Mixed chimerism following hematopoietic stem cell transplantation in pediatric thalassemia major patients: a single center experience/ Pediatrik talasemi major hastalarinda hematopoetik kok hucre nakli sonrasi karisik tip kimerizm-tek merkez sonuclari

Objective: Stable mixed chimerism (MC) may result in cure for thalassemia major patients following hematopoietic stem cell transplantation (HSCT), but rejection can occur. Twenty-eight HSCTs for thalassemia major were reviewed retrospectively to evaluate the clinical course of MC with possible risk...

Full description

Saved in:
Bibliographic Details
Published in:Turkish journal of haematology 2010-03, Vol.27 (1), p.8
Main Authors: Ince, Elif Unal, Ertem, Mehmet, Ileri, Talia, Dalva, Klara, Topcuoglu, Pervin, Uysal, Zumrut
Format: Article
Language:English
Subjects:
Care and treatment
Diagnosis
Hematopoietic stem cells
Risk factors
Thalassemia
Transplantation
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Objective: Stable mixed chimerism (MC) may result in cure for thalassemia major patients following hematopoietic stem cell transplantation (HSCT), but rejection can occur. Twenty-eight HSCTs for thalassemia major were reviewed retrospectively to evaluate the clinical course of MC with possible risk factors and predictors of outcome, with a median follow-up of 1669 days (811-3576 days). Materials and Methods: Chimerism was detected by fluorescence in situ hybridization (FISH) or multiplex polymerase chain reaction depending on the sex match between the donor and the recipient. Results: Primary rejection, stable MC and full donor chimerism was detected in 3.6%, 17.8% and 78.6% of patients, respectively. Clinically, 4/5 patients with stable MC had thalassemia trait with donor chimerism as low as 14%. One patient was started on pRBC transfusions at 2.5 years postHSCT. Conclusion: Stable MC can result in cure for thalassemia major patients. The clinical picture remains as the best guide for intervention until a more reliable predictor is available. (Turk J Hematol 2010; 27: 8-14) Key words: Thalassemia major, mixed chimerism, pediatrics, chimerism, hematopoietic stem cell transplantation, nonmalignant Amac: Talasemi major (TM) hastalarinda hematopoetik kok hucre transplantasyonu (HKHT) sonrasinda stabil karisik-tip kimerizm ile kur mumkundur fakat rejeksiyonla sonuclanabilir. TM tanisiyla yapilmis 28 HKHT karisik-tip kimerizm seyri, risk faktorleri ve sonuclarini degerlendirmek uzere retrospektif olarak gozden gecirildi. Yontem ve Gerecler: izlem suresi ortanca 1669 gundu (811-3576 gun). Kimerizm alici-verici cinsiyet uyumuna gore FISH veya multipleks PCR yontemiyle bakildi. Bulgular: Primer rejeksiyon, stabil karisik tip kimerizm ve tam verici tip kimerizm sirasiyla % 3.6, % 17.8 ve % 78.6 idi. 4/5 hastada % 14 kadar dusuk verici kimerizmine ragmen talasemi tasiyiciligi klinigi vardi. Sadece 1 hastada HKHT sonras? 2.5 yil sonra eritrosit ihtiyaci oldu. Sonuc: Stabil karisik-tip kimerizm, talasemi major hastalarinda kur saglayabilir ve bu hastalar icin rejeksiyonu onceden gosteren bir belirtec bulunana kadar hastalarin klinigi, herhangi bir mudahale icin belirleyici olmalidir. (Turk J Hematol 2010; 27: 8-14) Anahtar kelimeler: Talasemi major, kansik tip kimerizm, pediatri, kimerizm, hematopoetik kok hucre transplantasyonu, non-malign
ISSN:1300-7777
1308-5263