Altered Self-reactive Antibody Repertoires are a General Feature of Patients with Myelodysplastic Syndrome

Stahl, Dulcelina A.; Egerer, Gerlinde; Goldschmidt, Hartmut; Sibrowski, W.; Kazatchkine, Michel D.; Kaveri, Srini V.

doi:10.1006/jaut.2000.0459

Cited by 7 publications

(7 citation statements)

References 33 publications

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“…Organ specific as well as immune-complexes and generic auto-antibodies (apart from antinuclear antibodies in a minority of patients) are classically undetectable [8]. A broad disturbance of self-recognition mechanisms seems to be a general feature of patients with MDS [10]. A deficiency in the regulation of self-reactivity may thus contribute to the disease pathogenesis [10].…”

Section: Discussionmentioning

confidence: 99%

“…A broad disturbance of self-recognition mechanisms seems to be a general feature of patients with MDS [10]. A deficiency in the regulation of self-reactivity may thus contribute to the disease pathogenesis [10]. Fortunately, these so-called paraneoplastic autoimmune complications mostly respond to immunosuppressive agents (steroids, cyclosporin) with the occasionally non-responsive syndromes contributing to early patient mortality [7], [8], [9].…”

Section: Discussionmentioning

confidence: 99%

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Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease

Hassan

Dar²

2011

GMS German Medical Science; 9:Doc27; ISSN 1612-3174

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease

Hassan

Dar²

2011

GMS German Medical Science; 9:Doc27; ISSN 1612-3174

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“…These patients show higher caspace-3 activity and lower TNF-α and IL-4 production. Analysis of the total IgM and IgG antibody repertoire in 10 MDS patients without prominent autoimmune disease or known autoantibody and in 10 healthy controls revealed different patterns of antibodies against selfantigens in MDS patients from those of controls, and patterns of IgG antibodies had distinct profiles implying disturbed self-recognition related to pathogenetic mechanisms of the disease [97].…”

Section: Immunopathogenetic Aspects Of Myelodysplastic Syndromesmentioning

confidence: 98%

Immune Dysregulation in Myelodysplastic Syndromes: Pathogenetic-Pathophysiologic Aspects and Clinical Consequences

Symeonidis¹,

Kouraklis-Symeonidis²

2016

Myelodysplastic Syndromes

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Myelodysplastic syndromes are clonal hematopoietic stem cell disorders, in which the immune system plays a substantial pathogenetic role. Patients manifest frequent infections, mainly attributed to neutropenia, but sometimes opportunistic pathogens are isolated in non-neutropenic patients. They also exhibit autoimmune diseases or syndromes with a background of immune activation and various abnormalities of Tlymphocytes, "-lymphocytes, and NK cells. The most typical profile includes reduced total T lymphocytes mainly CD + helper T-cells, resulting in decrease or inversion of the CD /CD cell ratio and impaired NK cell function. Many TH direction cytokines, and particularly sIL-R, IL-, and TNF-are usually found increased in the serum and bone marrow, which have been strongly associated with advanced disease, anemia, and other disease-related features. Clonal origin of lymphocytes has been confirmed only in few cases. Mixed lymphocyte cultures and genomic assays have shown severely impaired immunoregulatory abnormalities, probably induced by the hematopoietic cells. In a minority of patients, immune activation is capable to prevent or delay clonal expansion, but these patients have more profound hematopoietic impairment. Immunosuppressive treatment may not only relieve the autoimmune manifestations but also improve hematopoiesis. However, this kind of treatment is not well tolerated, is associated with severe infections, and in some cases may enhance "ML evolution.

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“…In contrast, when considering reactivity toward non–self‐antigens, plasmatic IgM of patients and healthy controls were similar, whereas IgG reactivity of patients and healthy subjects were different. These data clearly suggested a failure in the regulation of self‐reactivity in MDS patients . Antierythroid autoimmunity was then investigated in unstimulated and mitogen‐stimulated peripheral blood and bone marrow cultures of 26 patients with early MDS.…”

Section: Autoimmune Manifestations Involving Specific Sitesmentioning

confidence: 99%

“…These data clearly suggested a failure in the regulation of self-reactivity in MDS patients. 16 Antierythroid autoimmunity was then investigated in unstimulated and mitogen-stimulated peripheral blood and bone marrow cultures of 26 patients with early MDS.…”

Section: Autoimmune Haemolytic Anaemiamentioning

confidence: 99%

The burden of autoimmunity in myelodysplastic syndromes

Fozza

2017

Hematological Oncology

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The clinical history of patients with myelodysplastic syndromes (MDS) is characterised by bone marrow insufficiency as well as by the possible evolution into acute leukaemia. However a number of reports highlight the frequent occurrence of autoimmune manifestations involving different sites and organs. The present review will first describe the clinical pictures most often observed in MDS patients. The actual burden of autoimmunity will be then addressed by focusing on the few available registry studies. Finally, the potential collateral impact of specific treatments for MDS on the evolution of autoimmune disorders will be considered.

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Altered Self-reactive Antibody Repertoires are a General Feature of Patients with Myelodysplastic Syndrome

Cited by 7 publications

References 33 publications

Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease

Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease

Immune Dysregulation in Myelodysplastic Syndromes: Pathogenetic-Pathophysiologic Aspects and Clinical Consequences

The burden of autoimmunity in myelodysplastic syndromes

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