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» Expert Histio Net » For Professionals » Guidelines » LCH adult » Organ involvement » Risk organs and special sites  · 

Risk organs and special sites

Author(s): Euro Histio Net Work Group for LCH Guidelines for adults (see introduction page), Created: 2013/07/08, last update: 2013/08/18

Possibly involved organs

After the diagnosis of LCH has been made, involvement of other organs should be evaluated and defined according to the clinical, biological or radiological criteria.

Risk organs (bone marrow, liver, spleen, CNS)

Involvement in the hematopoietic system (extremely rare in adults), spleen, liver or CNS indicates a less favorable prognosis, with possible mortality if the patient does not respond to therapy. Although this has never been proven for adults, retrospective analyses of national registries and the experts’ experience support the existence of the above mentioned “risk organs”.

Fever, night-sweats and weight loss combined with poor performance score might predict the rarely observed aggressive course of LCH in adults comparable to that of high grade non-Hodgkin lymphoma [Szturz et al 2010; Teng et al 2005]

“Special Sites” and “CNS-Risk” bone involvement

Vertebral lesions with intraspinal or cranofacial bone lesions with soft tissue extensions (orbit, mastoid, sphenoid or temporal bones) may cause immediate risk to the patient because of the critical anatomical site and the hazards of attempting local therapy. Isolated disease in these “Special Sites” justifies systemic therapy for children because of spinal cord compression and the association of cranio-facial bone lesions with an increased risk of developing diabetes insipidus [Grois et al 2006]. It is unclear if this connection might be extrapolated to adults, but most experts treating LCH patient follow the same guidelines for their adult patients as with the pediatric cases. (Grade D2 *)

* Grades of recommendation see here.


[Szturz et al 2010] Szturz P, Adam Z, Rehák Z, Koukalová R, Kodet R, Nebeský T, Neubauer J, Moulis M, Smardová L, Mayer J: [Lymphoma-like course in aggressive adult multisystem Langerhans cell histiocytosis and the benefit of PET/CT imaging in evaluation of diffuse metabolic activity of lung parenchyma]. [Article in Czech]. Vnitr Lek 2010, 56: 1177-93. [PMID: 21250497]

[Teng et al 2005] Teng CL, Lin TH, Young JH, Chou G, Young CS: Rapidly fatal Langerhans' cell histiocytosis in an adult. Journal of the Formosan Medical Association = Taiwan yi zhi 2005, 104: 955 [PMID: 16607456]

[Grois et al 2006] Grois N, Pötschger U, Prosch H, Minkov M, Arico M, Braier J, Henter JI, Janka-Schaub G, Ladisch S, Ritter J, Steiner M, Unger E, Gadner H, DALHX- and LCH I and II Study Committee: Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatric blood & cancer 2006, 46: 228 [PMID: 16047354]