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Langerhans Cell Histiocytosis

Abstract

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NORD is very grateful to Kenneth L. McClain, MD, PhD, Professor of Pediatrics, Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, for assistance in the preparation of this report.

Synonyms of Langerhans Cell Histiocytosis

  • Abt-Letterer-Siwe disease
  • eosinophilic granuloma
  • Hand-Schueller-Christian syndrome
  • Hashimoto-Pritzker syndrome
  • histiocytosis X
  • LCH
  • Letterer-Siwe disease
  • pure cutaneous histiocytosis
  • self-healing histiocytosis

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Langerhans cell histiocytosis (LCH) is a spectrum of rare disorders characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the various tissues and organs of the body (lesions). The lesions may include certain distinctive Langerhans cells involved in certain immune responses, as well as other white blood cells (e.g.,lymphocytes, monocytes, eosinophils). Associated symptoms and findings may vary from case to case, depending upon the specific tissues and organs affected and the extent of involvement. Most often the bone lesions are painful. Skin rashes may itch or cause painful ulcers especially under the arms or groin area. The pathogenesis (medical cause) is not clearly understood and an ongoing debate continues regarding its cause as a reactive immunologic or neoplastic (cancer-like) process. No infectious agent (virus, bacteria, or fungus) has been associated with LCH. Patients often have a strong family history of immune diseases such as thyroid disease, arthritis, or lupus.

Most affected individuals have single or multiple bone lesions characterized by lytic lesions (holes in the bones). Although the skull is most commonly affected, there may also be involvement of other bones, such as those of the spine (vertebrae) and the long bones of the arms and legs. Affected individuals may have no apparent symptoms (asymptomatic), or may experience associated pain and swelling, and/or develop certain complications, such as fractures or secondary compression of the spinal cord. Other organs may also be affected, including the skin, lungs, liver, spleen, bone marrow, thymus, thyroid,intestines and brain. In some individuals, LCH may be associated with involvement of the pituitary gland leading to diabetes insipidus, growth failure, hypothyroidism, or insufficitne production of sex hormones.

Langerhans cell histiocytosis was selected by the Histiocyte Society to replace the older, less specific term histiocytosis X. Histiocytosis X encompassed three entities known as eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease that were characterized by the accumulation of histiocytes. The "X" denoted that the cause and development of the disorder was not understood. Langerhans cell histiocytosis was chosen because it seemed that the Langerhans cells might play a central role in the development of these disorders. However, new research (Allen 2010) has shown that the skin Langerhans cell is not the cell of origin, but a myeloid dendritic cell.

Organizations related to Langerhans Cell Histiocytosis

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