Pseudo Chediak-Higashi Anomaly

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...

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...

Pseudo-Chediak-Higashi anomaly in a case of acute myeloid leukemia: electron microscopic studies.

The formation and fine structure of giant granules in neutrophil promyelocytes of a patient with a variant of acute myelogenous leukemia were investigated by electron microscopy. The patient presented with large lymph nodes and disseminated intravascular coagulation (DIC). By light microscopy, numerous giant granules, resembling those of Chediak-Higashi syndrome (CHS), were present, but Auer bo...

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

Chediak-Higashi Syndrome

Impaired vision Photophobia Albinism of the OCA2 type, giving a lighter complexion than unaffected family members [6] Silvery sheen to hair which may be fair in colour Frequent infections (skin, mucous membranes, respiratory) Epilepsy Mental retardation Enlarged liver and spleen Jaundice Ataxia causing incoordination and a typical ataxic gait Tremor Epilepsy Peripheral neuropathy causing motor ...