Mixed chimerism following hematopoietic stem cell transplantation in pediatric thalassemia major patients: a single center experience
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). 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.

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Authors: Elif Ünal İnce, Mehmet Ertem, Talia İleri, Klara Dalva, Pervin Topcuoğlu, Zümrüt Uysal

Keywords: Talasemi majör, karışık tip kimerizm, pediatri, kimerizm, hematopoetik kök hücre transplantasyonu, non-malign

• Year: 2010
• Number: 1
• Referance No: 92905

---

• 

  Figure 1. Summary of the chimerism outcome MC: Mixed chimerism; FRC: Full recipient chimerism; FDC: Full donor chimerism
• 

  Figure 2. Evolution of the early MC detected at 1st month postHSCT MC: Mixed chimerism; FRC: Full recipient chimerism; FDC: Full donor chimerism. *Two patients who died after 1 year postHSCT had FDC
• 

  Figure 3. Course of chimerism in patients with MC