[Survival rate among children with acute lymphoblastic leukemia based on their relapse status in Shiraz Shahid Faghihi hospital during 2004-9]

Relapse in leukemic patients is considered as a major cause of treatment failure and a decrease in patient survival rate. This study was conducted to determine the survival rate of patients with acute lymphoblastic leukemia [ALL] and acute myeloid leukemia [AML] based on their relapse status. This retrospective cohort study was performed on a total of 243 cases of leukemia aged less than 15 years in Shiraz Shahid Faghihi hospital. Data were analyzed using Kaplan-Meier method, log-rank test and Cox regression model. The 1-and 5-year survival rates for patients without relapsed leukemia were 96.9 and 76.9% and for relapsed leukemia were 82.4 and 28.6%, respectively. Therefore, there was a significant relationship between the relapse occurrence and survival rate among the patients [P=0.001]. The relapse occurrence is one of the main effective factors in survival rate of leukemic patients; a five-year survival rate in the patients is less than 30 percent in this center

Griscelli Syndrome; A case report and review of the literature

Griscelli syndrome [GS] is a rare disease first described in 1978. It is inherited in autosomal recessive pattern. This disease is characterized by partial albinism, pigmentation dilution, cellular immunodeficiency, neurological involvement and uncontrolled phases of macrophage and lymphocyte activation. We report a 5 months Old Iranian girl presenting with silver-gray hair, eyelashes and eyebrows, hepatosplenomegaly, pancytopenia, hemophagocytosis and progressive neurologic deterioration. Griscelli syndrome can be suggested according to her symptoms. The chemotherapy was not effective for her and she died due to multi organ failure