Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Clinically, its manifestations range from isolated bone lesions to multisystem disease.
LCH is part of a group of clinical syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes (an archaic term for activated dendritic cells and macrophages). These diseases are related to other forms of abnormal proliferation of white blood cells, such as leukemias and lymphomas.
The disease had gone by several names, including Hans-Schüller-Christian disease, Letterer-Siwe disease, and histiocytosis X, until it was renamed in 1985 by the Histiocyte Society.[1]
The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells, hence sometimes called dendritic cell histiocytosis. These cells in combination with lymphocytes, eosinophils, and normal histiocytes form typical LCH lesions that can be found in almost any organ.[2]
There are three types of histiocytoses: malignant (true histiocytic lymphomas), “reactive” (benign histiocytoses), Langerhans cell histiocytosis.[3]
“Reactive” in this context indicates that the abnormality may be due to a physiological reaction to infection. For example leukocytosis (proliferation of white blood cells) is a normal reaction to infection, and “histiocytes” are developmentally related to white blood cells.
LCH is traditionally divided into three groups: unifocal, multifocal unisystem, and multifocal multisystem.[3]
Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease. It was once considered a benign condition in adults, but long term complications like pulmonary hypertension are becoming increasingly recognized.
[tubepress mode=’tag’, tagValue=’Langerhans cell histiocytosis’]