ABSTRACT
The authors describe a Thai newborn boy who was presented with petechiae, hepatosplenomegaly and pancytopenia at birth caused by congenital HIV infection. His clinical presentations were appeared on the early onset after birth. The bone marrow finding has shown hypocellularity which was also rare in HIV-infected children.
Subject(s)
Adrenal Cortex Hormones/therapeutic use , Ampicillin/therapeutic use , Anti-Bacterial Agents/therapeutic use , Anti-HIV Agents/therapeutic use , Gentamicins/therapeutic use , HIV Infections/complications , Humans , Infant, Newborn , Male , Pancytopenia/diagnosis , Purpura/diagnosis , Splenomegaly/diagnosisABSTRACT
Chediak-Higashi syndrome (CHS) is a very rare autosomal recessive immunodeficiency disorder characterized by partial albinism, recurrent pyogenic infections, and large granules in all granule-containing cells. The author describes a Thai girl who was the first case of CHS in Thailand. She presented in the accelerated phase of CHS, which leads to repeated infections and bleeding, often resulting in fatal outcome. Pancytopenias, hepatosplenomegaly, lymphohistiocytic infiltration in bone marrow and the abnormal characteristic granules in leukocyte clinched the diagnosis.