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. 2018 Oct 22;14:65. doi: 10.1186/s13223-018-0272-7

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© The Author(s) 2018

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 1 — a Family pedigree: arrow indicates proband. Circles represent females, squares represent males; strikethrough represent deceased individuals, clinical conditions as outlined in key. b Flow cytometry of bone marrow aspirate demonstrating agranulocytosis with almost no cells in the granulocytic gate; lymphocytes are gated in green and blasts gated in blue. Bone marrow aspirate (c) and trephine (d) demonstrating a hypocellular marrow with adequate erythroid and megakaryocytic precursors but no maturing granulopoiesis. e Rapid resolution of tachycardia and fevers on commencement of systemic corticosteroids (green arrow). f Longitudinal neutrophil counts during course of admission with commencement of G-CSF (red arrow), corticosteroids (green arrow), oral methotrexate (blue arrow) and subcutaneous methotrexate (black arrow)