effectors may be 'mix and match.' For example, the knockout of Rab27 causes the hypopigmentation but also immunodeficiency due to deficiencies in cytotoxic killing activity in cytotoxic T cells (something that also depends on vesicle transport). While, the knockout of myosin Va does not cause immunodeficiency, but it does cause neural defects. Though some neural problems (i.e. brain damage) can be seen in Rab27A deficient children, this is thought to be a secondary effect of the immune problems, and not directly due to the lack of Rab27A. Munc13-4 has also drawn attention based on its involvement in causing bleeding manifestations in Griscelli syndrome.Munc13-4 through its interactions with Rab27a appears to be important for the dense granule release from platelets. The mutated Rab27a interaction with Munc13-4 is the cause of bleeding in type 2 Griscelli Syndrome. Diagnosis Types Griscelli syndrome is a disorder of melanosome transport, and divided into several types: Management Management and treatment is depended upon the type of syndrome one is diagnosed as having. Prognosis for long-term survival is however, relatively poor. Type 2 is usually rapidly fatal within 1 to 4 years without immediate, accelerated treatment. Chemotherapy have achieved remissions however is sometimes ineffective for the treatment of the primary disease and can fail to control relapses. Allogenic bone-marrow transplantation (BMT) is the only known curative treatment in this disease. The severe neurological impairment and retarded development of the human does not improve with time. During the accelerated phase, immunosuppressives can be used to control signs and symptoms. Since its discovery in 1976, only 40 citations have been found in modern literature. In order to treat patients, the aggressive therapy strategy approach must always be taken for acute bacterial infections and prophylactic antibiotics. This assists in minimising possible effects and prolonging life expectancy. Eponym It is named after Claude Griscelli, professor of pediatrics at Hôpital Necker Enfants-Malades in Paris (France). See also Chédiak–Higashi syndrome References External links Disturbances of human pigmentation Noninfectious immunodeficiency-related cutaneous conditions Autosomal recessive disorders Rare syndromes Syndromes affecting the skin Diseases of immune dysregulation Inherited disorders of trafficking