Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink

BACKGROUND: Langerhans cell histiocytosis (LCH) in children is relatively rare, and the long-term analysis of therapy results has not been done yet in Hungary. PURPOSE: In this review we summarise the incidence, clinical features, prognostic risk factors and treatment results of children's LCH in Hungary, using data from the National Childhood Cancer Registry in Hungary in a 20-year period between 1981 and 2000. RESULTS: From January 1981 to December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The male-female ratio was 1.36:1, the mean age: 4 years 11 months. The minimal and median follow-up time was 3.48 years and 10.98 years respectively. 38 children had single-system disease, while in 73 cases we found systemic dissemination already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation and 5 children received only local irradiation. In two cases remission was obtained with local steroid administration. 75 patient received chemotherapy. During the twenty years 14 children died, 9 due to the progression of the disease. Sixteen of the 111 patients had relapse with a mean of 2.16+/-1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n=111) was 88.3+/-3.1% at 5 years and 87.3+/-3.2% at 10 and 20 years. CONCLUSION: Childhood LCH is a well treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood

A gyermekkori Hodgkin-lymphoma kezeleési eredményei Magyarországon

Lymphomas are the third most frequent malignancies in childhood. The Hungarian Pediatric Oncology Group was founded in 1971, and since then the same chemotherapeutic protocols have been used in the whole country. In this study we analyzed the data of childhood Hodgkin's lymphoma in Hungary in the last 11 years (1988-1998). We also compared our results with the international (German) data. The incidence of Hodgkin's lymphoma (0-15 years) was 7.1/1,000,000 child/year (the same for non-Hodgkin's lymphoma was 7.5/1,000,000/year); 5.5% of all pediatric malignancies in Hungary). The patients were treated according to the German DAL-HD-82 and 90 protocols. The therapy consisted of 2-6 cytostatic blocks, depending on the stage, followed by involved field irradiation. The overall survival was 94.7+/-2.0% at 5 years and 91.9+/-2.7% at 10 years. These results are very similar to the German data: 94% at 5 years and 93% at 10 years. The good results are due to the well organised network and the uniformed treatment. The results may be ameliorated by using autologous bone marrow transplantation

Donor-cell myelodysplastic syndrome developing 13 years after marrow grafting for aplastic anemia

Donor-cell–derived hematopoietic malignancy is a rare event after bone marrow transplantation. Most cases in the literature occurred within the first year. We present a rare case of a female patient who had a bone marrow transplant for severe aplastic anemia (SAA) at the age of two and a half years from her human leukocyte antigen–identical brother. She developed a myelodysplastic syndrome (refractory cytopenia with multilineage dysplasia) 12 years later. Initially, the malignant clone was of recipient origin, but within several months, progression to a clinically more aggressive refractory anemia with excess blasts (RAEB) was accompanied by the outgrowth of a new clone of donor origin. In this report we provide evidence proving that the patient's final malignant clone arose in donor cells: cytogenetic analysis of the marrow showed a male karyotype and a t(3;21)(q26;q21) in all 62 metaphases analyzed. Interphase fluorescence in situ hybridization showed that all identifiable cells contained the Y chromosome. We conclude that donor-cell–derived hematopoietic malignancy after bone marrow transplantation can occur even after many years. We believe that the 13 years that elapsed between the transplant and the development of RAEB in our case represent the longest latency period in the literature

Gyermekkori Langerhans-sejtes histiocytosissal szerzett magyarországi tapasztalataink

Bevezetés: A gyermekkorban előforduló Langerhans-sejtes histiocytosis (LCH) viszonylag ritka betegség, és ezidáig a terápiás eredmények hosszú távú kiértékelése még nem történt meg hazánkban. Célkitűzés: Az Országos Gyermektumor Regiszter adatait felhasználva megvizsgáltuk az LCH incidenciáját, a klinikai paramétereket, a prognosztikai faktorokat és a terápiás eredményeket az 1981 és 2000 közötti 20 éves periódusban. Eredmények: 1981. január 1. és 2000. december 31. között 111 új, 18 év alatti gyermeket tartottunk nyilván Magyarországon LCH diagnózissal. A fiú – leány arány 1,36:1 volt, az átlagéletkor 4 év 11 hó. A minimális követési idő 3,48 év, a medián követési idő 10,98 év volt. 38 gyermeknél igazoltunk lokalizált betegséget, és 73 esetben már a diagnózis felállításakor több szervrendszer érintett volt. Lokalizált elváltozások közül 22 esetben csupán sebészeti beavatkozásra került sor, 7 gyermeknél a műtétet helyi sugárkezelés követte, 5 gyermek csak irradiációban részesült. 2 gyermeknél lokális szteroidadagolással sikerült remissziót elérni. Összesen 75 gyermek részesült kemoterápiás kezelésben. A fenti 20 éves periódusban 14 gyermeket veszítettünk el: 9 esetben az alapbetegség progressziója okozta a halált. 111 gyermek közül 16-nál észleltük az alapbetegség recidíváját átlagosan 2,16±1,29 évvel a primer diagnózist követően. A recidivált betegek közül három lymphoma esetén alkalmazott kemoterápiát kapott, mely hatására remissziót sikerült elérni. Az összes beteg (n=111) általános túlélése 5 évnél 88,3±3,1% és 10, illetve 20 évnél 87,3±3,2%. Összefoglalás: A gyermekkori LCH ritka, de jól kezelhető megbetegedés. A túlélési ráta magas, a prognózis még disszeminált betegség esetében is kedvező gyermekkorban. Background: Langerhans cell histiocytosis (LCH) in children is relatively rare, and the long-term analysis of therapy results has not been done yet in Hungary. Purpose: In this review we summarise the incidence, clinical features, prognostic risk factors and treatment results of children’s LCH in Hungary, using data from the National Childhood Cancer Registry in Hungary in a 20-year period between 1981 and 2000. Results: From January 1981 to December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The male-female ratio was 1.36:1, the mean age: 4 years 11 months. The minimal and median follow-up time was 3.48 years and 10.98 years respectively. 38 children had singlesystem disease, while in 73 cases we found systemic dissemination already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation and 5 children received only local irradiation. In two cases remission was obtained with local steroid administration. 75 patient received chemotherapy. During the twenty years 14 children died, 9 due to the progression of the disease. Sixteen of the 111 patients had relapse with a mean of 2.16±1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n=111) was 88.3±3.1% at 5 years and 87.3±3.2% at 10 and 20 years. Conclusion: Childhood LCH is a well treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood