiocytosis. The absence of parallelism between the severity of the disease and monoclonality is quite remarkable, albeit difficult to interpret.The cells of Langerhans cell histiocytosis are able to synthesize a number of cytokines including interleukin 1(IL-1), tumor necrosis factor alpha (TNF-) and granulocyte/macrophage-colony-stimulating factor (GM-CSF), which can explain certain systemic signs of the disease for example fever and cachexia.Symptoms:Symptoms of Langerhans cell histiocytosis depend on which tissues and organs it affects. Not all children with the disease have the same symptoms.Basic signs of illness, such as fever, fatigue and weakness
Pain, swelling or lump in a bone that does not go away, such as on the skin, arms or legs.
Bone fracture for no clear reason or from only minor trauma.
Loose teeth when you would not expect this, or swollen gums.
Ear infection, cysts in the ear or fluid that oozes from the ear.
Failure to thrive, which means not gaining weight and growing normally.
Not wanting to eat or having problems feeding.
Needing to urinate more often than normal and being very thirsty (signs of a health problem called diabetes insipidus).
Diagnosis:The diagnosis is always made based on histological or cytological examination. Examination of the fine needle biopsy can sufficient for the diagnosis. The cells of Langerhans cell histiocytosis are large with an excentric, kidney-shaped, or coffee-bean nucleus.A confirm diagnosis can only be made if the cells are labeled with anti-CD1a antibodies. This study can be conducted on frozen cytological samples.Other tests May include:Complete Blood Count: A complete blood count (CBC) is a type of blood test that is useful indetecting a drop in the number of red blood cells (oxygen carriers),white blood cells (infection fighters), or platelets (cells that help bloodclot properly). Blood chemistries such as BUN (blood urea nitrogen) andcreatinine monitor changes in kidney function. Other chemistries suchas ALT (alanine aminotransferase), AST (aspartate aminotransferase), andbilirubin may be done to assess liver function.
X rays: Your child will need X rays to determine which bones are affected byLangerhans cell histiocytosis. Abone surveyorskeletal survey,which X-rays all the bones in yourchild's body from head to toe, is the most common X-ray used to detectLangerhans cell histiocytosis. If Langerhans cell histiocytosis is suspected in a bone, the area is usually described aslytic,which means that some of the bone has been eaten away by the abnormal Langerhans cells.
Treatment:Treatment Options May Include:Steroids: Steroid medication may be injected into the places with excess Langerhans cells.
Chemotherapy: Chemotherapy medicines used to kill cancer cells may also help control Langerhans cells. Doctors give some types of these medicines through a vein (intravenously, or by IV). For skin problems, they may apply medicines right on the skin. The doses are lower than used for cancer.
Radiation: This treatment can help control cells that are damaging bone and making it weak. The doses are lower than for cancer.
Surgery: Doctors may operate to remove the excess Langerhans cells (used mostly for bones).
Chemotherapy and corticosteroids used to treat Langerhans cell histiocytosis may cause side effects, and your child's doctor will discuss these with you before treatment starts. Side effects of chemotherapy can include feeling sick (nausea) and vomiting, hair loss, an increased risk of bruising and bleeding, and tiredness. Corticosteroids may cause a rounding of the face (sometimes called a moon face), mood changes and an increased appetite, which can lead to weight gain. All of these side effects are reversible when treatment stops.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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