Cytochemical and Ultrastructural Studies of Aberrant Granules in the Neutrophils of Two Patients with Myeloperoxidase Deficiency during a Preleukemic State

The Morphology of Dyserythropoiesis in a Patient with Acute Erythroleukaemia Associated with Multiple Myeloma

“…Additional evidence can probably be derived from the presence of a labyrinth in the cytoplasm of bone marrow myeloid cells. These structures, originally described in guinea pig peritoneal macrophages (Brederoo & Daems 1972) were recently found in myeloid cells in cases of myelocytic leukaemia and preleukaemia (Machino & Shimamura 1973, Breton-Gorius 1976.…”

Partial Myeloperoxidase Deficiency

“…The presence of partial MPO deficiency in preleukemia has been previously de scribed [16][17][18][19][20], but its true frequency has never been systematically evaluated. This study shows the high occurrence of partial MPO deficiency in preleukemia, the only group in our series with significantly re duced MPO scores (p < 0.005) in compari son to the reference group.…”

“…A par tial deficiency of MPO has been described in heterozygous subjects [3,4], and in asso ciation with various clinical situations as an apparently acquired form. These include pregnant women [5], neonates [6], infec tious diseases [7,8], Hodgkin's disease and disseminated cancers [9], myeloid metapla sia [10], chronic granulocytic leukemia [11], acute myeloid leukemia [12][13][14][15], preleuke mia [16][17][18][19][20], neuronal lipofuscinosis [21], lead intoxication [22] and during adminis tration of certain drugs [23], The frequency of partial MPO deficiency and the physiol ogic importance of this enzyme has still not been well defined. In the present study, we determined MPO scores of PMNs in various clinical situations and examined if infectious manifestations occur more frequently in partial MPO deficiency.…”

Defective neutrophil function and microbicidal mechanisms in the myelodysplastic disorders.

“…Neutropenia is common and hypogranularity and Pelger-Huet-like changes are frequently observed in blood films. ' 2 An increased susceptibility to infection which is not necessarily simply a reflection of neutropenia (if present) has been noted and neutrophil dysfunction suggested as an underlying cause.34 The data substantiating this are sparse but clear-cut bactericidal and candidacidal defects have been described, notably by Breton-Gorius et al 4 ' Although there are other reports of clinically significant microbicidal defects in such patients,5-8 the numbers investigated have been small.…”