Idiopathic neutropenia: Antineutrophil antibodies and clinical correlations

Logue, Gerald L.; Shastri, Kaushik A.; Laughlin, Mary; Shimm, David S.; Ziolkowski, L; Iglehart, Janice L.

doi:10.1016/0002-9343(91)80162-f

Cited by 38 publications

(37 citation statements)

References 23 publications

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“…1 Severe chronic primary neutropenia (CPN) in adults includes true idiopathic neutropenia (CIN), primary autoimmune neutropenia defined by the presence of neutrophil autoantibodies, and CPN associated with a T-cell clone (excluding large granular lymphocyte leukemia). [2][3][4][5][6][7][8][9][10] So far, there is no evidence based on clinical characteristics and outcome to consider these entities as different.…”

Section: Introductionmentioning

confidence: 99%

Severe chronic primary neutropenia in adults: report on a series of 108 patients

Fontbrune

Moignet

Beaupain

et al. 2015

Blood

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International audienceSevere chronic primary neutropenia (CPN) is a rare entity, and long-term outcome and risk factors for infections in severe CPN adults have not been described to date. We report the characteristics and outcomes of 108 severe adult CPN patients enrolled in a multi-institutional observational study. Severe CPN adults were mostly female (78%), and median age at diagnosis was 28.3 years. Diagnosis was fortuitous in 62% of cases. The median absolute neutrophil count (ANC) at diagnosis was 0.4 × 109/L, and median ANC without granulocyte colony-stimulating factor (G-CSF) during follow-up was 0.5 × 109/L. Twenty-three of 66 (34.8%) evaluable patients had neutrophil autoantibodies, and 6 of 47 (12.8%) a T-cell clone. The presence of neutrophil autoantibodies or T-cell clone was not associated with any specific clinical or biological characteristics. No death or hematologic malignancies occurred, and 44 severe bacterial infections were reported in 27 patients with a median follow-up of 8.3 years. Fifty patients received G-CSF either sporadically (n = 24) or continuously (n = 26) and responded (96%). Nineteen patients received immunosuppressive therapies: overall response (OR) was 41%, and median duration of response was 3 months. At diagnosis, the only predictive factor for the occurrence of severe bacterial infections was an ANC count below 0.2 × 109/L (OR, 0.76). Severe CPN in adults is characterized by a female predominance and a benign outcome with a low rate of severe bacterial infections and no secondary malignancies. G-CSF is efficient and well tolerated but is not required in a majority of patient

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Section: Introductionmentioning

confidence: 99%

Severe chronic primary neutropenia in adults: report on a series of 108 patients

Fontbrune

Moignet

Beaupain

et al. 2015

Blood

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show abstract

Section: Introductionmentioning

confidence: 99%

“…Severe chronic neutropenia can be divided into congenital forms of neutropenia, cyclic neutropenia, and an ill-defined category of disorders including chronic idiopathic neutropenia and autoimmune neutropenia [4,16,21]. In primary autoimmune neutropenia (AIN) the neutropenia is the sole immunological abnormality, while in secondary AIN other immunological disorders are present [1,16,21]. In some of these patients AIN is associated with autoimmune hemolytic anemia, autoimmune thrombocytopenia, or a combination of both, with a roughly equal distribution among the three groups [16,21].…”

Section: Introductionmentioning

confidence: 99%

“…In primary autoimmune neutropenia (AIN) the neutropenia is the sole immunological abnormality, while in secondary AIN other immunological disorders are present [1,16,21]. In some of these patients AIN is associated with autoimmune hemolytic anemia, autoimmune thrombocytopenia, or a combination of both, with a roughly equal distribution among the three groups [16,21].…”

Section: Introductionmentioning

confidence: 99%

“…A number of anti-neutrophil antibody detection methods are available, the direct and indirect granulocyte immunofluorescence test (GIFT) being the most sensitive and the most widely used [1,22,23]. With this method immunoglobulin G (IgG) antibodies, mostly of the subclasses IgG 1 and IgG 3 , may be detected in patients with primary as well as with secondary AIN, sometimes together with IgM and IgA [1,16,21]. Several classes of neutrophil-specific antigens have been identified, which are present on mature myeloid cells, but also on myelocytes, promyelocytes, and even myeloblasts [1].…”

Section: Introductionmentioning

confidence: 99%

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G-CSF-induced decrease of the anti-granulocyte autoantibody levels in a patient with autoimmune granulocytopenia

Vlasveld

Haas

Ermens

et al. 1997

Annals of Hematology

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Disclaimer/Complaints regulationsIf you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: http://uba.uva.nl/en/contact, or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible. Abstract We present a case of an 81-year-old man with secondary autoimmune granulocytopenia in association with autoimmune thrombocytopenia. Treatment with granulocyte colony-stimulating factor (G-CSF) (5 mg/ kg/day s.c.) resulted in a rapid increase in the number of circulating granulocytes with a pronounced left shift. These changes were accompanied by up-regulation of the surface expression of FcgRI (CD64) and FcgRII (CD32) on the granulocytes. In addition, we noted a strong up-regulation of the FcgRIII (CD16) and the activation markers CD11b and CD66b on the granulocytes. The increase in the number of circulating granulocytes was followed by a dramatic decrease in the level of cell-bound as well as circulating anti-granulocyte antibodies. It is hypothezised that the decrease in the level of cell-bound as well as circulating anti-granulocyte antibodies may be the result of an increased adsorption of the antibodies by the granulocytes.

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Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry

Farruggia¹,

Fioredda

Puccio

et al. 2018

American J Hematol

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Autoimmune neutropenia of infancy (AIN) is characterized by low risk of severe infection, tendency to spontaneously resolve and typically onset at ≤4-5 years of age; it is due to auto-antibodies whose detection is often difficult. In case of negativity of 4 antineutrophils Justification of the high number of authors: this is a registry study and a lot of researchers and physicians contributed to the enrollment of patients and data analysis.

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Idiopathic neutropenia: Antineutrophil antibodies and clinical correlations

Cited by 38 publications

References 23 publications

Severe chronic primary neutropenia in adults: report on a series of 108 patients

Severe chronic primary neutropenia in adults: report on a series of 108 patients

G-CSF-induced decrease of the anti-granulocyte autoantibody levels in a patient with autoimmune granulocytopenia

Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry

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