Dyshematopoiesis in combined immune deficiency with congenital neutropenia.

Journal Article (Journal Article)

This report describes a patient with combined immune deficiency associated with congenital neutropenia (CID/CN) and reports a partial characterization of his hematopoietic abnormalities. The CID/CN syndrome described is characterized by neutropenia and by deficiencies in B-lymphoid and T-lymphoid cell number and function. Red cell and platelet counts were normal. In vitro assays indicate that the myeloid lineage was developmentally arrested at the level of the committed monocyte/granulocyte progenitor (CFU-GM), while precursors to the CFU-GM progenitor were normal. In vitro studies showed that the defect in myeloid development was not corrected with G-CSF or GM-CSF. However, combinations of cytokines present in conditioned media from the T-cell lines MO or C5MJ, or defined multiple cytokine combinations containing IL-1, IL-3, GM-CSF, kit ligand, IL-6, and IL-9, restored myelopoiesis in-vitro. In contrast, C5MJ-conditioned media did not correct deficiencies in immune function in the patient's lymphocytes and accessory cells. No abnormalities in the production of G-CSF, GM-CSF, M-CSF, or IL-1 from the patient could be identified to account for the defects in myelopoiesis orimmune function.

Full Text

Duke Authors

Cited Authors

  • Gasparetto, C; Smith, C; Firpo, M; Dennig, D; Small, T; Gillio, AP; Lichtenberg, R; O'Reilly, RJ; Moore, MA

Published Date

  • January 1994

Published In

Volume / Issue

  • 45 / 1

Start / End Page

  • 63 - 72

PubMed ID

  • 8250011

International Standard Serial Number (ISSN)

  • 0361-8609

Digital Object Identifier (DOI)

  • 10.1002/ajh.2830450110


  • eng

Conference Location

  • United States