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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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