Pathogenesis of aplastic anemia

Wang, Li; Liu, Hong

doi:10.1080/16078454.2019.1642548

Cited by 67 publications

(56 citation statements)

References 82 publications

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“…Furthermore, this result may even provide novel clues to the pathogenesis of AA. Some studies suggest that in addition to an abnormal immune system, other factors including telomere length, HLA or CAs, may trigger or be involved in the development of this disease (48)(49)(50), which may lead to diverse patterns of responsiveness to IST. Important future research includes large, multicenter clinical trials and laboratory research, in order to help fully elucidate the pathogenesis of AA.…”

Section: Discussionmentioning

confidence: 99%

Risk factors associated with poor response to immunosuppressive therapy in acquired aplastic anemia: A meta‑analysis of retrospective studies

Jia

Shen

et al. 2020

Exp Ther Med

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Acquired aplastic anemia (AA) is a rare hematological disease characterized by bone marrow hypocellularity and varying degrees of pancytopenia. Immunosuppressive therapy (IST) is currently one of the first-line treatments for AA; however, unresponsiveness remains a major concern. Although previous studies have suggested several common risk factors for unresponsiveness, there are currently no widely accepted predictors. Therefore, a meta-analysis of clinical trials including information on factors associated with unresponsiveness of AA to IST was performed in the present study. The PubMed, Embase and Cochrane Library databases were searched for clinical studies on AA evaluating the association between risk factors and unresponsiveness to IST. After the factors were defined from the selected studies, the association between these factors and unresponsiveness to IST was analyzed using Review Manager software. A total of 10 studies comprising 1,820 cases were included in the present meta-analysis. The following factors were identified as predictors of unresponsiveness: Age (≥60 years), sex, absolute neutrophil count, severity of the disease, paroxysmal nocturnal hemoglobinuria clone, human leukocyte antigen (HLA)-DR2 and cytogenetic abnormalities (CAs). Among these factors, only age (≥60 years) [odds ratio (OR)=1.65], HLA-DR2 negativity (OR=2.72) and CAs (OR=1.93) exhibited a statistically significant association with unresponsiveness to IST (P= 0.006, P= 0.04 and P=0.01, respectively). In conclusion, the present meta-analysis revealed that age ≥60 years, HLA-DR2 negativity and CAs are risk factors for unresponsiveness to IST. This result may enable clinicians to select an effective therapeutic scheme for patients with AA and even provide novel clues to the pathogenesis of AA.

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Section: Discussionmentioning

confidence: 99%

Risk factors associated with poor response to immunosuppressive therapy in acquired aplastic anemia: A meta‑analysis of retrospective studies

Jia

Shen

et al. 2020

Exp Ther Med

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“…An underlying stem cell abnormality has been postulated in patients with improved peripheral blood counts but persistent decreases in progenitor cells after treatment [28]. Genetic factors play an important role in the pathogenesis of AA with regard to uniparental disomy in 6p, somatic cell mutations, specific somatic mutation in HLA class I and class II genes, shorter telomerase with telomerase gene mutations and genetic susceptibility [8].…”

Section: Clonal Haematopoiesis Of Hematopoietic Stem Cellmentioning

confidence: 99%

“…The term 'bone marrow failure' is a broader concept, and the cause may not only be AA, but hypocellular myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Overall, AA can be divided into congenital and acquired with hematopoietic stem cells (HSC) immunemediated destruction [8]. The inherited forms are rare and mainly include; Fanconi anemia, congenital keratosis, congenital pure red cell aplasia and Schwachman-Diamond syndrome as a result of genetic lesions leading to diminish HSC's ability to repair DNA [9].…”

Section: Introductionmentioning

confidence: 99%

Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells

Brzeźniakiewicz‐Janus

Rupa‐Matysek

Gil

2020

Stem Cell Rev and Rep

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Aplastic anemia is rare disorder presenting with bone marrow failure syndrome due to autoimmune destruction of early hematopoietic stem cells (HSCs) and stem cell progenitors. Recent advances in newer genomic sequencing and other molecular techniques have contributed to a better understanding of the pathogenesis of aplastic anemia with respect to the inflammaging, somatic mutations, cytogenetic abnormalities and defective telomerase functions of HSCs. These have been summarized in this review and may be helpful in differentiating aplastic anemia from hypocellular myelodysplastic syndrome. Furthermore, responses to immunosuppressive therapy and outcomes may be determined by molecular pathogenesis of HSCs autoimmune destruction, as well as treatment personalization in the future.

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“…Aplastic anemia is a rare and serious blood disease characterized by a failure of the bone marrow to produce mature blood cells. In the majority of cases, this condition is caused by an autoimmune disorder in which the immune system, mainly T-cells, mistakenly attacks and destroys hematopoietic stem cells in the bone marrow leading to pancytopenia and bone marrow hypoplasia [1,2].…”

Section: Introductionmentioning

confidence: 99%

Epstein–Barr Virus-Induced Post-Transplant Lymphoproliferative Disorder of the Central Nervous System Successfully Treated with Chemo-Immunotherapy

Inoue

Rai

Tanaka

et al. 2020

Viruses

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Aplastic anemia is a rare blood disease characterized by the destruction of the hematopoietic stem cells (HSC) in the bone marrow that, in the majority of cases, is caused by an autoimmune reaction. Patients with aplastic anemia are treated with immunosuppressive drugs and some of them, especially younger individuals with a donor available, can be successfully treated with hematopoietic stem cell transplantation (HSCT). We report here a rare case of post-transplant lymphoproliferative disorder (PTLD) associated with Epstein–Barr virus (EBV) reactivation in a 30-year-old female patient who underwent allogeneic HSCT for severe aplastic anemia. The PTLD, which was diagnosed 230 days after transplantation, was localized exclusively in the central nervous system (specifically in the choroid plexus) and manifested with obvious signs of intracranial hypertension. After receiving three cycles of high dose methotrexate (HD-MTX) combined with rituximab, the patient achieved a complete clinical recovery with normalization of blood cell counts, no evidence of EBV reactivation, and no associated neurotoxicity.

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Pathogenesis of aplastic anemia

Cited by 67 publications

References 82 publications

Risk factors associated with poor response to immunosuppressive therapy in acquired aplastic anemia: A meta‑analysis of retrospective studies

Risk factors associated with poor response to immunosuppressive therapy in acquired aplastic anemia: A meta‑analysis of retrospective studies

Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells

Epstein–Barr Virus-Induced Post-Transplant Lymphoproliferative Disorder of the Central Nervous System Successfully Treated with Chemo-Immunotherapy

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