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Therapeutic apheresis
Published in Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond, 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.
The lymphoreticular system and bone marrow
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Most cases of neutropenia related to excessive destruction are immunologically mediated, because they may occur in systemic autoimmune diseases. Drugs, through haptenization, can also provoke autoimmune neutropenia. Other causes include overwhelming sepsis, where the formation of new granulocytes cannot keep pace with their consumption, and hypersplenism.
Immunopathology
Published in Constantin A. Bona, Francisco A. Bonilla, Textbook of Immunology, 2019
Constantin A. Bona, Francisco A. Bonilla
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.
Autoimmune disorders associated with common variable immunodeficiency: prediction, diagnosis, and treatment
Published in Expert Review of Clinical Immunology, 2022
Niloufar Yazdanpanah, Nima Rezaei
In patients who experience recurrent episodes of cytopenia, immunoglobulin replacement therapy (administered either IV or subcutaneous) has shown beneficial effects in reducing the risk of recurrence in some patients [192]. This is supported by the reports concerning the lower rates of cytopenia episodes after the periodic IVIG administration than the rate of same episodes before (or at the time of) CVID diagnosis [14]. For refractory cases, in contrary to ITP patients without CVID, splenectomy is not recommended in CVID patients due to the patient’s vulnerability to encapsulated bacteria after splenectomy. Respectively, pneumococcal sepsis and post-operative infections were reported in 10% and 16% of CVID patients after splenectomy [14]. As an alternative to splenectomy, immunomodulatory agents such as azathioprine and mycophenolate mofetil are beneficial in CVID patients. Meanwhile, rituximab has shown promising results as the second-line treatment for severe autoimmune cytopenia [193]. Regular periodic evaluation of the neutrophil count is strongly recommended in patients receiving rituximab to prevent the late-onset neutropenia, which is reported as a serious adverse effect following rituximab therapy, particularly in the first 5 month after the treatment [194]. In 2013, Wong and colleagues studied the outcome of splenectomy in 45 CVID patients. It turned out that splenectomy could be an appropriate long-term therapy in 75% of patients with confirmed CVID diagnosis associated with autoimmune cytopenia, even in cases that did not respond to rituximab. Interestingly, they reported no increased mortality rate attributed to splenectomy; they suggest a potential effect for IVIG in reducing the post-operative infections following splenectomy in CVID patients [195]. In addition, there are some novel treatment options available, including agonists of thrombopoietin receptor [196]. Neutropenia treatment in CVID is not extensively studied due to its lower prevalence. However, although the tapering might be challenging, high-dose IVIG could be prescribed in cases of absolute neutrophil count (ANC) <500/mm3. In patients that represent depletion of bone marrow reserves, granulocyte colony-stimulating factor (GCSF) could be useful; rituximab is also recommended in some cases of autoimmune neutropenia in CVID [188].