A Rare Case of Adult Autoimmune Neutropenia Successfully Treated with Prednisolone

Abstract

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.

Phagocytosis of Mature Granulocytes by Bone Marrow Macrophages in an Elderly Man with Adult-Onset Primary Autoimmune Neutropenia

“…Interestingly, this exacerbation of neutropenia was successfully improved by continuous low-dose prednisolone therapy over fourteen days, in accordance with the previous reports [ 14 , 15 , 16 , 17 ], but not by short-term dexamethasone therapy. Immunosuppressant agents have been used for G-CSF refractory AIN [ 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ].…”

“…Interestingly, this exacerbation of neutropenia was successfully improved by continuous low-dose prednisolone therapy over fourteen days, in accordance with the previous reports [ 14 , 15 , 16 , 17 ], but not by short-term dexamethasone therapy. Immunosuppressant agents have been used for G-CSF refractory AIN [ 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ]. Successful treatment with a single use of corticosteroid or combination with cyclosporine has been reported in several cases of AIN with [ 14 , 15 , 18 ] or without [ 16 , 17 , 18 , 19 , 20 ] other lineages of cytopenia, such as autoimmune hemolytic anemia or idiopathic thrombocytopenic purpura.…”

Two Cases of Autoimmune Neutropenia Complicated with Other Lineages of Autoimmune Cytopenia, Successfully Treated with Prednisolone

“…Although a standard treatment strategy for AIN has not yet been established, treatments with G-CSF or steroids are commonly reported (2,(6)(7)(8). To compare the responses to steroid treatment between adult primary AIN cases both with and without other lineages of autoimmune cytopenia, a review of the literature was performed.…”

“…To compare the responses to steroid treatment between adult primary AIN cases both with and without other lineages of autoimmune cytopenia, a review of the literature was performed. We initially focused on adult primary AIN without other lineages of cytopenia, and using PubMed search, we found only seven case reports of primary AIN that had been treated with steroid-containing immunosuppressive agents (Table 3) (6)(7)(8)(9)(10)(11)(12). This is because, for cases of primary AIN, G-CSF is recommended as a firstline treatment, and high response rates have been reported (2).…”

Human neutrophil antigens: Nature, clinical significance and detection

“…Autoimmune neutropenia (AIN), resulting from autoantibody, is less commonly observed in adults with antibody specificity less clearly defined. AIN is more commonly a secondary condition due to a number of other immunological, viral, bacterial or malignant conditions (Akhtari, Curtis, & Waller, 2009;Fujita et al, 2017). transfusion (Lucas, 2018).…”