Çocuklarda allojeneik hematopoetik kök hücre transplantasyonunun kemik mineral dansitesi üzerine etkisi

Cocuklarda Allojeneik Hematopoetik Kok Hucre Transplantasyonunun Kemik Mineral Dansitesi Uzerine EtkisiGiris ve Amac: Hematopoetik Kok Hucre Transplantasyonu (HKHT), son yillarda dunyada ve ulkemizde artan siklikta uygulanan bir tedavi yontemi haline gelmistir. Dunyada her yil 50.000' den fazla hastaya HKHT uygulanmaktadir. Gunumuzde transplantasyon tekniklerindeki gelismeler, myeloablatif olmayan transplant rejimlerinin kullanima girmesi, daha iyi enfeksiyon kontrolu ve destek tedavilerdeki gelismelerle birlikte HKHT sonrasi hastalar uzun sureli yasam sansi bulmustur. HKHT sonrasi yasam oranlarinin artmasiyla birlikte ilk zamanlarda fazla gundeme gelmeyen gec donem komplikasyonlari dikkat cekmeye baslamistir. Kemik mineral dansitesinde azalma (osteopeni/osteoporoz) HKHT sonrasi gec donemde gorulebilen, hayati tehlike yaratmayan ancak yasam kalitesini belirgin olarak etkileyen bir komplikasyondur. Bu calismada temel amac; cocuklarda uygulanan allojeneik hematopoetik kok hucre transplantasyonunun, kemik mineral dansitesi uzerine etkisini arastirmaktir.Metot: Bu calismada; Mayis 1996 ? Eylul 2008 tarihleri arasinda Ankara Universitesi Tip Fakultesi Pediatrik Kemik Iligi Transplantasyon Unitesi' nde, hematolojik hastaliklari nedeniyle allojeneik HKHT uygulanan 70 hasta uzerinde calisilmistir. Tum hastalarin bazal ve HKHT sonrasi 1. yildaki kemik mineral dansiteleri degerlendirilmistir. Hastalarin HKHT uygulamasi sirasindaki ortalama yasi 11 (0,9-17,5)' dir ve %60' i erkek, %40' i ise kiz cinsiyettedir. Hastalarin tanilarina bakildiginda, 34 (%48,5) hasta thalasemi major, 14 (%20) hasta akut myelositer losemi, 8 (%11,5) hasta Fankoni aplastik anemisi 4 (%5,5) hasta akkiz aplastik anemi, 3 (%4,3) hasta kronik myelositer losemi, 3 (%4,3) hasta myelodisplastik sendrom, 2 (%3) hasta hemofagositik lenfohistiyositoz, 1 (%1,5) hasta akut lenfoblastik losemi ve 1 (%1,5) hasta orak hucreli anemi tanilidir.Bulgular: Hastalarin bazal KMD Z skoru -0,32 ± 1,44 ve HKHT sonrasi 1. yilda ise -0,68 ± 1,38 olup HKHT sonrasi KMD Z skorunda anlamli oranda azalma oldugu gorulmustur. Hastalari Z skoru normal olan (?-1) ve normal olmayan (<-1) olarak iki gruba ayirarak degerlendirildiginde; bazalde 49 hasta (%70) normal ve 21 hasta (%30) dusuk, HKHT sonrasi 1. yilda ise 40 hasta (%57) normal ve 30 hasta (%43) dusuk Z skoruna sahip olarak bulunmustur. Bazalde Z skoru normalken HKHT sonrasi 1. yilda dusuk olan hasta sayisi ise 12 (% 17)' dir.HKHT sonrasi 1. yilda KMD Z skoru normal olan (?-1) ve dusuk olan (<-1) hastalar karsilastirildiginda, Z skoru dusuk olan hastalarin anlamli olarak daha fazla oranda hipogonadizm ve buyume hormonu eksikligine sahip olduklari gorulmustur. Ayrica HKHT oncesi yogun kemoterapi alan akut ve kronik losemiler gibi malign hematolojik hastaligi olan hastalarda dusuk KMD Z skoru gorulme riski, diger hasta gruplarina gore 5 kat daha fazla olarak bulunmustur. Odds Ratio (%95CI): 5,09 (1,193 ? 21,75 )Sonuc: Allojeneik HKHT sonrasi belirli oranlarda osteopeni ve osteoporoz gorulebilmektedir. Bu komplikasyon cogunlukla fraktur gelisene kadar asemptomatik kalmaktadir. Bu nedenle HKHT uygulamasi yapilacak tum hastalarin bazal KMD olcumleri yapilmali ve HKHT sonrasi ozellikle risk gruplari basta olmak uzere KMD olcumleri ile hastalar izlenmelidir. Boylece fraktur asamasina gelmeden kemik mineralizasyon bozukluklarinin erken tespiti ve tedavisiyle hastalarin yasam kalitesi arttirilabilecektir. AbstractEffect of Hematopoietic Stem Cell Transplantation on Bone Mineral Density in ChildrenIntroduction: Hematopoietic stem cell transplantation (HSCT) has been the treatment modality of choice in the last years in Turkey and worldwide. More than 50.000 patients has been treated with HSCT annually in the world. At the time present, patients treated with HSCT have prolonged lifespan by means of recent advances in transplantation techniques, use of non-myeloablative transplantation regimens, better control of infections and advances in supportive therapies. With the increase in lifespan after HSCT, overlooked long term complications have been come to attention. Decrease in bone mineral density (osteoporosis/osteopenia) which can be encountered with after HSCT is a complication reducing quality of life but not jeopardizing patient?s life. The main purpose of this study is to look for effect of HSCT on bone mineral density (BMD) in children who underwent HSCT.Methods: In this study, between periods of May 1996 and September 2008; 70 patients who had been treated with HSCT in Ankara University, Faculty of Medicine, Pediatric Stem Cell Transplantation Unit due to their hematological diseases was evaluated. All patients? basal and post-transplantation 1 year BMDs were evaluated. Average age of patients during HSCT were 11 (0.9-17.5) and 60% of patients were male and the rest 40% was female. When it is looked at their diagnoses, 34 (48,5) patients with thalassemia major, 14(20%) patients with acute myeloid leukemia, 8 (11,5%) patients Fanconi? s aplastic anemia, 4 (5,5) patients with akkiz aplastic anemia, 3 (%4,3) patients with chronic myeloid leukemia, 3 (4,3%) patients with myelodysplastic syndrome, 2 (3%) patients with hemophagocytic lymphohistiocytosis, 1 (1.5%) patient with acute lymphoblastic leukemia, and 1(1,5%) patient was diagnosed with sickle cell anemia.Findings: Patients? basal BMD Z score was in the range of -0,32±1,44 and was in the range of -0,68±1,38 a year after HSCT. Significant decrease was observed. When patients were evaluated in the groups of ones with normal Z score (?1) and others with abnormal Z score (<-1), according to their basal BMD, 49 (70%) were normal and 21 (%30) were low. A year after HSCT, 40 (57%) patients were normal and 30 (43%) were abnormal in Z score. When patients with normal Z score during first of HSCT compared to the ones with low scores, it was seen that patients with low Z scores had significant growth hormone deficiency and hypogonadism. In addition to this finding, risk of being with low Z score was five times more often in patients with malignant hematological diseases such as acute or chronic leukemia who had been treated by intensive chemotherapy before application of HSCT compared to other risk groups. Odds Ratio (95%CI): 5.09 (1.193-21.75)Results: Osteopenia and osteoporosis can be encountered with after HSCT at certain ratios. This complication can mostly be asymptomatic until a fracture occurs. This is why patients who are going to be treated with HSCT should be evaluated by BMD measures and after HSCT especially patients carrying high risk should be followed with BMD measures. Thus, without reaching fracture staging, bone mineral density disorders can be detected earlier and patient?s quality of life can be increased substantially.