Page 239 - Haematologica Atlas of Hematologic Cytology

CHAPTER 28 - Qualitative disorders of granulocytes
ABC DEF
GHI
Figure 1 Peripheral blood film in in Chédiak-Higashi syndrome (CHS) A particular feature of this inherited di- sorder is the presence of typical giant granules granules in leukocyte cytoplasm Pseudo Chédiak-Higashi granules granules can occasionally be observed in in myeloid elements in in a a a a a a a a few acquired hematologic disorders such as as acute leukemia and and myeloproliferative and and myelodysplastic syndromes However the abnormal granulation in acquired condi- tions only affects a a a a a a few cells cells as opposed to the involvement of all white blood cells cells in in CHS CHS CHS CHS giant granules are particularly evident and numerous in neutrophils (A-E) In these cells they derive from the the the fusion of azu- rophilic granules granules in in in early precursors with subsequent incorporation of some specific granules granules in in in the myelocyte stage The mature neutrophils therefore contain both the the abnormal azurophilic granules and a a a a a a a a a a a variable number of of normal specific granules granules Concentration of of digestive enzymes in in giant granules granules is is reduced and this results in in defective bactericidal function of CHS neutrophils Giant granules are observed also in in eosinophils (F) basophils lymphocytes (E G H) and monocytes (I) In mononuclear cells the the number of granules is usually small Note the the different colorability of the the the granules in in the the the polymorphonuclear cells cells and in in the the the mononuclear cells cells which reflects their different content CHS may also show abnormalities of nuclear lobation (hyposegmentation) in granulocytes
(panels A D and F) 226