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Acquired aplastic anemia is a T-cell mediated autoimmune disease, in which regulatory T cells are decreased and T-bet, a transcription factor and key regulator of Th1 development and function, is upregulated in affected T-cells.
Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of aplastic anemia affecting the precursors to red blood cells but usually not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells. There are multiple etiologies that can cause PRCA. The condition has been first described by Paul Kaznelson in 1922. [1]
While acquired aplastic anemia with an unknown cause is rare, it is commonly permanent and life-threatening as half of those with this condition die within the first six months. [10] The prevalence of bone marrow failure is over three times higher in Japan and East Asia than in the United States and Europe. [10]
It is characterized by anemia and reticulocytopenia and can be chronic or acute. [3] Diamond–Blackfan anemia is a type of PRCA that occurs at birth. PRCA can be acquired as a primary disorder or as a result of another disorder. [4] Immunosuppressive drugs, particularly corticosteroids, will usually result in a temporary or permanent remission.
Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. [27] Autoimmune hemolytic anemia: D59.0-D59.1: Autoimmune hemolytic anemia (AIHA) is a type of hemolytic anemia where the body's immune system attacks its own red blood cells (RBCs), leading to their destruction .
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With isolated reticulocytopenia, the main cause is Parvovirus B19 infection of reticulocytes leading to transient anemia. [2] In patients who rely on frequent red cell regeneration e.g. sickle cell disease, a reticulocytopenia can lead to a severe anemia due to the cessation in red cell production (erythropoiesis), referred to as aplastic ...
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