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True histiocytic lymphoma

From Writing Group of the Histiocyte Society (Chu T, D'Angio GJ, Favara B, Ladisch S, Nebit M, Pritchard J). Lancet 1987;1:208-9, with permission.

From Writing Group of the Histiocyte Society (Chu T, D'Angio GJ, Favara B, Ladisch S, Nebit M, Pritchard J). Lancet 1987;1:208-9, with permission.

Table 22-4. Histologic, Histochemical, and Electron Microscopic Diagnosis of Langerhans Cell Histiocytosis

1. Presumptive diagnosis: light morphologic characteristics

2. Designated diagnosis a. Light morphologic features plus b. Two or more supplemental positive stains for

(1) Adenosine triphosphatase

(2) S-100 protein

(3) a-D-Mannosidase

(4) Peanut lectin

3. Definitive diagnosis a. Light morphologic characteristics plus b. Birbeck granules in the lesional cell with electron microscopy and/or c. Staining positive for CD1a antigen on the lesional cell

From Writing Group of the Histiocyte Society (Chu T, D'Angio GJ, Favara B, Ladisch S, Nebit M, Pritchard J). Lancet 1987;1:208-9, with permission.

The following organs are commonly involved in LCH: bone (pelvis, femur, ribs, skull, and orbit), skin, lymph nodes, bone marrow, lungs, hypothalamic pituitary axis, spleen, and liver. The basic lesion is a granuloma formed by histiocytes, granulocytes, and lymphocytes. Early in the course of the disease, the lesions are usually proliferative and locally destructive. Later, the lesions undergo necrosis. The damaged tissue is cleared by macrophages, which eventually undergo xanthomatous change, and fibrosis ensues. These lesions may no longer contain demonstrable Langerhans cells.

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