Lymphoma
Anju Sahdev, Sarah J. Vinnicombe in Husband & Reznek's Imaging in Oncology, 2020
In NHL, immunosuppression is a very important aetiological factor, as evidenced by the increased incidence in patients with AIDS or on long-term immunosuppressant therapy, e.g. following organ transplantation (12). The latter is associated with DLBCL and extranodal and CNS disease, but also HL, probably through the increased risk of EBV infection secondary to immunosuppression. Up to 25% of patients with congenital immunodeficiency syndromes such as ataxia-telangiectasia will develop malignancies, of which over 50% are lymphoproliferative disorders (including HL and EBV-driven NHL) (13). Non–organ-specific autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematous, and Sjogren's syndrome are associated with HL and DLBCL, whereas organ-specific autoimmune diseases predispose to the development of extranodal marginal zone lymphomas of MALT type within the affected organs (e.g. the thyroid and salivary glands). Coeliac disease is associated with enteropathy-associated T-cell lymphomas. Immune dysregulation is thought to underpin both autoimmune disease and development of NHL.
Medical Conditions and Diseases
Clete A. Kushida in Sleep Deprivation, 2004
One way of understanding the relationship between sleep and CFS is through the immune system. While the evidence for a link between these variables is growing, the complexity of this relationship needs further investigation. Indeed it is well established that sleep deprivation leads to altered immune markers (reviewed in Refs. 53,54). These alterations may be mediated by neuroendocrine changes or be a direct result of sleep loss. Alternatively, sleep disruption may be a consequence of an underlying pathophysiological mechanism, for example an immune or infectious anomaly. To illustrate, allergic rhinitis is common in individuals with CFS and is known to disrupt sleep (reviewed in Ref. 55). Fatigue therefore may be secondary to such an immune reaction. More specific examinations may include the study of cytokine activity during immune dysregulation. It has been theorized that cytokines may play a role in the increased fatigue and sleepiness experienced by CFS patients (53,56).
Molecular Biology and Gene Therapy
R James A England, Eamon Shamil, Rajeev Mathew, Manohar Bance, Pavol Surda, Jemy Jose, Omar Hilmi, Adam J Donne in Scott-Brown's Essential Otorhinolaryngology, 2022
The host immune response has been shown to play a role in cancer eradication. In addition to generalised suppression increasing the risk of carcinogenesis, it has been shown that individuals with head and neck cancer lack an effective local immune response even early in the disease. This dysfunction occurs as a result of the normal immune system not recognising the tumour cells. Causes of this include immunological ignorance, downregulation of major histocompatibility complexes and loss of costimulatory receptor and pathways. In some tumours, the cytokine stimulatory pathways (interleukin [IL]-2, interferon-gamma and IL-12) that normally upregulate the normal tumour immune response are suppressed. One method to break this immune dysregulation is to overexpress the downregulated cytokines.
The Clinical Utility of Autoantibodies in Patients with Idiopathic Granulomatous Mastitis
Published in Journal of Investigative Surgery, 2022
In conclusion, it is known that autoimmune diseases are the result of interaction between genetic factors, immune dysregulation and environmental trigger factors. To interpret correctly, for the presence of autoimmune disease related symptoms, a specific autoantibody test should be chosen. Instead of simultaneous testing for multiple autoantibodies, there seems a need for specific autoantibody test to detect may be newly developed molecules against breast lobules because our data show that autoantibodies such as RF, ANA, anti-ds-DNA, pANCA and anti-CCP do not contribute to clinical practice neither in diagnosis nor in follow up of patients with IGM. In order to use autoantibodies as fingerprints to identify subgroups of patients with different prognosis and different therapeutic responses, large datasets are required.
Allergic-like disorders and asthma in patients with common variable immunodeficiency: a multi-center experience
Published in Journal of Asthma, 2022
Limor Rubin, Oded Shamriz, Ori Toker, Ela Kadish, Yaarit Ribak, Aviv Talmon, Alon Y. Hershko, Yuval Tal
It is important to note that allergies and autoimmunity can both result from immune dysregulation. DNA sequencing has revealed genetic variation in the FOXP3 gene among children simultaneously suffering from allergic and autoimmune diseases (17). Accordingly, FOXP3 mutations reportedly lead to decreased Tregs or functional defects, such as in patients with immunodysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX), phenotypically expressed with autoimmune endocrinopathy, immune-mediated cytopenias, and allergic dysregulation, including food allergy and eczematous dermatitis (18). Atopy is also observed in other primary immune dysregulation disorders involving Tregs dysfunction, such as cytotoxic T-lymphocyte-associated protein (CTLA)-4 haploinsufficiency and LPS-responsive beige-like anchor protein (LRBA) deficiency (19).
Approaches to patients with variants in RAG genes: from diagnosis to timely treatment
Published in Expert Review of Clinical Immunology, 2019
Adeeb A. Bulkhi, Joseph F. Dasso, Catharina Schuetz, Jolan E. Walter
Specifically, a recent case series of 85 patients with RAG deficiencies and immune dysregulation highlighted that molecular diagnosis was delayed to a median of 5 years from the first clinical signs of immune dysregulation in this group [22]. The majority of patients presented with more than one complication indicative of immune dysregulation, with the most common etiologies being AIC (84.1%), granulomas (23.8%), and inflammatory skin disorders (19.0%). Infections, including live viral vaccinations, were an initiating trigger for autoimmunity in 36.5% of the cases. Similar findings were recently reported in a review that included 68 patients with CID-G/AI in which autoimmune cytopenia was the most common manifestation (53%) followed by other organ-specific autoimmunity [55]. Granulomas were identified in about 35% of the cases, and lung and skin were the commonly affected organs. Of note, these two cohorts that had shared patients as part of the data were based on previously published cases. Lastly, recently, several adult patients with RAG deficiencies have been reported with progressive inflammatory lung disease [21].
Related Knowledge Centers
- Enteropathy
- Mutation
- Regulatory T Cell
- Immunity
- Immune System
- Autoimmune Disease
- Cancer
- Ipex Syndrome
- Foxp3
- Transcription Factor