Çocukluk çağı hastalıklarında kemik iliği ve kök hücre nakli sonuçları
Küçük Resim Yok
Tarih
2001
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Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Kemik iliği Transplantasyonu (KİT), kemoterapötik ajanları çok yüksek dozlarda, çoğu zaman radyoterapi ile birlikte, nonhematolojik toksisiteler sınırında hastaya vermek ve böylece maksimal antitümör etki veya eradikasyonu sağlayıp, oluşan kemik iliği supresyonunu da allojenik, sinjeneik veya otolog koşullarda, transplantasyonla desteklemek esasına dayanan bir uygulamadır. Başlangıçta maliyn hastalıklarda uygulanan KİT'nun bir başka uygulama alanı ise bazı doğmalık hastalıklarda, Kİ hücrelerinin sağlıklı olanlarla değiştirilmesi olmaktadır. Bu çalışmada, pediatrik yaş grubundaki maliyn ve maliyn olmayan hastalıklara sahip çocuklarda, kemik iliği ve periferik kan kök hücre naklinden oluşan "Kök Hücre Nakli" uygulandı. Çalışma, Sosyal Sigortalar Kurumu (SSK) Tepecik Eğitim Hastanesi ve Ege Üniversitesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çocuk Onkoloji ve Kemik iliği Transplantasyonu Merkezleri'nde yapıldı. Kasım 1994 - Haziran 2000 yılları arasında toplam 38 olguya otolog veya allojenik transplantasyon uygulandı. Transplantasyon işlemi için maliyn hastalık gurubunda 9 Akut Myeloblastik Lösemi (AML), 5 Akut Lenfoblastik Lösemi (ALL), 3 Kronik Myelositer Lösemi, 3 Hodgkin Hastalığı (HD) ve 5 Solid Tümör olgusu, maliyn olmayan grupta ise 10 Talasemi Majör (TM), bir Aplastik Anemi (AA)'Iİ ve 2 ağır kombine yetmezliği (SCID) olan hastalar seçildi. Üç TM'lu ve bir HD'lı olguya iki kez allojenik veya otolog TX (ikincil Transplantasyon / Second TX) uygulandı. Olguların 20'sı (%52.6) kız, 18'i (%47.4) erkek, ilk tanı anında yaş ortalaması 94.1±67.3 ay (3 - 200), ortanca değer 113.5 ay idi. Transplant zamanındaki yaş ortalaması 119.2 ± 55.7 (8 - 207), ortanca değer 125.0 ay idi. Otolog transplantasyon grubunda 16 olguya periferik kök hücre (OPKH) uygulandı. Allojenik transplantasyon gurubunda toplam 22 olgunun 16'sına kemik iliği transplantasyonu (AKİT), 6 hastaya ise periferik kök hücre nakli (APKH) yapıldı. Allojenik transplantasyonların biri hariç tümünde HLA doku gurupları tam uygun olan kardeş donörler seçildi. Tüm olgular birlikte değerlendirildiğinde; TX yaşı 119.3 ± 55.7 ay (8 ay - 17 yaş), Kök hücre miktarı ortanca 4.46 x 108 (1.50 - 13.2), engraftment günü ortalama 16.4 ± 5.3 gün (7 - 28 gün), lökosit düzelmesi 22.3 ± 17.9 gün (12 - 66 gün), platelet düzelmesi ortalama 26.9 ± 27.3 gün (13 - 67 gün), Post TX DFS 30.7±18.1 ay (5 - 66 ay), Post TX OS ortalama 60.5 ± 52.2 ay (4.5 - 238 ay) bulundu. Sonuç olarak, maliyn ve bazı maliyn olmayan hastalıklarda "Kemik iliği" ve "Kan Kök Hücre" nakilleri ile kür şansı elde edilebilmektedir. Maliyn hastalığı olanlar primer hastalık ile beraber kemoterapi ve radyoterapinin erken ve geç etkilerinden uzaklaşmaktadır. Bazı doğmalık kan hastalıklarında, hasta transfüzyon ihtiyacından kurtulmakta ve geç endokrin etkilere maruz kalmamakta, normal bir yaşam süresine kavuşabilmektedir.
Bone marrow transplantation (BMT) is a treatment option for cancer that allows maximal anti-tumor effect or eradication of a malignancy by the administration of high-dose chemotherapy with or without radiotherapy at less nonhematologic toxic doses. the hematologic toxicitiy in this procedure is dealt with transplanted cells which are essentially allogeneic, syngeneic or autologous. BMT has been used for malignant diseases previously, however, it has been getting more common as a treatment of choice for some congenital disorders in a sense of replacing healthy marrow cells. in the present study we used stem cell transplantation, either marrow-derived or peripheral, for malignant or non-malignant disorders of childhood. This study was carrried out in the Oncology and Transplantation Centers of SSK Tepecik Training Hospital and of Ege University Hospital between November 1994 and June 2000. Thirthy-eight pediatric cases were performed autologous or allogeneic transplantation. the malignant group consisted of 9 ANLL, 5 ALL, 3 CML, 3 Hodgkin's disease and 5 solid tumors. Among the non-malignant group there were 10 thalassemia major (TM), 1 aplastic anemia and 2 severe combined immunodeficiencies. Transplantation procedure was repeated in three TM patients and in a case with Hodgkin's disease. There were 20 female (52.6%) and 18 male (47.4%) in the study group. the mean age at diagnosis was 94.1±67.3 mos (3-200) while the mean age at BMT was 119.2 ± 55.7 mos (8-207). Peripheral blood stem cell transplantation was done in 16 of all autologous transplantations. Sixteen out of 22 allogeneic transplantations were marrow derived transplants whereas 6 were peripheral stem cell. All allogeneic transplantations, but one, were from full-matched siblings. For all patients, the mean age at transplantation was 119.3 ± 55.7 mos (8-17) and the median transplanted stem cell count was 4.46x108 (1.50-13.2). the mean engraftment time was 16.4 ± 5.3 days (7-28). Leukocyte recovery was observed at mean 22.3 ± 17.9 days (12-66 days) and platelet recovery at mean 26.9 ± 27.3 days (13-67 days). Disease free survival at post-transplantation was mean 30.7±18.1 mos (5-66 mos) and overall survival at post-transplantation period was mean 60.5±52.2 mos (4.5-238 mos). in conclusion, transplantation procedure, either marrow-derived or peripheral, can provide a cure status for some malignant and non-malignant diseases in childhood. Besides malignant disease control by transplantation, patients become free of hazardous effects of chemo- and radiotherapy. Some patients with congenital blood disorders become transfusion-free, do not subject to late-term endocrine side effects and survive a normal life.
Bone marrow transplantation (BMT) is a treatment option for cancer that allows maximal anti-tumor effect or eradication of a malignancy by the administration of high-dose chemotherapy with or without radiotherapy at less nonhematologic toxic doses. the hematologic toxicitiy in this procedure is dealt with transplanted cells which are essentially allogeneic, syngeneic or autologous. BMT has been used for malignant diseases previously, however, it has been getting more common as a treatment of choice for some congenital disorders in a sense of replacing healthy marrow cells. in the present study we used stem cell transplantation, either marrow-derived or peripheral, for malignant or non-malignant disorders of childhood. This study was carrried out in the Oncology and Transplantation Centers of SSK Tepecik Training Hospital and of Ege University Hospital between November 1994 and June 2000. Thirthy-eight pediatric cases were performed autologous or allogeneic transplantation. the malignant group consisted of 9 ANLL, 5 ALL, 3 CML, 3 Hodgkin's disease and 5 solid tumors. Among the non-malignant group there were 10 thalassemia major (TM), 1 aplastic anemia and 2 severe combined immunodeficiencies. Transplantation procedure was repeated in three TM patients and in a case with Hodgkin's disease. There were 20 female (52.6%) and 18 male (47.4%) in the study group. the mean age at diagnosis was 94.1±67.3 mos (3-200) while the mean age at BMT was 119.2 ± 55.7 mos (8-207). Peripheral blood stem cell transplantation was done in 16 of all autologous transplantations. Sixteen out of 22 allogeneic transplantations were marrow derived transplants whereas 6 were peripheral stem cell. All allogeneic transplantations, but one, were from full-matched siblings. For all patients, the mean age at transplantation was 119.3 ± 55.7 mos (8-17) and the median transplanted stem cell count was 4.46x108 (1.50-13.2). the mean engraftment time was 16.4 ± 5.3 days (7-28). Leukocyte recovery was observed at mean 22.3 ± 17.9 days (12-66 days) and platelet recovery at mean 26.9 ± 27.3 days (13-67 days). Disease free survival at post-transplantation was mean 30.7±18.1 mos (5-66 mos) and overall survival at post-transplantation period was mean 60.5±52.2 mos (4.5-238 mos). in conclusion, transplantation procedure, either marrow-derived or peripheral, can provide a cure status for some malignant and non-malignant diseases in childhood. Besides malignant disease control by transplantation, patients become free of hazardous effects of chemo- and radiotherapy. Some patients with congenital blood disorders become transfusion-free, do not subject to late-term endocrine side effects and survive a normal life.
Açıklama
Anahtar Kelimeler
Pediatri, Cerrahi, Transplantasyon
Kaynak
Ege Pediatri Bülteni
WoS Q Değeri
Scopus Q Değeri
Cilt
8
Sayı
1