Immunosuppression therapy is effective for both acquired tumor-associated and primary pure red cell aplasia: a match pair case-control study

Chen, Zesong; Yang, Chen; Han, Bing

doi:10.1007/s00277-020-04105-3

Cited by 4 publications

(1 citation statement)

References 18 publications

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“…The use of recombinant human erythropoietin (EPO) can lead to PRCA by the formation of autoantibodies to endogenous EPO [ 14 ]. PRCA can also evolve in the context of T-Large Granular Lymphocyte Leukemia (T-LGL) and there is an association between the presence of a thymoma and PRCA [ 15 , 16 , 17 ]. If no concomitant treatable disease has been found, acquired PRCA is termed idiopathic.…”

Section: Atg For Acquired Pure Red Cell Aplasiamentioning

confidence: 99%

Anti Thymocyte Globulin-Based Treatment for Acquired Bone Marrow Failure in Adults

Tjon

Langemeijer

Halkes

2021

Cells

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Idiopathic acquired aplastic anemia can be successfully treated with Anti Thymocyte Globulin (ATG)-based immune suppressive therapy and is therefore considered a T cell-mediated auto immune disease. Based on this finding, several other forms of idiopathic acquired bone marrow failure are treated with ATG as well. For this review, we extensively searched the present literature for evidence that ATG can lead to enduring remissions in different forms of acquired multi- or single-lineage bone marrow failure. We conclude that ATG-based therapy can lead to an enduring hematopoietic response and increased overall survival (OS) in patients with acquired aplastic aplasia. In patients with hypocellular myelodysplastic syndrome, ATG can lead to a hematological improvement without changing the OS. ATG seems less effective in acquired single-lineage failure diseases like Pure Red Cell Aplasia, Amegakaryocytic Thrombocytopenia and Pure White Cell Aplasia, suggesting a different pathogenesis in these bone marrow failure states compared to aplastic anemia. T cell depletion is hypothesized to play an important role in the beneficial effect of ATG but, as ATG is a mixture of polyclonal antibodies binding to different antigens, other anti-inflammatory or immunomodulatory effects could play a role as well.

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