RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164071658 T1 Griscelli Syndrome T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164071658 RD 2024/03/29 AB Griscelli Syndrome is a rare autosomal recessive disorder characterized by albinism with immunodeficiency that usually causes death by early childhood. It is characterized by partial pigmentary dilution of the skin and hair (silvery gray hair), frequent infections, neurologic abnormalities, and fatal outcome caused by uncontrolled T lymphocyte and macrophage activation. Clinical features include the presence of large clumps of pigment in hair shafts and a pathological accumulation of melanosomes in melanocytes. Two types are described: Type I with severe neurologic impairment and Type II with immunologic deficiency.