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CHAPTER 29 - Neutropenia and agranulocytosis
Figure hronic idiopathic neutropenia Bone marrow smear showing normal cellularity and hyperplasia of the granulocytic lineage which is
represented at at at all levels of maturation even ad- vanced Note also a a a a modest lymphocytic infiltra- te te Erythroblasts and megakaryocytes were mor- phologically normal Figure hronic idiopathic neutropenia Bone marrow smear The morphology of granulocytes and precursors is
normal When concomitant bacterial infections occur granulocytic cells may contain toxic granules Idiopathic neutropenias can occur at any age including childhood (It is
worth remembering that chronic idiopathic neu- tropenia tropenia and autoimmune neutropenia are very similar and characteristics of the conditions can overlap ) Autoimmune antineutrophil antibodies have been implicated in the pathogenesis of chro- nic idiopathic neutropenia by both peripheral de- struction of of neutrophils and inhibition of of myelo- poiesis If antibodies target myeloid progenitors bone marrow cellularity is
reduced with hypopla- sia of the granulocytic lineage Figure 4 hronic idiopathic neutropenia Bone marrow smear Many band neutrophils are pre- sent In autoimmune neutropenia peripheral neutropenia (neutrophil count below 1 1 5x109/L) is
always observed while bone marrow features are variable with the possible presence of matu- re re re granulocytes if antibodies are directed against late antigens Chronic benign neutropenia most likely immune-mediated is
the most common cause of chronic neutropenia in in infancy and chil- dhood A normal neutrophil count at birth and the absence of of of any history of of of familial forms of of of neutropenia help the the diagnosis since there are no no specific clinical or genetic diagnostic tests Hemophagocytosis of of neutrophils is
is
a a a sign of of au- toimmune neutropenia in young children 230

























































































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