An unusual form of chronic neutropenia in a father and daughter with hypogammaglobulinaemia

WC Mentzer Jr, RB Johnston Jr… - British Journal of …, 1977 - Wiley Online Library
WC Mentzer Jr, RB Johnston Jr, RL Baehner, DG Nathan
British Journal of Haematology, 1977Wiley Online Library
Chronic neutropenia, with an abundance of mature neutrophils in the bone marrow, was
discovered in a father and daughter who also had common variable
hypogammaglobulinaemia. Under the stress of infection or following the administration of
typhoid vaccine, release of neutrophils from the marrow was enhanced sufficiently to abolish
neutropenia temporarily. Although their morphology was abnormal, the function of
neutrophils in vitro was normal. Thus, the increased susceptibility to infection that …
Chronic neutropenia, with an abundance of mature neutrophils in the bone marrow, was discovered in a father and daughter who also had common variable hypogammaglobulinaemia. Under the stress of infection or following the administration of typhoid vaccine, release of neutrophils from the marrow was enhanced sufficiently to abolish neutropenia temporarily. Although their morphology was abnormal, the function of neutrophils in vitro was normal. Thus, the increased susceptibility to infection that characterized these patients appeared to be due primarily to their defect in humoral immunity rather than their neutropenia.
Wiley Online Library