Therapeutic apheresis
Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond in Transfusion Medicine in Practice, 2020
Affinity adsorption apheresis involves the selective extraction of immunologic or non-immunologic substances from the circulation by means of a column, with the benefit of returning non-extracted proteins, such as clotting factors, to the patient. The column contains a sorbent or ligand attached to a carrier (sepharose or silica) to which patient plasma is exposed. Such columns include the Staphylococcus protein A columns, which adsorb immune complexes and immunoglobulins via their Fc portion (with greatest affinity for IgG subclasses 1, 2, and 4, and lower affinity for IgG3, IgM, and IgA).9 The use of such columns invokes an immunomodulation that is presently poorly understood. They are approved for clinical use in the USA for the treatment of idiopathic thrombocytopenic purpura (ITP) and, most recently, refractory rheumatoid arthritis. However, they have been used anecdotally to treat a variety of other immune-mediated medical conditions, such as clotting factor inhibitors, platelet refractoriness, and autoimmune neutropenia. Alternatively, affinity adsorption columns composed of dextran sulfate cellulose columns are used for the removal of low-density lipoproteins.
Immunohematology
Gabriel Virella in Medical Immunology, 2019
Human granulocytes are known to have five sets of human neutrophil antigens. Antibodies to one or more of these antigens can result in immune neutropenia. Alloimmune neutropenia can be seen in neutrophil incompatible fetal-maternal conditions and post blood or bone marrow transfusions. Primary autoimmune neutropenia is caused by autoantibodies to neutrophil antigens and is usually seen in children. Secondary autoimmune neutropenia may be associated with a variety of autoimmune diseases, including patients with rheumatoid arthritis, usually in association with splenomegaly (Felty's Syndrome), and also with some lymphoproliferative disorders. Treatment includes administration of granulocyte stimulating factor and immunosuppression when indicated.
Immunopathology
Constantin A. Bona, Francisco A. Bonilla in Textbook of Immunology, 2019
As mentioned above, other cytopenias may also be the result of autoantibody production. Idiopathic thrombocytopenic purpura (ITP) is caused by anti-platelet autoantibodies. These are directed mainly against the surface glycoprotein heterodimer gpIIb/IIIa (CD41/CD61 β3 integrin, see Chapter 10). Autoimmune neutropenia is caused by antibodies reactive with neutrophil surface antigens.
Chronic neutropenia: how best to assess severity and approach management?
Published in Expert Review of Hematology, 2021
Jean Donadieu, Stephanie Frenz, Lauren Merz, Flore Sicre De Fontbrune, Gioacchino Andrea Rotulo, Blandine Beaupain, Martin Biosse-Duplan, Marie Audrain, Laure Croisille, Phil Ancliff, Christoph Klein, Christine Bellanné-Chantelot
This entity usually corresponds to a benign condition diagnosed in young adults and has some similarities to both autoimmune neutropenia in children and the so-called ethnic neutropenia. There are three distinguishing features: it occurs in young adults (in practice, from puberty onwards); is usually profound with < 0.5 x 109/L [48,49] and can be thought of as a primary autoimmune neutropenia in young adults; is frequently associated with other organ-specific autoimmune diseases (autoimmune thyroiditis, pernicious anemia, etc.). Unlike primary autoimmune neutropenia in children, in adults it is more persistent (usually several years or even life-long) with a female predominance (80% women). Bone marrow evaluation is mandatory to exclude other diagnoses but there is not typical pattern: most of the patients have normal marrow, left deviation or terminal maturation blocking but a few have also hypoplastic granular maturation. In addition, this idiopathic neutropenia is different from chronic neutropenia associated with other autoimmune pathologies [49], such as systemic lupus erythematosus, rheumatoid arthritis/Felty syndrome, Gougerot-Sjögren syndrome [50], Mixed connective tissue disease sometimes associated with LGL [51–53], and those of common variable immunodeficiency [54].
The evaluation of neutropenia in common variable immune deficiency patients
Published in Expert Review of Clinical Immunology, 2019
Mohammad Ghorbani, Saba Fekrvand, Sepideh Shahkarami, Reza Yazdani, Mahsa Sohani, Mohammadreza Shaghaghi, Gholamreza Hassanpour, Javad Mohammadi, Babak Negahdari, Hassan Abolhassani, Asghar Aghamohammadi
The underlying heterogeneous pathogenesis of CVID complications is not fully understood [1]. The affected patients present with various immunologic profiles and abnormalities. Although decreased serum level of switched immunoglobulins is the most frequent manifestation among CVID patients, neutropenia could manifest in some of these patients. Few previous studies have been conducted on neutropenia in CVID patients and the majority of reports have indicated neutropenia just as a rare complication in CVID [30–32]. In our study, 8.1% of 220 CVID patients had persistent (recurrent or chronic) neutropenia, which is somewhat higher than what is reported in previous studies. For example, in a study performed by Resnik et al. only 1% of the total 473 CVID patients had neutropenia with autoimmune subtype [31]; in another study autoimmune neutropenia was reported in 1.1% of 224 CVID patients during follow-up [32]. This difference is probably due to the different definition of neutropenia applied in our study design compared to others. Both of the aforementioned studies only reported the frequency of autoimmune neutropenia rather than persistent neutropenia, which consists of a few subsets. Only in one cohort study, different etiologies were considered for neutropenia consequently with a slightly higher reported frequency of neutropenia (3.4% of 473 CVID patients) compared to the other mentioned studies, however even in this study autoimmune neutropenia accounted for the most of neutropenic episodes with a relatively similar frequency compared to other studies (11 of total 16 neutropenic cases, or 2.3% of the total 473 CVID patients) [30]. However different population and underlying genetic predispositions could play a significant role in a higher rate of neutropenia in our cohort as well.
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