LCH

Langerhans cell histiocytosis (LCH) is a rare disease that involves 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.

Signs and Symptoms

LCH is part of a group of clinical syndromes called Histiocytosis, 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 signs and symptoms of LCH depend on where in the body the abnormal cells accumulate. They can cause damage by compressing and infiltrating normal tissue. LCH can affect any organ in the body, but it most commonly affects the bones, skin, and pituitary gland.

Diagnosis

Diagnosis is confirmed by biopsy, which shows histiocytes, eosinophils, neutrophils, and fibrosis. The histiocytes (Langerhans cells) may be identified by their characteristic appearance (reniform nuclei), and immunohistochemical markers such as CD1a and S100 protein.

Treatment

Treatment depends on the extent of disease. Some patients with single-system disease may not require any treatment, while others may require chemotherapy. For multisystem disease, chemotherapy is usually necessary.

Prognosis

The prognosis of LCH depends on the extent of disease, with single-system disease having a better prognosis than multisystem disease.

See Also

• Histiocytosis
• Dendritic cells
• Macrophages

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