Vnitr Lek 2006, 52(4):355-370

Histiocytosis from Langerhans cell in adults - experience of one department and review of treatment alternatives

Z. Adam^1,*, J. Vaníček², P. Šlampa³, P. Čoupek³, I. Mareschova¹, J. Neubauer⁴, L. Babičková⁵, Z. Adamová⁶, M. Tomíška¹, M. Navrátil¹

¹ Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.

² Klinika zobrazovacích metod Lékařské fakulty MU a FN u sv. Anny, Brno, přednosta doc. MUDr. Petr Krupa, CSc.

³ Radioterapeutické oddělení Masarykova onkologického ústavu, Brno, přednosta doc. MUDr. Pavel Šlampa, CSc.

⁴ Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil Válek, CSc.

⁵ Klinika tuberkulózy a respiračních nemocní Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednostka doc. MUDr. Jana Skřičková, CSc.

⁶ Zdavotní středisko pro děti a dorost, Obilní trh č. 9, Brno, vedoucí MUDr. Zdenka Adamová

Thirteen patients with Langerhans cell histiocytosis (LCH) have been treated in hospital Brno Masaryk University during the last 15 years. In 4 cases of this total amount, the diagnosis was made in childhood and these young adults were referred to our department from Pediatric cancer center. In 9 cases, the diagnosis has been made in people elder than 18 years. The disease recidived in 3 patients with LCH diagnosed in childhood, in one case with neurodegenerative impairment of brain. In 4 patients from the total of 9 with LCH diagnosed in age over 18 years, the disease had aggressive course with several recidives. In one case it was pulmonal and multifocal osseal manifestation, in two cases multifocal osseal disease. Isolated pulmonal form of LCH was diagnosed only in one patient. By the first patient, high dose melphalan and etoposide with peripheral blood stem cell transplantation was performed after failure of vincristin and prednison therapy and failure of etoposide therapy. The first remission after this high dose therapy lasted only 2.5 years. For relapse second cycle of the same high dose therapy was administered, but the next remission was much shorter. By the two patients with multifocal recidives in bones 2-chlordeoxyadenosine was administered as initial therapy. These patients are followed up for more then 24 months and they are without relapse of this disease. The 2-chlordeoxyadenosine is very efficient in multifocal bone form of LCH and has potential to reach long remission. Therefore in a case of aggressive multifocal disease we would prefer 2-chlordeoxyadenosine therapy as therapy of the first choice.

Keywords: Langerhans cell histiocytosis; 2-chlordeoxyadenosine

Received: January 3, 2006; Accepted: January 31, 2006; Published: April 1, 2006  Show citation