Turner syndrome (TS) is a genetic disorder affecting 1/2500-3000 live-born females. It is characterized by the complete or partial absence of one of the X-chromosomes, and is frequently accompanied by mosaicism (45,X/46,XX). Its most consistent clinical features are short stature and ovarian failure. Loss of specific genes located on the Xchromosome are thought to be responsible for a range of disorders, including osteoporosis, hypothyroidism, diabetes mellitus (DM), cardiovascular and gastrointestinal diseases, ophthalmic disorders, as well as various immunological defects. 1,2 We report two cases of allogeneic stem cell transplantation (SCT) in which 45,X/46,XX stem cells derived from donors with mosaic TS were used.Patient 1, a 27-year-old woman, had common acute lymphoblastic leukemia with an abnormal karyotype: 46,X,t(X;22)(p11.3;q12),t(2;16)(p11;q11)[15]/46,XX [5]. She underwent a consolidative allogeneic SCT after a busulfancyclophosphamide (Bu-Cy) conditioning regimen from her phenotypically human leukocyte antigen (HLA)-matched 2.5-year-old daughter. The post transplant course was unremarkable and mild acute and chronic graft-versus-host disease (GVHD) were seen. A routine cytogenetic study of the marrow at 6 months revealed 45,X[9]/46,XX [11]. To investigate the origin of 45,X cells, the donor's constitutional karyotype was evaluated, confirming a mosaic TS 45,X[15]/46,XX [5]. The disease relapsed at 9 months and cytogenetic analysis revealed 45,X[8]/46,X,t(X;22)(p11.3; q12),t(2;16)(p11;q11)[2]/46,XX [16]. A second allograft was performed from the same donor in second remission after total body irradiation-based conditioning and attenuated GVHD prophylaxis. The patient developed grade II acute GVHD again. The disease relapsed 5 months after the second allograft and the patient died of relapse.Patient 2, a 47-year-old man, had acute myeloid leukemia with a normal karyotype who was allografted in first relapse after Bu-Cy conditioning from his 54-year-old one HLA-A antigen-mismatched sister. Post transplant course was characterized by grade II acute GVHD and thrombotic microangiopathy. Reports of leukemia in patients with TS are rare. Therefore, toxicity and outcome after intensive chemotherapy for patients with 45,X/46,XX mosaicism are still unclear. 3 Various immunological disturbances have been detected in patients with TS, including decreased numbers of circulating T-lymphocytes. 2,4 Consequently, the study of immune recovery of the donor's 45,X T cells following SCT is particularly interesting, because it might influence infectious complications, antitumor activity, the development and the extent of acute and chronic GVHD, as well as the process of engraftment of hematopoietic stem cells. It is also not clear if 45,X hematopoietic stem cells would respond normally to immunosuppression.A prompt hematopoietic recovery and immune reconstitution were noticed in both of our cases. Both recipients manifested acute GVHD and CMV infections, which were successfully treated. The second patient developed sever...