Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries.

The results of hematopoietic stem cell transplant (HSCT) for primary immunodeficiency diseases (PID) have been improving over time. Unfortunately, developing countries do not experience the same results. This first report of Brazilian experience of HSCT for PID describes the development and results in the field. We included data from transplants in 221 patients, performed at 11 centers which participated in the Brazilian collaborative group, from July 1990 to December 2015. The majority of transplants were concentrated in one center (n = 123). The median age at HSCT was 22 months, and the most common diseases were severe combined immunodeficiency (SCID) (n = 67) and Wiskott-Aldrich syndrome (WAS) (n = 67). Only 15 patients received unconditioned transplants. Cumulative incidence of GVHD grades II to IV was 23%, and GVHD grades III to IV was 10%. The 5-year overall survival was 71.6%. WAS patients had better survival compared to other diseases. Most deaths (n = 53) occurred in the first year after transplantation mainly due to infection (55%) and GVHD (13%). Although transplant for PID patients in Brazil has evolved since its beginning, we still face some challenges like delayed diagnosis and referral, severe infections before transplant, a limited number of transplant centers with expertise, and resources for more advanced techniques. Measures like newborn screening for SCID may hasten the diagnosis and ameliorate patients' conditions at the moment of transplant.

Corneal ectasia detected 32 months after LASIK for correction of myopia and asymmetric astigmatism.

We report a case of corneal ectasia detected 32 months after laser in situ keratomileusis (LASIK) for correction of -4.25 diopters (D) of myopia associated with -2.00 D of regular but slight asymmetric astigmatism. The patient retained stable visual acuity for 15 months postoperatively. The preoperative corneal thickness was 540 microm, and the postablation untouched stroma was assumed to be 290 microm. Although a rare complication of LASIK, corneal ectasia can occur, and there is no consensus regarding how much stroma should be left intact to avoid it. Until we have a better understanding of corneal strength, we think surface photorefractive keratectomy or laser-assisted subepithelial keratectomy ablations should be considered instead of LASIK in borderline cases.