Chediak-Higashi syndrome (SCH) is a rare autosomal recessive genetic disorder characterized by oculo-cutaneous albinism,
immunodeficiency responsible for recurrent infections, predisposition to bleeding, And late neurological deterioration. The
pathognomonic sign is the presence of giant intracytoplasmic granules in most of the cells of the organism but often they are
identified in peripheral blood. In 85% of cases, CHS patients develop the accelerated phase characterized by an Hemophagocytic
Lymphohistiocytosis syndrome (HLH) responsible for a high mortality rate. The only current effective treatment of haematological
and immunological abnormalities remains allogeneic bone marrow transplantation, but without impact on skin manifestations or
subsequent neurological deterioration. It is all the more effective as it is performed before the onset of an HLH syndrome.