Human cyclic neutropenia occurs in children and adults. Adult-onset cyclic neutropenia is an acquired disease characterized by increased numbers of large granular lymphocytes (LGL), in contrast to childhood-onset cyclic neutropenia in which LGL counts are normal. We investigated the clonality of lymphocytes in these two groups of patients by assessing the rearrangement status of the T cell receptor beta chain gene. Patients with adult-onset cyclic neutropenia showed clonal rearrangement of the T beta gene whereas the children did not. Since LGL are known to have multiple regulatory effects on normal hematopoiesis, the finding of a clonal proliferation of this lymphocyte population implicates these cells in the pathogenesis of cyclic neutropenia.
Adult-onset cyclic neutropenia is a benign neoplasm associated with clonal proliferation of large granular lymphocytes.
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T P Loughran, W P Hammond; Adult-onset cyclic neutropenia is a benign neoplasm associated with clonal proliferation of large granular lymphocytes.. J Exp Med 1 December 1986; 164 (6): 2089–2094. doi: https://doi.org/10.1084/jem.164.6.2089
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