n INTRODUCTION Hearing that your child has a disease is shocking and overwhelming. At first, you may not believe it, or you may hope that the diagnosis is wrong. However, the reality of the situation will be confirmed as you see the changes in your child and experience being in the hospital and beginning treatment. Educating yourself on your child’s diagnosis, treatment plan, and possible side effects, and familiarizing yourself with the members of your child’s healthcare team and resources available, may help ease some of these feelings. n WHAT IS LANGERHANS CELL HISTIOCYTOSIS? Langerhans cell histiocytosis is a rare disease that occurs when normal Langerhans cells lose their ability to fight infection and instead group together to destroy healthy tissue. Langerhans cell histiocytosis has been known by many names, including histiocytosis X, malignant histiocytosis syndrome (now known as T cell lymphoma), eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, self- healing histiocytosis, Langerhans cell granulomatosis, and Type II histiocytosis. To prevent confusion, researchers and healthcare professionals have agreed to use one name to refer to this disease: Langerhans cell histiocytosis (LCH). n WHAT ARE NORMAL LANGERHANS CELLS? Normal Langerhans cells 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 lookout 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. n WHAT CAUSES LANGERHANS CELL HISTIOCYTOSIS? LCH 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 to the start of this process.
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