An update on acquired aplastic anemia

  title={An update on acquired aplastic anemia},
  author={Elena E Solomou},
  journal={Hematology \& Transfusion International Journal},
  • E. Solomou
  • Published 6 April 2017
  • Medicine
  • Hematology & Transfusion International Journal
Acquired Aplastic anemia is a rare bone marrow failure syndrome, characterised in most of the cases by autoimmune destruction of hematopoietic stem cells in the bone marrow [1]. The activated cytotoxic T cells in aplastic anemia display various abnormalities, leading to INF-g overproduction. This aberrant IFN-g levels lead to Fas-mediated stem cell death, resulting in replacement of the cellular component of the bone marrow by fat. The result of this replacement is pancytopenia in the… 



How I treat acquired aplastic anemia.

Patient age is a strong predictor of outcome for both IST and BMT, and must be considered when designing therapeutic strategies, as well as long-term monitoring for successful treatment of SAA.

Eltrombopag in aplastic anemia.

There is evidence that this drug works by directly stimulating marrow stem and progenitor cells thereby promoting hematopoietic recovery in patients with bone marrow failure, and several trials are ongoing in this institution using this very promising drug in combination therapy.

Current concepts in the pathophysiology and treatment of aplastic anemia.

  • N. Young
  • Medicine, Biology
    Hematology. American Society of Hematology. Education Program
  • 2013
This update emphasizes developments in the understanding of immune mechanisms and hematopoietic stem cell biology and new clinical approaches to stem cell stimulation as a therapy, alone and in combination with conventional suppression of the aberrant immune system.

Somatic Mutations and Clonal Hematopoiesis in Aplastic Anemia

The first updated data, which were collected in March 2015 with a median follow-up of 8.9 months, showed that the rate of pulmonary embolism remained low among patients treated with palbociclib, and these findings support the long-term safety of palbokiclib that was reported in the smaller PALOMA1 study.

T-bet, a Th1 transcription factor, is up-regulated in T cells from patients with aplastic anemia.

The data suggest that the increased IFN-gamma levels observed in aplastic anemia patients are the result of active transcription of the IFN -gamma gene by T-bet, which might lead to the development of novel therapeutic agents for patients with aplastics anemia and other autoimmune diseases.

Association of telomere length of peripheral blood leukocytes with hematopoietic relapse, malignant transformation, and survival in severe aplastic anemia.

In a cohort of patients with severe aplastic anemia receiving immunosuppressive therapy, telomere length was unrelated to response but was associated with risk of relapse, clonal evolution, and overall survival.

Th17 immune responses contribute to the pathophysiology of aplastic anemia.

T helper type 17 (Th17) cells have been characterized based on production of interleukin-17 (IL-17) and association with autoimmune diseases and contribute to aplastic anemia pathophysiology, especially at the early stage during disease progression.

Eltrombopag Added to Standard Immunosuppression for Aplastic Anemia Accelerates Count Recovery and Increases Response Rates

An investigator-initiated, phase II, single-center trial to test the efficacy of adding EPAG to h-ATG/CsA using a triple cohort design to optimize the regimen, which suggested that CR correlates with better survival and less evolution to MDS/AML.

Mutations in TERT, the gene for telomerase reverse transcriptase, in aplastic anemia.

The results of coexpression of wild-type TERT and TERT with aplastic anemia-associated mutations in a telomerase-deficient cell line suggested that haploinsufficiency was the mechanism of telomere shortening due to TERT mutations.

Aplastic anemia: pathophysiology and treatment

  • Dutcher
  • Medicine
    Medical oncology
  • 2000
Current thinking on the disease anemia classification etiology pathophysiology and lab guidelines for the diagnosis and management of aplastic anemia, pediatric aspects alphamed press the epidemiology of acquired aplastics anemia isdbweb in brief.