Bone marrow aspirate in Chédiak–Higashi syndrome

✍ Scribed by Abbas Hashim Abdulsalam; Nafila Sabeeh; Barbara J. Bain

• Publisher: John Wiley and Sons
• Year: 2011
• Tongue: English
• Weight: 105 KB
• Volume: 87
• Category: Article
• ISSN: 0361-8609
• DOI: 10.1002/ajh.22123

✦ Synopsis

A 10-month-old Iraqi male presented with meningoencephalitis and was treated with antibiotics. The blood count showed: white cell count 2.5 3 10 9 /l, neutrophil count 0.08 3 10 9 /l, hemoglobin concentration 96 g/l and platelet count 160 3 10 9 /l. The severe neutropenia led to a bone marrow aspirate being performed. This revealed the diagnosis of Che ´diak-Higashi syndrome. The child's skin was normally pigmented and the diagnosis had not previously been suspected.

The inclusions of Che ´diak-Higashi syndrome sometimes have staining characteristics appropriate to a lineage but sometimes they stain abnormally. In this composite image of a Romanowsky-stained bone marrow film, eosinophil granules are giant but retain their eosinophilic characteristics whereas many of the giant neutrophil granules stain deep purple. In addition to globular inclusions, some cells contain crystals (arrows).