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Letterer–Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. Oral cavity and gastrointestinal involvement may also be seen.
[8] [21] Shortly afterwards, Letterer in 1924 and Siwe in 1933 described a fatal condition in children who presented with large livers and spleens, large lymph nodes and bone damage. In 1940, Louis Litchtenstein and Henry L. Jaffe described a self-limiting disease characterised by "isolated bone lesions". [9]
Multifocal multisystem LCH, also called Letterer-Siwe disease, is an often rapidly progressing disease in which Langerhans cells proliferate in many tissues. It is mostly seen in children under age 2, and the prognosis is poor: even with aggressive chemotherapy, the five-year survival is only 50%.
The Histiocyte Society, a nonprofit organization, is a group of more than 200 physicians and scientists from around the world committed to improving the lives of patients with histiocytic disorders by conducting clinical and laboratory research into the causes and treatment of this disease. The Society has instituted several clinical trials and ...
Indeterminate cell histiocytosis (LCH) is an uncommon proliferative illness where the predominant cells have characteristics from both non-Langerhans cell histiocytosis (NLCH) and Langerhans cell histiocytosis (LCH) in terms of morphology and immunophenotypic characteristics. [1]
Langerhans cell sarcoma (LCS) is a rare form of malignant histiocytosis.It should not be confused with Langerhans cell histiocytosis, which is cytologically benign. [1] It can present most commonly in the skin and lymphatic tissue, but may also present in the lung, liver, and bone marrow.
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Treatment for these patients should focus on the underlying contributors. Additionally, treatment of the inflammation of HLH itself is often required. [citation needed] While optimal treatment of HLH is still being debated, current treatment regimes usually involve high dose corticosteroids, etoposide and cyclosporin.
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