Different courses of recurrent or multisystem Langerhans cells histiocytosis in adults – description of 22 cases from one centre

Authors: Z. Adam 1;  M. Krejčí 1;  L. Pour 1;  P. Szturz 1;  J. Neubauer 2;  T. Nebeský 2;  Z. Řehák 3;  R. Koukalová 3;  J. Mayer 1;  J. Vorlíček 1
Authors‘ workplace: Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Jiří Vorlíček, CSc. 2Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Vlastimil A. Válek, CSc. 3Odd 1
Published in: Vnitř Lék 2010; 56(6): 542-556
Category: 65th Birthday - Petr Svacina, MD


Since its establishment in 1990, a total of 22 patients with confirmed Langerhans cell histiocytosis (LCH) have been monitored and treated at the Clinic of Internal Medicine Haemato-Oncology in Brno. In 5 patients, the disease was diagnosed in childhood and 2 of these 5 patients had late neurodegenerative changes in the CNS with a typical picture on MR and a typical PET-CT imaging fluorodeoxyglucose hypometabolism in the cerebellar area. In 5 patients from the cohort of 22, the disease had unifocal form, dominant in the area of skeleton with no recurrence after the treatment. However, in 12 patients, the disease affected a number of organs simultaneously (multifocal form of LCH). The aim of the description below is to characterise the monitored cohort of 22 patients and describe the very different courses of multifocal forms of LCH in 12 patients.

Key words:
Langerhans cell histiocytosis – diabetes insipidus – panhypopituitarizm – 2-chlorodeoxyadenosine – high-dose chemotherapy


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