Immune dysregulation in myelodysplastic syndrome: Clinical features, pathogenesis and therapeutic strategies

Wang, Cong; Yang, Yan; Gao, Sujun; Chen, Jingcheng; Yu, Jinyuan; Zhang, Han; Li, Mingxi; Zhan, Xingying; Li, Wei

doi:10.1016/j.critrevonc.2017.12.013

Cited by 69 publications

(67 citation statements)

References 113 publications

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“…Recent reviews have described the multifaceted roles of the aforementioned cytokines in MDS and ineffective hematopoiesis. 107,108 Targeting innate immune pathways in MDS…”

Section: Cytokinesmentioning

confidence: 99%

Chronic immune response dysregulation in MDS pathogenesis

Barreyro

Chlon

Starczynowski

2018

Blood

179

166

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Chronic innate immune signaling in hematopoietic cells is widely described in myelodysplastic syndromes (MDS), and innate immune pathway activation, predominantly via pattern recognition receptors, increases the risk of developing MDS. An inflammatory component to MDS has been reported for many years, but only recently has evidence supported a more direct role of chronic innate immune signaling and associated inflammatory pathways in the pathogenesis of MDS. Here we review recent findings and discuss relevant questions related to chronic immune response dysregulation in MDS.

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“…Recent reviews have described the multifaceted roles of the aforementioned cytokines in MDS and ineffective hematopoiesis. 107,108 Targeting innate immune pathways in MDS…”

Section: Cytokinesmentioning

confidence: 99%

Chronic immune response dysregulation in MDS pathogenesis

Barreyro

Chlon

Starczynowski

2018

Blood

179

166

View full text Add to dashboard Cite

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“…This Fas receptor when bound to its cognate Fas ligand triggers apoptosis of the cell carrying the Fas receptor via the Fas associated death domain (FADD). Hence higher levels of TNF-alpha have been associated with rate of apoptosis in MDS patients [27].…”

Section: Role Of Cytokines In Immune Dysregulation In Mdsmentioning

confidence: 99%

“…IFN-gamma and IL-6 are involved in apoptosis induction in BM. Hence, when they are present in higher levels, it will result in a low risk MDS [27,32]. + glycoprotein is to recognize and destroy foreign/infectious particles.…”

Section: Role Of Cytokines In Immune Dysregulation In Mdsmentioning

confidence: 99%

“…Regulatory T cells (Tregs) cells are CD4 + CD25 high FOXP3 + or CD4 + CD25 high CD127 low . Tregs carry out immune tolerance as well as modulation of immune response and are the important for immune surveillance [27] by secreting IL10 and TGF-Beta which are immunosuppressive cytokines [78]. Any impairment in Tregs results in defective immune activation and decreased immune surveillance against the tumor cells [26].…”

Section: Regulatory T Cellsmentioning

confidence: 99%

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Immune Dysregulation in MDS: The Role of Cytokines and Immune Cells

D’Silva¹,

Rajadhyaksha²,

Singh³

2019

Recent Developments in Myelodysplastic Syndromes

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Myelodysplastic syndrome (MDS) is a hematopoietic stem cell disorder affecting individuals over the age of 60 years. It is characterized by ineffective hematopoiesis and extensive apoptosis of hematopoietic cells. MDS patients are at a high risk of transforming in to acute myeloid leukemia. The main cause of apoptosis and escape from immune surveillance in MDS is immune dysregulation caused by a number of factors such as aberrant cytokine production and influence of various immune cells. In the past decade various pro-inflammatory cytokines and a number of immune cells such as Natural Killer cells, regulatory T cells, cytotoxic T cells, mesenchymal stem cells, myeloid derived suppressor cells and dendritic cells have been implicated in immune dysregulation leading to MDS pathogenesis. In this review we focus on the current data available on the role of these immune factors.

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“…Chronic myelomonocytic leukemia (CMML), initially categorized within MDS, is currently classified as myelodysplastic/myeloproliferative neoplasm [1,3]. There is a correlation between MDS/CMML and the presence of a broad spectrum of autoimmune manifestations (AIM) [4][5][6][7][8][9]. Patients with autoimmune diseases have an increased risk for the development of MDS with an odds ratio of 1.5 to 3.5, and clinically apparent AIM occur in 5-35% of the MDS, while up to 60% of MDS patients may have serological signs of autoimmunity [8,9].…”

Section: Introductionmentioning

confidence: 99%

Vasculitis in Myelodysplastic Syndrome and Chronic Myelomonocytic Leukemia: A Report of Two Cases

et al. 2018

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There is a clear association between myelodysplastic syndrome (MDS)/chronic myelomonocytic leukemia (CMML) and autoimmune manifestations such as vasculitis. It is not clear if autoimmune manifestations in myelodysplastic syndrome are a cause or consequence. We describe two patients with polyarteritis nodosa and large vessel vasculitis, as presenting symptom of a myelodysplastic syndrome with excess blasts type 2 and chronic myelomonocytic leukemia respectively. Immunosuppressive treatment resulted in amelioration of the vasculitis with improvement of the myelodysplastic features in the first patient and rapid evolution to acute myeloid leukemia in the other patient. The association between MDS/CMML and autoimmune manifestations, such as vasculitis, emphasizes the role of autoimmunity in the clinical features and even pathogenesis of MDS/CMML.

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Immune dysregulation in myelodysplastic syndrome: Clinical features, pathogenesis and therapeutic strategies

Cited by 69 publications

References 113 publications

Chronic immune response dysregulation in MDS pathogenesis

Chronic immune response dysregulation in MDS pathogenesis

Immune Dysregulation in MDS: The Role of Cytokines and Immune Cells

Vasculitis in Myelodysplastic Syndrome and Chronic Myelomonocytic Leukemia: A Report of Two Cases

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