Pulmonary Histiocytosis X (Pulmonary Langerhans’ Cell Granulomatosis)

  • Samuel P. Hammar


A conceptual understanding of pulmonary histiocytosis X, also known as pulmonary eosinophilic granuloma and pulmonary Langerhans’ cell granulomatosis, is based on a knowledge of the Langerhans’ cell. The Langerhans’ cell was named after Paul Langerhans, a 24-year-old German medical student who identified it as a dendritic cell of the epidermis of uncertain origin and function.1 He thought these cells represented nerve cells. In 1961, Birbeck et al.2 discovered by electron microscopy that Langerhans’ cells contained unique rod- and racquet-shaped granules that are now referred to as Langerhans’ cell granules or Birbeck granules. In 1965, Basset and Turiaf3 found that histiocytosis X cells contained the same type of unusual cytoplasmic granule that was found in Langerhans’ cells, and called them histiocytosis X bodies or X bodies. Langerhans’ cells and histiocytosis X cells are bone marrow derived4,5 and have features similar but not identical to those of monocytes and macrophages6–13; these are shown in Table 17-1. More recent studies have shown that Langerhans’ cells belong to a specific subset of macrophages, termed dendritic cells or antigen-presenting cells.14,15 In 1986, Murphy and coworkers16 demonstrated, in skin biopsies from patients who had undergone bone marrow transplantation, that phagocytic dermal macrophages transform into Langerhans’ cells. Langerhans’ cells are stated to reach normal levels 4 to 12 months after transplantation.17


Cell Granule Alveolar Macrophage Idiopathic Pulmonary Fibrosis Eosinophilic Granuloma Open Lung Biopsy 
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© Springer Science+Business Media New York 1994

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  • Samuel P. Hammar

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