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Griscelli Syndrome Type 2; A Pediatric Case with Immunodeficiency

TABATABAIE PARVIZ نويسنده , Mahjoub Fatemeh نويسنده , CHERAGHI TAHER نويسنده , Parvaneh Nima نويسنده

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A 3,5 month-old girl was admitted with silvery gray hair, light colored skin, recurrent diarrhea, chest infections, hepatosplenomegaly, episodes of pancyropel1Ia, and hemophagocytosis in the bone marrow. Light microscopy of hair showcd charnctcrisrir large and irregular clumps of melanin in the middle of hair shaft. Peripheral blood smear examination did not show giant granules in granulocytes. On the basis of these clinical and laboratory findings, Griscelli syndrome was diagnosed, The child succumbed to infection during an accelerated phase of the disease

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