Histiocitoza moždanog tkiva sa preranim pubertetom i deficitom hormona rasta u ranom detinjstvu

  • Dragan Katanić University of Novi Sad, Faculty of Medicine, Institute for Child and Youth Health Care of Vojvodina, Novi Sad, Serbia
  • Jovanka Kolarović University of Novi Sad, Faculty of Medicine, I ns titute for Child and Youth Health Care of Vojvodina, Novi Sad, Serbia
  • Danica Grujičić Clinical Centre of Serbia, Neurosurgery Clinic, Belgrade, Serbia
  • Milica Skender Gazibara University of Belgrade, Faculty of Medicine, Institute of Pathology, Belgrade, Serbia
  • Marija Knežević Pogančev University of Novi Sad, Faculty of Medicine, Institute for Child and Youth Health Care of Vojvodina, Novi Sad, Serbia
  • Katarina Koprivšek University of Novi Sad, Faculty of Medicine, Institute of Oncology – Sremska Kamenica, Novi Sad, Serbia
  • Jovan Vlaški University of Novi Sad, Faculty of Medicine, Institute for Child and Youth Health Care of Vojvodina, Novi Sad, Serbia
  • Ivana Vorgučin University of Novi Sad, Faculty of Medicine, Institute for Child and Youth Health Care of Vojvodina, Novi Sad, Serbia
  • Jasmina Katanić University of Novi Sad, Faculty of Medicine, Institute for Child and Youth Health Care of Vojvodina, Novi Sad, Serbia
DOI: https://doi.org/10.2298/VSP1702080028K
Ključne reči: histocitoza, mozak, dijagnoza, dijabetes inspidus, lečenje lekovima, lečenje, ishod

Sažetak


Uvod. Histiocitoza Langerhans-ovih ćelija (LCH) je retko hronično granulomatozno multisitemsko oboljenje sporog toka i nejasne etiologije, sa najvišom incidencom u ranom detinjstvu. Izolovana histiocitoza moždanog tkiva je vrlo retko stanje, a neurološki nalaz ne korelira sa obimom anatomskih lezija i degenerativnih promena. Prikaz bolesnika. Opisana je devojčica uzrasta dve i po godine sa insipidnim dijabetesom, punim spektrom histiocitoze moždanog tkiva i skoro fatalnim ishodom. Magnetna rezonanca (MR) je pokazala multiple noduluse sa perifokalnim edemom u regiji hipotalamusa, stalka hipofize i pinealnoj žlezdi. Slične promene nađene su u nucleus caudatus-u i putamenu, levom insularnom korteksu, oba temporalna režnja, tegmentnom delu mezencefalona, medijalnom delu ponsa i medule, obe cerebelarne hemisfere i na leptomeningama, dajući sliku snežnih grudvi i pahuljica. Biopsija je pokazala pozitivitet na vimentin, S-100, CD-68 i CD1a markere. Terapijski protokol LCH-III nije dao povoljan rezultat, a salvage terapiju su roditelji odbili. Devojčica se ponovo javila na pregled u uzrastu od sedam godina sa deceleracijom rasta i preranim pubertetom, kada je uvedena terapija analogom “rilizing” hormona luteinizirajućeg hormona (LH-RH). Kontrolni MR snimak endokranijuma pokazao je slične noduluse u diskretnoj regresiji. Uspeh u školi je bio osrednji, govor usporen i ponašanje ekstrovertno. Zaključak. Histiocitoza mozga je potencijalno fatalno oboljenje hroničnog, varijabilnog, sporo progredirajućeg toka i nepredvidivog odgovora na terapijske protokole.

Reference

Foulet-Roge A, Josselin N, Guyetant S, Gardet J, Bescancon A, Saint-Andre J, et al. Incidental langerhans cell histiocytosis of thyroid: case report and review of the literature. Endo Pathol 2002; 13(3): 227–33.

Grois N, Prayer D, Prosch H, Lassmann H. Neuropathology of CNS disease in Langerhans cell histiocytosis. Brain 2005; 128(Pt 4): 829–38.

Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ. MR imag-ing presentation of intracranial disease associated with Lang-erhans cell histiocytosis. AJNR Am J Neuroradiol 2004; 25(5): 880–91.

Prosch H, Grois N, Wnorowski M, Steiner M, Prayer D. Long-term MR imaging course of Langerhans cell histiocytosis neu-rodegenerative. Am J Neuroradiol 2007; 28(6): 1022–8.

Imashuku S, Shioda Y, Kobayashi R, Hosoi G, Fujino H, Seto S, et al. Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Ja-pan LCH Study Group. Haematologica 2008; 93(4): 615–8.

Grois N, Fahrner B, Arceci RJ, Henter J, McClain K, Lassmann H, et al. Central Nervous System Disease in Langerhans Cell His-tiocytosis. J Pediatr 2010; 156(6): 873–81.e1.

Schmidt S, Eich G, Hanquinet S, Tschäppeler H, Waibel P, Gudinchet F. Extra-osseous involvement of Langerhans? cell histiocytosis in children. Pediatr Radiol 2004; 34(4): 313–21.

Badalian-Very G, Vergilio JA, Degar BA, MacConaill LE, Brandner B, Calicchio ML, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 2010; 116(11): 1919–23.

Imashuku S, Fujita N, Shioda Y, Noma H, Seto S, Minato T, et al. Follow-up of pediatric patients treated by IVIG for Langer-hans cell histiocytosis (LCH)-related neurodegenerative CNS disease. Int J Hematol 2015; 101(2): 191–7.

Haroche J, Cohen-Aubart F, Emile JF, Arnaud L, Maksud P, Charlotte F, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mu-tation. Blood 2013; 121(9): 1495–500.

Héritier S, Jehanne M, Leverger G, Emile J, Alvarez J, Haroche J, et al. Vemurafenib Use in an Infant for High-Risk Langerhans Cell Histiocytosis. JAMA Oncol 2015; 1(6): 836–38.

Cugati G, Pande A, Vasudevan M, Singh M, Ramamurthi R. Hand Schuller Christian disease. Indian J Med Paediatr Oncol 2011; 32(3): 183–4.

Minkov M. Multisystem Langerhans cell histiocytosis in chil-dren: current treatment and future directions. Pediatr Drugs 2011; 13(2): 75–86.

Baumann M, Cerny T, Sommacal A, Koeberle D. Langerhans cell histiocytosis with central nervous system involvement-complete response to 2-chlorodeoxyadenosine after failure of tyrosine kinase inhibitor therapies with sorafenib and imatinib. Hematol Oncol 2011; 30(2): 101–4.

Objavljeno
2021/08/19
Broj časopisa
Vol. 77 Br. 1 (2020): Vojnosanitetski pregled
Rubrika
Prikaz bolesnika