Br J Haematol 2021 Aug 2. Epub 2021 Aug 2.
Registre des Neutropénies Chroniques, Centre de Référence des Neutropénies Chroniques, Hôpital Trousseau, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
Among 143 patients with elastase, neutrophil-expressed (ELANE)-related neutropenia enrolled in the French Severe Chronic Neutropenia Registry, 94 were classified as having severe chronic neutropenia (SCN) and 49 with cyclic neutropenia (CyN). Their infectious episodes were classified as severe, mild or oral, and analysed according to their natural occurrence without granulocyte-colony stimulating factor (G-CSF), on G-CSF, after myelodysplasia/acute leukaemia or after haematopoietic stem-cell transplantation. During the disease's natural history period (without G-CSF; 1913 person-years), 302, 957 and 754 severe, mild and oral infectious events, respectively, occurred. Read More