Chediak-Higashi Syndromein Accelerated Phase: A Case Report

Authors

  • Maryam Zulfiqar Department of Pathology, Pakistan Institute of Medical Sciences, Islamabad Shaheed Zulfiqar Ali Bhutto Medical University, Islamabad
  • Asma Mustafa Department of Pathology, Pakistan Institute of Medical Sciences, Islamabad Shaheed Zulfiqar Ali Bhutto Medical University, Islamabad
  • Bushra Anam Ali Department of Pathology, Pakistan Institute of Medical Sciences, Islamabad Shaheed Zulfiqar Ali Bhutto Medical University, Islamabad
  • Lubna Naseem Department of Pathology, Pakistan Institute of Medical Sciences, Islamabad Shaheed Zulfiqar Ali Bhutto Medical University, Islamabad

Keywords:

Chediac-Higashi syndrome,, large azurophilicgranules, repeated bacterial infections,, albinism, progressive peripheral neuropathy, cranial nerve abnormalities, silvery grey hair accelerated phase, lymphohistiocytic infiltration

Abstract

Chediak Higachi Syndrome (CHS) is a very rare autosomal recessive disorder which presents with
repeated bacterial infections, albinism, progressive peripheral neuropathy, cranial nerve abnormalities and
patients have characteristic silvery grey hair and giant bluish grey granules in cytoplasm of white blood cells.
Late stages presents with accelerated phase of this disease which is characterized by lymphohistiocytic infiltration
of spleen, liver and lymph nodes. Here, we report a case of one year old child who presented to us in accelerated
phase of CHS and diagnosis was made on clinical characteristic findings and presence of largeazurophilic
granules in granulocytes and lymphocytes in peripheral blood smear and bone marrow

Downloads

Published

2018-11-21

Issue

Section

Original article

How to Cite

1.
Zulfiqar M, Mustafa A, Ali BA, Naseem L. Chediak-Higashi Syndromein Accelerated Phase: A Case Report. Int J Pathol [Internet]. 2018 Nov. 21 [cited 2024 Jun. 20];:125-8. Available from: https://jpathology.com/index.php/OJS/article/view/297

Most read articles by the same author(s)

1 2 > >>