Pulmonary histiocytosis X

Theodore W. Marcy, Herbert Y. Reynolds

Research output: Contribution to journalReview article

29 Citations (Scopus)

Abstract

Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion, and, occasionally, chest pain from a pneumothorax or bone involvement. However, it is not unusual for patients to be asymptomatic with only a diffuse interstitial infiltrate on their chest radiograph. The course is unpredictable and can range from spontaneous remission to progressive respiratory insufficiency and death. No therapy, beyond symptomatic and supportive care, has been shown to be effective. Pulmonary histiocytosis X appears to be a variation of the more disseminated histiocytosis X disorders, such as Letterer-Siwe disease and the Hand-Schüller-Christian syndrome. All of the histiocytosis X disorders have a characteristic histiocyte on pathologic study, with a unique ultrastructural organelle. Recent work suggests that this histiocyte is similar to macrophages found in the skin and some lymphoid organs. The histiocytosis X disorders may represent an abnormal response of the mononuclear phagocyte system to an antigen, to an immunodeficiency state, or both.

Original languageEnglish (US)
Pages (from-to)129-150
Number of pages22
JournalLung
Volume163
Issue number1
DOIs
StatePublished - Dec 1 1985

Fingerprint

Langerhans Cell Histiocytosis
Histiocytes
Spontaneous Remission
Mononuclear Phagocyte System
Pneumothorax
Chest Pain
Cough
Respiratory Insufficiency
Organelles
Dyspnea
Thorax
Hand
Macrophages
Antigens
Bone and Bones
Lung
Skin

All Science Journal Classification (ASJC) codes

  • Pulmonary and Respiratory Medicine

Cite this

Marcy, T. W., & Reynolds, H. Y. (1985). Pulmonary histiocytosis X. Lung, 163(1), 129-150. https://doi.org/10.1007/BF02713816
Marcy, Theodore W. ; Reynolds, Herbert Y. / Pulmonary histiocytosis X. In: Lung. 1985 ; Vol. 163, No. 1. pp. 129-150.
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Marcy, TW & Reynolds, HY 1985, 'Pulmonary histiocytosis X', Lung, vol. 163, no. 1, pp. 129-150. https://doi.org/10.1007/BF02713816

Pulmonary histiocytosis X. / Marcy, Theodore W.; Reynolds, Herbert Y.

In: Lung, Vol. 163, No. 1, 01.12.1985, p. 129-150.

Research output: Contribution to journalReview article

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AB - Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion, and, occasionally, chest pain from a pneumothorax or bone involvement. However, it is not unusual for patients to be asymptomatic with only a diffuse interstitial infiltrate on their chest radiograph. The course is unpredictable and can range from spontaneous remission to progressive respiratory insufficiency and death. No therapy, beyond symptomatic and supportive care, has been shown to be effective. Pulmonary histiocytosis X appears to be a variation of the more disseminated histiocytosis X disorders, such as Letterer-Siwe disease and the Hand-Schüller-Christian syndrome. All of the histiocytosis X disorders have a characteristic histiocyte on pathologic study, with a unique ultrastructural organelle. Recent work suggests that this histiocyte is similar to macrophages found in the skin and some lymphoid organs. The histiocytosis X disorders may represent an abnormal response of the mononuclear phagocyte system to an antigen, to an immunodeficiency state, or both.

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Marcy TW, Reynolds HY. Pulmonary histiocytosis X. Lung. 1985 Dec 1;163(1):129-150. https://doi.org/10.1007/BF02713816