Uspešna primena kladribina u lečenje Edhajm-Česterove bolesti sa zahvatanjem orbita i centralnog nervnog sistema nastale nakon lečenja Langerhansove histiocitoze

  • Predrag Perić Clinic for Neurosurgery, Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
  • Branislav Antić Clinic for Neurosurgery, Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
  • Slavica Knezević-Ušaj Department of Pathology, Faculty of Medicine, University of Novi Sad, Novi Sad, Serbia
  • Olga Radić-Tasić Institute for Pathology and Forensic Medicine, Military Medical Academy, Belgrade, Serbia
  • Sanja Radovinović-Tasić Institute for Radiology Military Medical Academy, Belgrade, Serbia
  • Jasenka Vasić-Vilić Institute for Radiology Military Medical Academy, Belgrade, Serbia
  • Leposava Sekulović Institute for Radiology Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
  • Olivera Tarabar Clinic for Hematology Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
  • Ljiljana Tukić Clinic for Hematology Military Medical Academy, Belgrade, Serbia; †Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
  • Stevo Jovandić Institute for Medical Research, Military Medical Academy, Belgrade, Serbia
  • Zvonko Magić Institute for Medical Research, Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
DOI: https://doi.org/10.2298/6740
Ključne reči: erdheim-chester disease||, ||erdheim-chesterova bolest, histiocytosis, non-langerhans cells||, ||histiocitoza, non-langerhans ćelije, orbital pseudotumor||, ||pseudotumor orbite, central nervous system||, ||centralni nervni sistem, brain stem||, ||moždano stablo, cerebellum||, ||mozak, mali, proto-oncogene proteins b-raf||, ||proteini, protoonkogeni, b-raf, cladribine||, ||kladribin, magnetic resonance imaging||, ||magnetna rezonanca, snimanje,

Sažetak


Uvod. Edhajm-Česterova bolest (EČB) je redak sistemski oblik ne-Langerhansove histiocitoze iz porodice juvenilnih ksantogranuloma, koga karakteriše simetrična bilateralna osteoskleroza dugih kostiju sa ksantogranulomskom infiltracijom različitih organa. Kod EČB često su prisutne lezije centralnog nervnog sistema (CNS) i orbita, a više od polovine bolesnika sa ovom bolesti ima V600E mutaciju protoonkogena BRAF. Sinhroni ili metahroni razvoj EČB i Langerhansove histiocitoze (LH) kod istog bolesnika je redak, a moguća veza između njih još uvek je nejasna. Kladribin je purinski analog koji deluje toksično na limfocite i monocite sa dobrom hematoencefalnom penetracijom. Prikaz bolesnika. Prikazali smo muškarca, starog 23 godine, koji je uspešno lečen kladribinom zbog EČB sa obostranim zahvatanjem orbita i CNS. Kod prikazanog bolesnika BRAF V600E mutacija nije bila prisutna, a EČB je nastala metahrono, šest godina nakon hemioterapijskog lečenja multisistemske LH, sa kompletnom remisijom bolesti i zaostajanjem centralnog insipidnog dijabetesa. Tokom lečenja EČB, bolesnik je dobio pet ciklusa monohemioterapije kladribinom (5 mg/m2 tokom pet dana, svake 4 nedelje), uz redukciju doze na 4 mg/m2 u petom odloženom ciklusu zbog izražene neutropenije i pojave herpes-zoster infekcije po okončanju četvrtog ciklusa. Rezolucija radiološki viđenih sistemskih i CNS lezija počela je nakon tri meseca, uz potpuni nestanak lezija u CNS unutar dve godine od završetka hemioterapije. Bolesnik je periodično praćen tokom 12 godina, pri čemu nije uočena pojava relapsa bolesti kao ni sekundarnog maligniteta. Zaključak. U odnosu na naše prikazano iskustvo i preporuke drugih autora, kladribin se može razmatrati kao efikasna alternativna terapija za EČB, naročito kod bolesnika sa lezijama u CNS i odsustvom BRAF V600E mutacije, kada je primena interferona α kao leka prvog izbora bila neuspešna.

 

 

Biografija autora

Predrag Perić, Clinic for Neurosurgery, Military Medical Academy, Belgrade, Serbia; Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia

Doc. dr sc. med.

specijalista neurohirurgije

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Objavljeno
2017/01/23
Broj časopisa
Vol. 73 Br. 1 (2016)
Rubrika
Prikaz bolesnika