| Sažetak | UVOD:
Histiocitoze su rijetke skupine bolesti karakterizirane izrazitom proliferacijom i nakupljanjem stanica monocitno-makrofagnog sustava. Mogu se očitovati lokalno i diseminirano, akutnim i kroničnim tijekom te različitim intenzitetom i ishodom ovisno o tipu i dobi pojavljivanja. Histiocitoza Langerhansovih stanica (engl. Langerhans Cell Histiocytosis - LCH) najčešča je među histiocitozama. Najčešće obolijevaju djeca do 15. godine života, ali može se pojaviti i u odraslih. Tijek i prognoza bolesti je nepredvidiva, od spontane regresije do brzo progredirajuće bolesti sa smrtnim ishodom.
CILJEVI ISTRAŽIVANJA:
Cilj istraživanja bio je prikazati sve bolesnike s LCH koji su dijagnosticirani i liječeni na Kliničkom odjelu za hematologiju, onkologiju, imunologiju i medicinsku genetiku Klinike za dječje bolesti KBC Splitu u periodu od 1999. - 2014. godine. Cilj istraživanja nadalje je analizirati raznolikost kliničke prezentacije LCH u djece uz opis pojedinačnih slučajeva te prikazati metodologiju i rezultate liječenja.
MATERIJALI I METODE ISTRAŽIVANJA:
Ispitanici su djeca oboljela od LCH koja su liječena na Kliničkom odjelu za hematologiju, onkologiju, imunologiju i medicinsku genetiku Klinike za dječje bolesti KBC Split u periodu od 1999. - 2014. godine, a korišteni su podaci iz arhive istog. Analizirala se dob i spol bolesnika, prisutnost subjektivnih simptoma, boli, vrućice, kožnih i koštanih promjena, zahvaćenosti organa i organskih sustava, primjenjene dijagnostičke metode, protokoli liječenja, praćenje bolesnika, učestalost povrata ili progresije bolesti.
REZULTATI:
Prikazali smo jedanaest slučajeva djece oboljele od LCH. Analizirali smo našu skupinu bolesnika s obzirom na spol, dob pri pojavi prvih simptoma, zahvaćanje organskih sustava, liječenje i tijek bolesti. Neznatno češće se nalazi u muškog spola (1,2:1). Primjetili smo kako se bolest najčešće pojavila u dobi od navršene prve do sedme godine života (46 %), potom u dojenačkoj (37 %) te na kraju u adolescentnoj dobi (18 %). SS-LCH imalo je 6, dok je MS-LCH imalo 5 bolesnika. Bolest je najčešće zahvaćala koštani sustav (64 %), potom kožu (45 %), limfne čvorove (27 %), meka tkiva (18 %) te najrijeđe pluća (9 %). Rizični organi (jetra, slezena i koštana srž) bili su zahvaćeni u 18 % slučajeva. Relaps bolesti zabilježen je u dva bolesnika (18 %), dok je progresija bolesti zabilježena je u 4 bolesnika (36 %). Najčešći protokoli korišteni u liječenju su bili LCH protokoli kreirani sa strane ekspertne skupine liječenika međunarodnog Udruženja za histiocitozu. Terapiju održavanja alfa interferonom primalo 5 bolesnika. Niti jedan bolesnik nije umro.
ZAKLJUČCI:
LCH se klinički prezentira na više načina. Najčešće je bolešću zahvaćena koža i kosti. Rizični organi su češće zahvaćeni u mlađoj životnoj dobi, dok su pluća rijetko zahvaćena. Naši su bolesnici uspješno liječeni prema protokolima LCH. Iako je LCH rijetka bolest na nju valja misliti u svakodnevnoj kliničkoj praksi, naročito ako postoje dugotrajne kožne promjene koje ne regrediraju na terapiju, verificirana osteolitička žarišta te recidivirajuće upale uha. Precizna dijagnostika doprinosi uspješnom liječenju bolesti. |
| Sažetak (engleski) | INTRODUCTION:
Histiocytic disorders are a group of diseases that occur when there is an over-production of histiocytes. That can lead to organ damage and tumor formation. Lesions can be localized or disseminated, with acute and chronic progress, with different intensity and outcome depending on their type and on the age of occurrence. Langerhans cell histiocytosis is the most common. It primarily affects children under the age of 15, but it can have adult onset. The course and prognosis of the disease are unpredictable, from spontaneous regression to rapidly progressive disease with fatal outcome.
OBJECTIVES:
The aim of the study was to show all patients with LCH who were diagnosed and treated at the Pediatric Hematology, Oncology, Immunology and Medical Genetics Department Clinical Hospital Centre Split from 1999 to 2014 and analyse the variety of clinical presentation of LCH in children with a description of individual cases, methodology and results of treatment.
MATERIALS AND METHODS:
The examinees are children suffering from LCH treated at the Pediatric Hematology, Oncology, Immunology and Medical Genetics Department Clinical Hospital Centre Split from 1999 to 2014. The data were taken from the archive of above-mentioned department. Subject of the analysis was age and sex of the patient, presence of subjective symptoms such as pain, fever, skin, bone changes, involvement of organs and organ systems, applied diagnostics methods, treatment protocols, follow-up, relapse frequency or disease progression.
RESULTS:
We have presented eleven cases of children suffering from LCH and analysed our group of patients with respect to gender, age at onset of the first symptoms, tissue and organ involvement, treatment and course of the disease. LCH is slightly more often found in males (1.2:1). In our cohort of children LCH usually occurred in the age of 1 to 7 (46 %), followed by the infant age (37 %) and adolescence (18 %). Six patients had SS-LCH and five had MS-LCH. Bones were most frequently affected (64 %), then the skin (45 %), the lymphnodes (27 %), soft tissues (18 %) and least frequently lungs (9 %). Risk organs (liver, spleen and bone marrow) were affected in 18 % of cases. Relapse was recorded in two patients (18 %) while progression was recorded in 4 patients (36 %). The more often applied treatment protocols were LCH ones created by Histiocyte Socitey experts. Five patients received the maintenance therapy with alpha interferon. No one patient died.
CONCLUSIONS:
LCH presents clinically in several ways. The disease most frequently affects skin and bones. The risk organs are often affected at a younger age while the lungs are rarely affected. Our patients are successfully treated according to LCH protocols. Although LCH is a rare disease, it has to be considered in everyday clinical practice, especially with long-term skin changes that do not react to therapy, verified osteolytic focuses and recurrent ear inflammation. Precise diagnosis contributes to the successful treatment of the disease. |
