Griscelli syndrome (GS) is an autosomal recessive disorder with partial albinism, silver gray hair, hepatosplenomegaly, immune deficiency, hemophagocytic lymphohistiocytosis (HL), and neurological manifestations (1,2). There are three types: Griscelli syndrome type 1 (GS 1): Early severe psychomotor retardation and a normal immune state; Griscelli syndrome type 2 (GS 2): Immune deficiency, hemophagocytic lymphohystiocytosis, and neurological findings in the absence of a primary neurologic disease; Griscelli syndrome type 3 (GS 3): Includes partial albinism with hypopigmentation of the hair and skin (1). Central nervous system involvement has been described previously. Here, we describe a case of GS 2 without HL.
Radiosynovectomy (RS) is a proven important technique which has been used efficently as a local form of radiotherapy to control resistant synovitis and recurrent hemarthrosis. It was first introduced by by Fellinger et al. (1) in 1952 and has been used succesfully for 60 years as an alternative to surgery for treatment of chronic synovitis of patients in whom systemic antiinflammatory medications, intraarticular steroid and chemical injections don’t provide relief (2-6). The indications of treatment with 90Y-Silicate/citrate, 186Resulfide, 32P-chromic phosphate or 169Er-citrate are (7): RA, undifferentiated arthritis characterized by synovitis, inflammatory joint diseases of other origin (Lyme’s borreliosis, psoriatic arthritis, ankylosing spondylitis), persistent synovial effusion (e.g., after endoprosthesis placement), osteoarthritis characterized by synovitis, villonodular synovitis, and hemarthrosis and synovitis associated with hemophilia.
