Isolated neutropenia without concomitant anemia or thrombocytopenia is a common clinical problem seen by primary care physicians and hematologists. The etiologies of neutropenia vary from transient suppression by self-limited viral illnesses to previously undetected congenital syndromes to serious systemic diseases. The clinical significance likewise ranges from amild laboratory abnormalitywith...

The patient was diagnosed in childhood as having severe congenital neutropenia and had recurrent admissions with severe infections. In 1987, prior to getting married, she was sterilized. She continued to require i.v. antibiotics when she contracted a severe infection. On one occasion, she was treated with growth colony stimulating factor (G-CSF). Her increased neutrophil count was sustained fol...

In a recent publication in Blood, Donini et al1 conclude that granulocyte colony-stimulating factor (G-CSF) treatment of severe congenital neutropenia (SCN) results in abnormal expression of granule-associated proteins. We have previously published this concept,2 and showed that neutrophils from patients with SCN lack the antimicrobial peptide LL-37 and have reduced levels of defensins (HNP1–3)...

Methods The HRF panel includes MEFV, MVK, NLRP3, TNFRSF1A, PSTPIP1, LPIN2 and ELANE, which are associated with familial Mediterranean fever (FMF), hyper-IgD syndrome, cryopyrin-associated periodic syndrome, tumor necrosis factor receptor-associated periodic syndrome (TRAPS), pyogenic sterile arthritis-pyoderma gangrenosum and acne syndrome, Majeed syndrome, and cyclic/severe congenital neutrope...

Neutrophil extracellular traps (NETs) are involved in bacterial killing as well as autoimmunity, because NETs contain proteases, bactericidal peptides, DNA and ribonucleoprotein. NETs are formed via a novel type of cell death called NETosis. NETosis is distinct from apoptosis, but it resembles necrosis in that both membranes are not intact so that they allow intracellular proteins to leak outsi...