Chédiak-Higashi syndrome
From www.haematologyetc.co.uk
Appearance:
Reduced numbers of substantially enlarged granules, seen within neutrophils, eosinophils, monocytes and some lymphocytes.
Images of Chédiak-Higashi cells
Note the characteristic abnormally large and coarse granulation affecting each cell type. A neutrophil (image 1); neutrophil and eosinophil (image 2).Images 3 and 4 also show typical lymphocytes with a single large granule
Described by:
The Cuban physician Alejandro Moisés Chédiak, and the Japanese pediatrician Otokata Higashi in 1943
Cause:
A rare recessive inherited condition that is caused through mutation affecting the LYST (lysosomal trafficking regulator) gene. The condition is usually first recognised in childhood - causing oculocutaneous albinism, increased susceptibility to infections, and a bleeding tendency.
Consider also:
Pseudo Chédiak-Higashi granules that resemble Chédiak-Higashi granules may arise in acute leukemia, chronic myeloid leukemia, or myelodysplastic syndrome.
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