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Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Symptoms range from isolated bone lesions to multisystem disease . [ 1 ]
Before the Histiocyte Society classified histiocytoses in the 1980s, the condition was also known as "Histiocytosis X", where "X" denoted the then unknown cause. [9] [10] [11] It is now known as chronic multifocal Langerhans cell histiocytosis, a subtype of LCH. [3] The disease is rare. [12] Most present between the ages of two and six. [2]
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.
non-Langerhans-cell histiocytosis Hemophagocytic lymphohistiocytosis (HLH) II: D76.1: non-Langerhans-cell histiocytosis Niemann–Pick disease: II: E75.2: non-Langerhans-cell histiocytosis Sea-blue histiocytosis: II – non-Langerhans-cell histiocytosis Acute monocytic leukemia: III: C93.0: malignant histiocytic disorders Malignant ...
Non-Langerhans cell histiocytosis, also known as rare histiocytoses, comprise all histiocyte, macrophage, and dendritic cell proliferative disorders that are not categorized as hemophagocytic lymphohistiocytosis (HLH) or Langerhans cell histiocytosis (LCH). [1] The spectrum of non-langerhans cell histiocytoses include: Benign cephalic histiocytosis
In the rare disease Langerhans cell histiocytosis (LCH), an excess of cells similar to these cells are produced. However LCH cells stain positive to CD14 which is a monocyte marker and shows a different, hematopoietic origin for the disorder. [12] LCH can cause damage to skin, bone and other organs. [citation needed]
Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis). It was declared a histiocytic neoplasm by the World Health Organization in 2016. [1]
First described in 1961 (where they were simply termed "characteristic granules"), [2] they are solely found in Langerhans cells. [1] Although part of normal Langerhans cell histology, they also provide a mechanism to differentiate Langerhans cell histiocytoses (which are a group of rare conditions collectively known as histiocytoses ) from ...