Blood neutrophil function in primary myelodysplastic syndromes

Abstract

Ten different tests of blood neutrophil function were studied in 20 patients with primary myelodysplastic syndromes (PMDS). The patients were selected according to the new diagnostic criteria for PMDS of the FAB-cooperation group. Impairments of granulocyte functions were found in all patients. Moreover, several steps in the mobilization of granulocytes at the site of injury seemed to be affected: decreased adhesion (P less than 0.05), deficient chemotaxis (P less than 0.05), decreased enzyme content (P less than 0.001), 'slower' chemiluminescence (P less than 0.005), decreased phagocytosis (P less than 0.05) and impaired microbicidal capacity (P less than 0.025). No significant correlation between disease category and severity of granulocyte dysfunction was discerned, though an increasing number of blasts was associated with more severe granulocytic disability. Results in seven patients with abnormal karyotypes were not significantly different from 13 others with normal karyotypes. Our results indicate that defects in blood neutrophil function are a common feature in PMDS and might account for the increased frequency of infection in these patients.

Proposals for the classification of the myelodysplastic syndromes

“…In 392 Understanding the Myelodysplastic Syndromes Cytopenias 0/1 2/3 (Hemoglobin <10 g%, absolute neutrophil count (ANC)<1,800/µl, platelet count <100,000/µl. the remaining patients, the majority will succumb to infection in part from neutropenia (in one series, 60% of MDS patients had a neutrophil count <2,500/µl [48]), and in part from granulocyte dysfunction, as the MDS clone can have impairment in phagocytic adhesion, chemotaxis, and microbicidal killing [49,50]. This may also account for slowly resolving abscesses as well as the lack of fever, with such patients often just manifesting malaise [51].…”

The growth of myelodysplastic bone marrow in long-term cultures

“…Even in the absence of leukaemic transformation, MDS are lethal haematological disorders (Weisdorf et al, 1983) with high mortality from infection and bleeding due not only to the cytopaenias but also to defective function of the neutrophils (Boogaerts et al, 1983) and platelets (Russel et al, 1979). Although the development of an acute leukaemia is not obligatory and the time of appearance varies considerably (Coiffier et al, 1985, Greenberg, 1983, MDS is the only human model of leukaemogenesis that can be investigated in detail.…”

Síndromes mieolodisplásicos.

“…Boogaerts (20), reporta que además de alteraciones morfológicas, los granulocitos presentan alteraciones funcionales como una adhesión disminuida, quimiotaxis deficiente, menor contenido enzimático, fagocitosis disminuida y capacidad germicida anormal. Esto podía explicar el porque de infecciones severas en dos de nuestros pacientes que tuvieron granulocitos en número normal, uno de los cuales falleció por una neumonía severa con solo cuatro días de enfermedad.…”

“…Giralt (10) y Pomeroy (30), también encuentran que las infecciones respiratorias son las principales causas de infecciones pero con un porcentaje menor al nuestro (53.8 y 23% respectivamente). El 50% de los pacientes que presentaron alguna infección tuvieron neutrófilos en un número mayor a 1000/mm 3 , lo que nos haría suponer que el riesgo de infección no solamente es debida a la neutropenia sino también a disfunción del neutrófilo (20).…”