Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis

Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis Publisher Pubmed

Safavi M¹ ; Parvaneh N²

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1. Pathology Department, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran
2. Division of Allergy and Clinical Immunology, Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran

Source: Fetal and Pediatric Pathology Published:2023

Abstract

Introduction: Chediak-Higashi syndrome (CHS) is caused by dysfunction of lysosomal trafficking and presents with hypopigmentation, bleeding tendencies, neurological symptoms, and NK cell dysfunction. Hemophagocytic lymphohistiocytosis (HLH) can complicate CHS due to the abnormal function of NK cells. Case presentation: This 1.5-year-old light-skinned gray-haired girl microscopically had abnormal hair pigment clumps and lilac inclusions in the myeloid series, characteristic of CHS. She presented with HLH, requiring treatment with etoposide and dexamethasone followed by cyclosporine and dexamethasone. Conclusion: CHS is one of the underlying primary causes of HLH. © 2022 Taylor & Francis Group, LLC.

Style	Citing Format
MLA	Safavi M, Parvaneh N. "Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis." Fetal and Pediatric Pathology, vol. 42, no. 2, 2023, pp. 259-262.
APA	Safavi M, Parvaneh N (2023). Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis. Fetal and Pediatric Pathology, 42(2), 259-262.
Chicago	Safavi M, Parvaneh N. "Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis." Fetal and Pediatric Pathology 42, no. 2 (2023): 259-262.
Harvard	Safavi M, Parvaneh N (2023) 'Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis', Fetal and Pediatric Pathology, 42(2), pp. 259-262.
Vancouver	Safavi M, Parvaneh N. Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis. Fetal and Pediatric Pathology. 2023;42(2):259-262.
BibTex	@article{ author = {Safavi M and Parvaneh N}, title = {Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis}, journal = {Fetal and Pediatric Pathology}, volume = {42}, number = {2}, pages = {259-262}, year = {2023} }
RIS	TY - JOUR AU - Safavi M AU - Parvaneh N TI - Chediak Higashi Syndrome With Hemophagocytic Lymphohistiocytosis JO - Fetal and Pediatric Pathology VL - 42 IS - 2 SP - 259 EP - 262 PY - 2023 ER -

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