Pub Date : 2019-01-30DOI: 10.5772/INTECHOPEN.82532
Gamal Abdul Hamid, Abdul Wahab Al-Nehmi, S. Shukry
Myelodysplastic syndrome (MDS) is a clonal hematopoietic stem cell disorder characterized by morphological dysplastic changes in one or more of the major hematopoietic cell lines. MDS can present with varying degrees of single or multiple cytopenias including neutropenia, anemia and thrombocytopenia. Presentation of MDS can range from asymptomatic to life threatening. MDS diagnosis and classification present important challenges, particularly in the distinction from benign conditions. French-American-British (FAB) classification proposed a classification based on easily obtainable laboratory information and was recommended in early and as modified by guidelines of new classification of World Health Organization (WHO). The strategy of diagnostic laboratory in MDS depends on morphological changes and is based on existence of dysplastic changes in the peripheral blood and bone marrow including peripheral blood smear, bone marrow aspirate smear and bone marrow trephine biopsy. The correct morphological interpretation and the use of cytogenetics, immunophenotyping, immunohistochemistry and molecular analysis will give valuable information on diagnosis and prognosis.
{"title":"Diagnosis and Classification of Myelodysplastic Syndrome","authors":"Gamal Abdul Hamid, Abdul Wahab Al-Nehmi, S. Shukry","doi":"10.5772/INTECHOPEN.82532","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.82532","url":null,"abstract":"Myelodysplastic syndrome (MDS) is a clonal hematopoietic stem cell disorder characterized by morphological dysplastic changes in one or more of the major hematopoietic cell lines. MDS can present with varying degrees of single or multiple cytopenias including neutropenia, anemia and thrombocytopenia. Presentation of MDS can range from asymptomatic to life threatening. MDS diagnosis and classification present important challenges, particularly in the distinction from benign conditions. French-American-British (FAB) classification proposed a classification based on easily obtainable laboratory information and was recommended in early and as modified by guidelines of new classification of World Health Organization (WHO). The strategy of diagnostic laboratory in MDS depends on morphological changes and is based on existence of dysplastic changes in the peripheral blood and bone marrow including peripheral blood smear, bone marrow aspirate smear and bone marrow trephine biopsy. The correct morphological interpretation and the use of cytogenetics, immunophenotyping, immunohistochemistry and molecular analysis will give valuable information on diagnosis and prognosis.","PeriodicalId":405900,"journal":{"name":"Recent Developments in Myelodysplastic Syndromes","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124742900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-01-28DOI: 10.5772/INTECHOPEN.82101
Selma Z D'silva, S. Rajadhyaksha, Meenakshi Singh
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.
{"title":"Immune Dysregulation in MDS: The Role of Cytokines and Immune Cells","authors":"Selma Z D'silva, S. Rajadhyaksha, Meenakshi Singh","doi":"10.5772/INTECHOPEN.82101","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.82101","url":null,"abstract":"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.","PeriodicalId":405900,"journal":{"name":"Recent Developments in Myelodysplastic Syndromes","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123401227","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-11-27DOI: 10.5772/INTECHOPEN.82166
W. Chai-Ho, G. Schiller
Myelodysplastic syndromes (MDS) comprise a set of clonal hematopoietic stem cell (HSC) disorders characterized by ineffective hematopoiesis that manifest as cytopenia of variable severity. The result often is an increased risk of infection, transfusion dependence, and a potential to transform to acute myeloid leukemia (AML). For the past decade, hypomethylating agents remain the only FDA-approved therapy. Given that MDS is more prevalent in the elderly who often have comorbid conditions, supportive care remains the mainstay of therapy. Curative treatments are restricted to younger, healthy individuals with histocompatible-matched donors for allogeneic transplant able to tolerate more intensive chemotherapeutic treatment. Understanding of the pathophysiology of MDS advanced over the past decade, which leads to an increasing array of new agents under clinical investigation. This review focuses on our recent enhanced understanding of MDS molecular biology, and promising novel agents that go beyond the hypomethylating agent.
{"title":"Myelodysplastic Syndromes: An Update on Pathophysiology and Management","authors":"W. Chai-Ho, G. Schiller","doi":"10.5772/INTECHOPEN.82166","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.82166","url":null,"abstract":"Myelodysplastic syndromes (MDS) comprise a set of clonal hematopoietic stem cell (HSC) disorders characterized by ineffective hematopoiesis that manifest as cytopenia of variable severity. The result often is an increased risk of infection, transfusion dependence, and a potential to transform to acute myeloid leukemia (AML). For the past decade, hypomethylating agents remain the only FDA-approved therapy. Given that MDS is more prevalent in the elderly who often have comorbid conditions, supportive care remains the mainstay of therapy. Curative treatments are restricted to younger, healthy individuals with histocompatible-matched donors for allogeneic transplant able to tolerate more intensive chemotherapeutic treatment. Understanding of the pathophysiology of MDS advanced over the past decade, which leads to an increasing array of new agents under clinical investigation. This review focuses on our recent enhanced understanding of MDS molecular biology, and promising novel agents that go beyond the hypomethylating agent.","PeriodicalId":405900,"journal":{"name":"Recent Developments in Myelodysplastic Syndromes","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123758736","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-11-05DOI: 10.5772/INTECHOPEN.79767
A. Hruštincová, K. Szikszai, Z. Krejcík, Nikoleta Loudová, M. Merkerová
The discovery of short regulatory RNAs has recently directed the attention of scientists to parts of the genome that previously had been regarded as “ junk ” DNA because they did not encode protein products. The revelation that even protein-noncoding sequences had biological functions began the era of discovering the world of noncoding RNAs (ncRNAs). Of these ncRNAs, microRNAs (miRNAs) and long noncoding RNAs (lncRNAs) are the most numerous and best-known ncRNA groups. miRNAs and lncRNAs are important regulators of hematopoiesis, and their abnormal function has serious implications for phenotypes. Deregulation of these ncRNAs is found in hematopoietic disorders, and they also contribute to the development and progression of myelodysplastic syndromes (MDS). Properties of ncRNAs such as stability and tissue specificity make these molecules highly promising diagnostic and prognostic markers as well as interesting therapeutic targets. This chapter summarizes our knowledge on the contribution of ncRNAs to the pathogenesis of MDS and discusses their potential applicability in disease diagnos-tics and prognosis. H19-Igf2
{"title":"Noncoding RNAs in Myelodysplastic Syndromes","authors":"A. Hruštincová, K. Szikszai, Z. Krejcík, Nikoleta Loudová, M. Merkerová","doi":"10.5772/INTECHOPEN.79767","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.79767","url":null,"abstract":"The discovery of short regulatory RNAs has recently directed the attention of scientists to parts of the genome that previously had been regarded as “ junk ” DNA because they did not encode protein products. The revelation that even protein-noncoding sequences had biological functions began the era of discovering the world of noncoding RNAs (ncRNAs). Of these ncRNAs, microRNAs (miRNAs) and long noncoding RNAs (lncRNAs) are the most numerous and best-known ncRNA groups. miRNAs and lncRNAs are important regulators of hematopoiesis, and their abnormal function has serious implications for phenotypes. Deregulation of these ncRNAs is found in hematopoietic disorders, and they also contribute to the development and progression of myelodysplastic syndromes (MDS). Properties of ncRNAs such as stability and tissue specificity make these molecules highly promising diagnostic and prognostic markers as well as interesting therapeutic targets. This chapter summarizes our knowledge on the contribution of ncRNAs to the pathogenesis of MDS and discusses their potential applicability in disease diagnos-tics and prognosis. H19-Igf2","PeriodicalId":405900,"journal":{"name":"Recent Developments in Myelodysplastic Syndromes","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124105810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2018-11-05DOI: 10.5772/INTECHOPEN.80710
K. Newman, M. Akhtari, S. Heidarian
Myelodysplastic syndromes are heterogeneous group of clonal hematologic malignancies characterized by peripheral blood cytopenias secondary to the ineffective hematopoiesis. ADs are frequently reported in MDS, the incidence ranging from 10 to 30%, and particularly ADs are more frequently seen at CMML. ADs may prone patient to MDS, especially when immune suppressors such as azathioprine are used for the underlying AD. Both innate and adaptive immune systems, and different cytokines including interleukins, TNF- α , and C-X-C motif chemokine 10 (CXCL10) contribute in immune dysregulation of MDS. Vasculitis, seronegative rheumatoid arthritis, SLE, Behçet ’ s disease, RP, and AIHA are just some of the ADs occurring concomitantly with MDS. Although hematopoietic growth factors are recommended by the American Society of Clinical Oncology (ASCO), it has been recognized from several case reports that treatment of the underlying MDS may resolve the associated autoimmune disorders. The heterogeneity and complexity of pathology, clinical manifestations, response to therapy, and prognosis of MDS and its immune dysregulation make the prognosis of MDS with autoimmune diseases a matter of debate. Better understanding of the immune dysregulation of MDS in the molecular level may help to design prospective, double blind clinical trials to find the best treatment options for autoimmune disorders associated with MDS. vasculitis, chronic inflammatory demyelinating polyneuropathy (CIDP), neutrophilic dermatosis, Henoch-Schonlein purpura, relapsing polychondritis (RP), granulomatosis with polyangiitis (GPA), giant cell arteritis (GCA), polyarteritis nodosa (PAN)
{"title":"Myelodysplastic Syndrome and Autoimmune Disorders: Causal Relationship or Coincidence?","authors":"K. Newman, M. Akhtari, S. Heidarian","doi":"10.5772/INTECHOPEN.80710","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.80710","url":null,"abstract":"Myelodysplastic syndromes are heterogeneous group of clonal hematologic malignancies characterized by peripheral blood cytopenias secondary to the ineffective hematopoiesis. ADs are frequently reported in MDS, the incidence ranging from 10 to 30%, and particularly ADs are more frequently seen at CMML. ADs may prone patient to MDS, especially when immune suppressors such as azathioprine are used for the underlying AD. Both innate and adaptive immune systems, and different cytokines including interleukins, TNF- α , and C-X-C motif chemokine 10 (CXCL10) contribute in immune dysregulation of MDS. Vasculitis, seronegative rheumatoid arthritis, SLE, Behçet ’ s disease, RP, and AIHA are just some of the ADs occurring concomitantly with MDS. Although hematopoietic growth factors are recommended by the American Society of Clinical Oncology (ASCO), it has been recognized from several case reports that treatment of the underlying MDS may resolve the associated autoimmune disorders. The heterogeneity and complexity of pathology, clinical manifestations, response to therapy, and prognosis of MDS and its immune dysregulation make the prognosis of MDS with autoimmune diseases a matter of debate. Better understanding of the immune dysregulation of MDS in the molecular level may help to design prospective, double blind clinical trials to find the best treatment options for autoimmune disorders associated with MDS. vasculitis, chronic inflammatory demyelinating polyneuropathy (CIDP), neutrophilic dermatosis, Henoch-Schonlein purpura, relapsing polychondritis (RP), granulomatosis with polyangiitis (GPA), giant cell arteritis (GCA), polyarteritis nodosa (PAN)","PeriodicalId":405900,"journal":{"name":"Recent Developments in Myelodysplastic Syndromes","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132244713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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