دورية أكاديمية
Chediak Higashi Syndrome with Hemophagocytic Lymphohistiocytosis.
| العنوان: | Chediak Higashi Syndrome with Hemophagocytic Lymphohistiocytosis. |
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| المؤلفون: | Safavi M; Pathology Department, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran., Parvaneh N; Division of Allergy and Clinical Immunology, Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran. |
| المصدر: | Fetal and pediatric pathology [Fetal Pediatr Pathol] 2023 Apr; Vol. 42 (2), pp. 259-262. Date of Electronic Publication: 2022 May 24. |
| نوع المنشور: | Case Reports; Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Informa Healthcare Country of Publication: England NLM ID: 101230972 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1551-3823 (Electronic) Linking ISSN: 15513815 NLM ISO Abbreviation: Fetal Pediatr Pathol Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: London : Informa Healthcare Original Publication: Philadelphia, PA : Taylor & Francis, c2004- |
| مواضيع طبية MeSH: | Chediak-Higashi Syndrome*/complications , Chediak-Higashi Syndrome*/diagnosis , Lymphohistiocytosis, Hemophagocytic*/complications , Lymphohistiocytosis, Hemophagocytic*/diagnosis, Female ; Humans ; Infant ; Skin ; Vesicular Transport Proteins ; Dexamethasone/therapeutic use |
| مستخلص: | 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. |
| فهرسة مساهمة: | Keywords: Chediak-Higashi syndrome; hemophagocytic lymphohistiocytosis; immune deficiency |
| المشرفين على المادة: | 0 (Vesicular Transport Proteins) 7S5I7G3JQL (Dexamethasone) |
| تواريخ الأحداث: | Date Created: 20220524 Date Completed: 20230328 Latest Revision: 20230328 |
| رمز التحديث: | 20240628 |
| DOI: | 10.1080/15513815.2022.2077489 |
| PMID: | 35608383 |
| قاعدة البيانات: | MEDLINE |
PDF full text from Publisher | تدمد: | 1551-3823 |
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| DOI: | 10.1080/15513815.2022.2077489 |