Primary autoimmune neutropenia in adults: case report and review of the literature

Mariotti, Jacopo; Caberlon, Sabrina; Bertinato, Elena; Podda, Gian Marco; Pugliano, Mariateresa; Cattaneo, Marco

doi:10.1111/trf.12711

Cited by 11 publications

(12 citation statements)

References 18 publications

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“…Marrow examination is of great help in excluding hematologic malignancy (e.g., acute leukemia and myelodysplastic syndrome). Additionally, as observed in our case, deficient mature neutrophils, irrespective of a normal or increased number of myeloid progenitors, is the typical marrow finding of AIN . Our patient was finally diagnosed with primary AIN, since no underlying etiologies were found, including collagen diseases, infection, medication, or hematologic malignancy.…”

Section: Discussionsupporting

confidence: 73%

“…In one retrospective study of 240 cases, the major infection sites were the upper respiratory tract (19%), auris media (17%), and skin (12%), although 28 patients (12%) experienced severe infections such as pneumonia, sepsis, or meningitis . Episodes of mild infection in severely neutropenic patients are considered an important clue that suggest the presence of AIN . The presumable reason for the lower incidence of infection in AIN patients is that the marrow reserve of myeloid precursors is generally preserved, which allows for activation of the innate immune response at infection sites when necessary .…”

Section: Discussionmentioning

confidence: 99%

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Adult‐onset primary cyclic autoimmune neutropenia: a case report

et al. 2018

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Section: Discussionsupporting

confidence: 73%

Section: Discussionmentioning

confidence: 99%

Adult‐onset primary cyclic autoimmune neutropenia: a case report

et al. 2018

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“…A PubMed search found only four case reports (Table 2) (6, 18-20). In most AIN cases, the administration of G-CSF (5 μg/kg daily) was effective, and the effect was observed within a few days (7).…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Adult Autoimmune Neutropenia Successfully Treated with Prednisolone

et al. 2017

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Autoimmune neutropenia (AIN) is a rare disorder that may cause life-threatening infections. In adults, most cases are secondary to other pathological conditions, and primary AIN is extremely rare. We herein report a case involving a 57-year-old woman diagnosed with AIN. A granulocyte immunofluorescence test detected autoantibodies against human neutrophil antigens in her serum, while various examinations revealed no other causes of neutropenia, suggesting her AIN was primary. She was refractory to granulocyte-colony-stimulating factor but responded to prednisolone. Her neutrophil count remained normal after gradual discontinuation of prednisolone. Diagnostic procedures and optimal treatments for this disorder need to be established.

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“…Neutropenia, caused by chemotherapy, HIV infection or autoimmune disorders, predisposes patients to P . aeruginosa pneumonia [13–15]. Only patients with the full defensive arsenal of PMNs are able to defeat P .…”

Section: Introductionmentioning

confidence: 99%

Swimming Motility Mediates the Formation of Neutrophil Extracellular Traps Induced by Flagellated Pseudomonas aeruginosa

et al. 2016

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Pseudomonas aeruginosa is an opportunistic pathogen causing severe infections often characterized by robust neutrophilic infiltration. Neutrophils provide the first line of defense against P. aeruginosa. Aside from their defense conferred by phagocytic activity, neutrophils also release neutrophil extracellular traps (NETs) to immobilize bacteria. Although NET formation is an important antimicrobial process, the details of its mechanism are largely unknown. The identity of the main components of P. aeruginosa responsible for triggering NET formation is unclear. In this study, our focus was to identify the main bacterial factors mediating NET formation and to gain insight into the underlying mechanism. We found that P. aeruginosa in its exponential growth phase promoted strong NET formation in human neutrophils while its NET-inducing ability dramatically decreased at later stages of bacterial growth. We identified the flagellum as the primary component of P. aeruginosa responsible for inducing NET extrusion as flagellum-deficient bacteria remained seriously impaired in triggering NET formation. Purified P. aeruginosa flagellin, the monomeric component of the flagellum, does not stimulate NET formation in human neutrophils. P. aeruginosa-induced NET formation is independent of the flagellum-sensing receptors TLR5 and NLRC4 in both human and mouse neutrophils. Interestingly, we found that flagellar motility, not flagellum binding to neutrophils per se, mediates NET release induced by flagellated bacteria. Immotile, flagellar motor-deficient bacterial strains producing paralyzed flagella did not induce NET formation. Forced contact between immotile P. aeruginosa and neutrophils restored their NET-inducing ability. Both the motAB and motCD genetic loci encoding flagellar motor genes contribute to maximal NET release; however the motCD genes play a more important role. Phagocytosis of P. aeruginosa and superoxide production by neutrophils were also largely dependent upon a functional flagellum. Taken together, the flagellum is herein presented for the first time as the main organelle of planktonic bacteria responsible for mediating NET release. Furthermore, flagellar motility, rather than binding of the flagellum to flagellum-sensing receptors on host cells, is required for P. aeruginosa to induce NET release.

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Primary autoimmune neutropenia in adults: case report and review of the literature

Abstract: Primary AIN should be considered early in the diagnostic process of isolated neutropenia, to avoid expensive and time-consuming unnecessary diagnostic procedures.

Cited by 11 publications

References 18 publications

Adult‐onset primary cyclic autoimmune neutropenia: a case report

Adult‐onset primary cyclic autoimmune neutropenia: a case report

A Rare Case of Adult Autoimmune Neutropenia Successfully Treated with Prednisolone

Swimming Motility Mediates the Formation of Neutrophil Extracellular Traps Induced by Flagellated Pseudomonas aeruginosa

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