Cytological, cytochemical and immunological findings from two children with Chediak-Higashi syndrome.

Adele  Caterino-de-Araújo; Elizabeth de los  Santos-Fortuna; Magda Maria de Sales  Carneiro-Sampaio

doi:10.53393/rial.1990.50.35100

Vol. 50 No. 1-2 (1990), ORIGINAL ARTICLE

Vol. 50 No. 1-2 (1990)

Cytological, cytochemical and immunological findings from two children with Chediak-Higashi syndrome.

ORIGINAL ARTICLE

https://doi.org/10.53393/rial.1990.50.35100

Published 1990-12-28

Adele Caterino-de-Araújo⁺⁻
Elizabeth de los Santos-Fortuna⁺⁻
Magda Maria de Sales Carneiro-Sampaio⁺⁻

Adele Caterino-de-Araújo

Instituto Adolfo Lutz

Elizabeth de los Santos-Fortuna

Instituto Adolfo Lutz

Magda Maria de Sales Carneiro-Sampaio

Departamento de Imunologia Clínica, Instituto da Criança "Prof. Pedro de Alcântara", Hospital das Clinicas, Universidade de São Paulo, SP

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Keywords

Chediak-Higashi Syndrome (CHS)
peripheral blood leukocytes;
abnorrnal granules
cytological
cytochemical and immunological study
lymphocyte subsets

How to Cite

1.

Caterino-de-Araújo A, Santos-Fortuna E de los, Carneiro-Sampaio MM de S. Cytological, cytochemical and immunological findings from two children with Chediak-Higashi syndrome. Rev Inst Adolfo Lutz [Internet]. 1990 Dec. 28 [cited 2024 Jul. 22];50(1-2):261-7. Available from: https://periodicos.saude.sp.gov.br/RIAL/article/view/35100

Abstract

This article describes studics of two unrelated patients, aged near 2 years, with leukocyte granulation abnonnalitics similar to those presented in the Chediak-Higashi Syndrome (CHS). Both patients showed dermatologic manifestations characterized by hypopigmentation of the hair and skin, The cytological and cytochemical study of the peripheral blood leukocytes demonstrated giant abnonnal granules with lisosomal content presented in neutrophils, eosinophils and monocytes. The mononuclear cells displayed one large azurophil granule. The cytochemical functional study of neutrophils showed normal nitrobluc-tetrazolium reduction test, The immunological status of the patients were made and in patient 1 was detected a low number of T helper lymphocytes and a maintenance of lymphocytes in continuous cell culture for five weeks without addition of external growth factor. These findings with clinical manifestations permited to establish the CHS, in these cases.

https://doi.org/10.53393/rial.1990.50.35100

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