The Chediak-Higashi anomaly. A report of two cases.

The Chediak-Higashi anomaly. A report of two cases.

Pseudo Chediak-Higashi Anomaly

A 9-year-old girl was admitted to hospital with a 1-month history of fever, weight loss, epistaxis, and abdominal pain. The girl’s parents were non-consanguineous. Her medical history was unremarkable. Upon admission she weighed 27 kg (25th-50th percentile), was 131 cm tall (50th percentile), and was pale. Physical examination showed multiple cervical and inguinal microlymphadenopathies, withou...

Chediak-Higashi Syndrome: A case report

Introduction: Chediak-Higashi syndrome is a rare autosomal recessive disorder that characterized by severe immunodeficiency. It is also associated with a lymphoproliferative disorder termed the accelerated phase with lymphocytic infiltration of the major organ of the body. Case Report: The patients was a 1-year old boy with intermittent fever, anorexia, malaise. On physical examination h...

Chediak-Higashi syndrome - a report of two cases with unusual hyperpigmentation of the face.

Chediak-Higashi syndrome is a rare autosomal recessive disorder due to a qualitative defect in leucocyte function characterized clinically by partial oculocutaneous albinism, recurrent bacterial infections, photophobia etc. The diagnostic feature is the presence of abnormal giant intracytoplasmic granules in neutrophils and their precursors. Here we report this syndrome in two siblings who pres...

Acute promyelocytic leukemia with pseudo-Chediak-Higashi anomaly: a case report and review of the literature.

The hypergranular type of acute promyelocytic leukemia (APL) is characterized by heavy granulation and the presence of Auer rods. These granules are usually small, azurophilic, and have the appearance of the primary granules of normal promyelocytes. There have been several reports of myeloid leukemias, including APL, with the unusual finding of large granules (inclusions) simulating the inclusi...