Scindeks Asistent Scindeks Asistent — sistem za ozbiljne časopise i one koji to žele da postanu
Vol. 73 Br. 1 (2016)
Vol. 73 Br. 1 (2016)

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

DOI: 10.2298/6740
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

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.

 

 

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DOI: 10.2298/6740

Reference

Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, et al. Erdheim-Chester disease. Curr Rheumatol Rep 2014; 16(4): 412.

Mazor RD, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester Disease: a comprehensive review of the literature. Orphanet J Rare Dis 2013; 8: 137.

Weitzman S, Jaffe R. Uncommon histiocytic disorders: the non-Langerhans cell histiocytoses. Pediatr Blood Cancer 2005; 45(3): 256−64.

Drier A, Haroche J, Savatovsky J, Godenèche G, Dormont D, Chiras J, et al. Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology 2010; 255(2): 586−94.

Lachenal F, Cotton F, Desmurs-Clavel H, Haroche J, Taillia H, Magy N, et al. Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. J Neurol 2006; 253(10): 1267−77.

Conley A, Manjila S, Guan H, Guthikonda M, Kupsky WJ, Mittal S. Non-Langerhans cell histiocytosis with isolated CNS involvement: an unusual variant of Erdheim-Chester disease. Neuropathology 2010; 30(6): 634−47.

Pineles SL, Liu GT, Acebes X, Arruga J, Nasta S, Glaser R, et al. Presence of Erdheim-Chester disease and Langerhans cell histiocytosis in the same patient: a report of 2 cases. J Neuroophthalmol 2011; 31(3): 217−23.

Saven A, Burian C. Cladribine activity in adult langerhans-cell histiocytosis. Blood 1999; 93(12): 4125−30.

Stine KC, Saylors RL, Williams LL, Becton DL. 2-Chlorodeoxyadenosine (2-CDA) for the treatment of refractory or recurrent Langerhans cell histiocytosis (LCH) in pediatric patients. Med Pediatr Oncol 1997; 29(4): 288−92.

Arnaud L, Hervier B, Néel A, Hamidou MA, Kahn JE, Wechsler B, et al. CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. Blood 2011; 117(10): 2778−82.

Alfieri A, Gazzeri R, Galarza M, Neroni M. Surgical treatment of intracranial Erdheim-Chester disease. J Clin Neurosci 2010; 17(12): 1489−92.

Myra C, Sloper L, Tighe PJ, McIntosh RS, Stevens SE, Gregson RH, et al. Treatment of Erdheim-Chester disease with cladribine: a rational approach. Br J Ophthalmol 2004; 88(6): 844−7.

Liliemark J. The clinical pharmacokinetics of cladribine. Clin Pharmacokinet 1997; 32(2): 120−31.

Mazor RD, Manevich-Mazor M, Shoenfeld Y. Strategies and treatment alternatives in the management of Erdheim–Chester disease. Expert Opin Orphan Drugs 2013; 1(11): 891−9.

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