Ad. Gbadoe et al., ELECTIVE PHAGOCYTOSIS OF NEUTROPHILS BY M ARROW MACROPHAGES IN CHILDHOOD AUTOIMMUNE NEUTROPENIA, Archives de pediatrie, 4(5), 1997, pp. 398-405
Autoimmune neutropenia (AIN) is a frequent cause of chronic neutropeni
a especially in youngest children. Its diagnosis is established by imm
unological proof of the autoimmune mechanism. The aim of this study is
to better describe this autoimmune process and to show the contributi
on of bone marrow smears to this diagnosis. Patients and methods. - Te
n children, six girls and four boys, were examined between 1990 and 19
95. Eight of them had typical AIN, confirmed by the presence of antibo
dies against neutrophils. Two other patients were included on the basi
s of bone marrow pictures. Five non-neutropenic children with normal b
one marrow smears were chosen as controls. Bone marrow analysis was al
ways performed by the same cytologist according to a reproducible tech
nique. Results. - Six out of ten patients had important features of el
ective phagocytosis of neutrophils by marrow macrophages (unlike contr
ols) without signs of dysgranulopoiesis or hemophagocytosis. Antibodie
s against neutrophils were detected in six patients with phagocytosis
and in four patients without these cytological features. In two other
children presenting the same bone marrow picture and clinical profile,
an autoimmune process was probable, even in the absence of antibodies
against neutrophils. Some patients had several infections and were gi
ven immunoglobulins and/or granulocytes colony-stimulating factor (G-C
SF) therapy. The efficacy of Immunoglobulin was not constant, whereas
G-CSF was effective at low doses and shortened the duration of infecti
ons. Conclusion. - Prolonged neutropenia in childhood must lead to loo
k for phagocytosis by marrow macrophages in bone marrow smears, as a p
ossible sign of autoimmunity. Growth factors may temporarily be used a
ssociated with antibiotics therapy in severe and prolonged infections.