We present a 3-year-old female born of a consanguineous marriage with the complaints of high-grade fever, petechial spots, abdominal distension, and lymphadenopathy for 20 days. She had pallor, hypopigmented hairs, petechial rashes, and palpable lymph nodes (up to 1 cm) in the bilateral inguinal and cervical region. Systemic examination revealed hepatosplenomegaly. Her hematological profile was...

Beige mice express the Chediak-Higashi syndrome. Large inclusions, identified as abnormal lysosomes, are found in many cells. The inclusions in type II alveolar epithelial cells are enlarged lamellar bodies and they are associated with an increase in total lung surface-active material and phospholipid. Comparison of recovery of phospholipid in surface-active materials from beige and black (norm...

This is a Chediak-Higashi Syndrome (CHS) case report in afro-descendant individual, male, 3 months old, born from consanguineous union. On admission he had fever for a month, unresolved pneumonia, and hepatosplenomegaly. He evolved to bacterial sepsis, septic shock, and death. CHS presents quantitative and morphological and hematological changes. Abnormal leukocyte inclusions are the pathognomo...