Pulmonary involvement in patients with multiorgan Langerhans cell histiocytosis. Eight case studies and literature review
Authors:
Z. Adam 1; T. Nebeský 2; P. Szturz 1; J. Neubauer 3; M. Krejčí 1; L. Pour 1; I. Hanke 4; M. Doubková 5; Z. Merta 5; R. Hájek 1; Z. Řehák 3; R. Koukalová 3; J. Mayer 1
Authors place of work:
Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Mayer, CSc.
1; Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil A. Válek, CSc.
2; Oddělení nukleární medicíny a pozitronové emisní tomografie Masarykova onkologického ústavu Brno, přednosta prim. MUDr. Karol Bolčák
3; Chirurgická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Zdeněk Kala, CSc.
4; Klinika tuberkulózy a respiračních nemocí Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednostka prof. MUDr. Jana Skřičková, CSc.
5
Published in the journal:
Vnitř Lék 2010; 56(Supplementum 2): 105-122
Category:
Langerhans cell histiocytosis and some other Hematology rare diseases
Summary
Pulmonary Langerhans cell histiocytosis (LCH) manifests with dyspnoea and a cough with no significant expectoration; in some patients, spontaneous pneumothorax is the first symptom. The disease is caused by multiple granulomas in terminal bronchioles, detectable as nodules on high resolution CT (HRCT). During further course of the disease, these nodules progressed through cavitating nodules into thick-walled and, subsequently, thin-walled cysts. LCH may affect the lungs only or multiple organs simultaneously. Pulmonary LCH may continually progress or remit spontaneously. Treatment is indicated in patients in whom pulmonary lesions are associated with multi-system involvement or when a progression of the pulmonary lesions has been confirmed. Our centre has a register of 24 patients with LCH; pulmonary LCH had been diagnosed in 8 patients. The present paper provides information on the course of pulmonary LCH in the individual patients and HRCT images of the pulmonary lesions in these patients.
Key words:
Langerhans cell histiocytosis – pneumothorax – emphysema – 2-chlorodeoxyadenosine – cladribine
Zdroje
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Štítky
Diabetology Endocrinology Internal medicineČlánok vyšiel v časopise
Internal Medicine
2010 Číslo Supplementum 2
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