Clinical presentations of the most common histiocytic disorders
Authors:
prof. MUDr. Adam Zdeněk, CSc. 1; MUDr. Eid Michal, Ph.D. 1,2; doc. MUDr. Řehák Zdeněk, Ph.D. 3; MUDr. Kubeš Václav, Ph.D. 4; doc. MUDr. Horváth Teodor, Ph.D. 5; doc. MUDr. Doubková Martina, Ph.D. 6; MUDr. Starý Karel 7; prof. MUDr. Šlampa Pavel, CSc. 8; MUDr. Čermák Aleš, Ph.D. 9; Doc. MUDr. Fadrus Pavel, Ph.D. 10; MUDr. Adamová Zuzana, Ph.D. 11; doc. MUDr. Vaníček Jiří, Ph.D. 12; MUDr. Nebeský Tomáš 13; MUDr. Kamarádová Kateřina, Ph.D. 14; MUDr. Boichuk Ivanna 1; prof. MUDr. Pour Luděk, Ph.D. 1
Authors‘ workplace:
Interní hematologická a onkologická klinika LF MU a FN Brno
1; Centrum precizní medicíny, FN Brno
2; Ústav nukleární medicíny, MOÚ Brno
3; Ústav patologie, LF MU a FN Brno
4; Chirurgická klinika LF MU a FN Brno
5; Klinika nemocí plicních a tuberkulózy LF MU a FN Brno
6; Interní gastroenterologická klinika LF MU a FN Brno
7; Klinika radiační onkologie, MOÚ Brno
8; Urologické klinika LF MU a FN Brno
9; Neurochirurgická klinika LF MU a FN Brno
10; Chirurgické oddělení, Moravskoslezská nemocnice Frýdek-Místek
11; Klinika zobrazovacích metod LF MU a FN u sv. Anny v Brně
12; Klinika radiologie a nukleární medicíny LF MU a Brno
13; Fingerlandův ústav patologie, LF UK v Hradci Králové
14
Published in:
Klin Onkol 2026; 39(3): 150-168
Category:
Review
doi:
https://doi.org/10.48095/ccko2026150
Overview
Background: Histiocytic diseases are significantly rarer than diseases derived from lymphocytic, plasmacytic, or myeloid lineages, and thus are encountered infrequently in hematology and oncology clinics. The most common form is Langerhans cell histiocytosis, which in adults has an incidence of 1–2 cases per 1 million; the others are considerably rarer, with their occurrence reported only by the number of described cases rather than through incidence or prevalence. Their rarity leads to delays in establishing an accurate diagnosis. Objective: The group of histiocytic diseases includes seven clinical units: Langerhans cell histiocytosis, indeterminate dendritic cell histiocytosis, diseases from the juvenile xanthogranuloma group, Erdheim-Chester disease, Rosai-Dorfman disease, ALK-positive histiocytosis, and histiocytic sarcoma. Each of the described diseases has specific manifestations that distinguish it from the manifestations of other malignant blood disorders. The aim of this article is to remind the reader of these manifestations through images and text, thereby contributing to the timely recognition of these rare diseases. Conclusion: Treatment procedures are rapidly evolving, but the clinical presentations of these diseases remain unchanged. The disease profiles presented in this publication should aid in their early diagnosis and consequently in timely treatment.
Keywords:
histiocytosis
Sources
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2026 Issue 3
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