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Aplastic anemia is a rare, noncancerous disorder in which the blood marrow is unable to adequately produce blood cells required for survival. [44] [45] It is estimated that the incidence of aplastic anemia is 0.7–4.1 cases per million people worldwide, with the prevalence between men and women being approximately equal. [46]
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 ]
The prognosis without disease-modifying treatment is 10–20 years. [54] Many cases develop in people who have previously been diagnosed with myelodysplastic syndrome. The fact that PNH develops in MDS also explains why there appears to be a higher rate of leukemia in PNH, as MDS can sometimes transform into leukemia or aplastic anemia. [5]
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]
The mechanism of pancytopenia involves either haemopoiesis itself, decreasing blood cell productions in number (aplastic anemia), haemopoietic stem cells are displaced by malignant cells (Leukemia, lymphoma, MDS) or they are being pooled (sequestrated) (spleen)/destroyed (immune) outside bone marrow.
This is a life-threatening disorder that is a characteristic of aplastic anemia. [3] There are also two general causes of cytopenia: autoimmune and refractory. Autoimmune cytopenia is caused by an autoimmune disease when your body produces antibodies to destroy the healthy blood cells.
Researchers believe there may be a link between long-term vitamin D deficiency and the development of autoimmune conditions, such as type 1 diabetes and rheumatoid arthritis, but more research is ...
Long-term outcomes in patients with severe aplastic anemia treated with immunosuppression and eltrombopag: a phase 2 study. Blood. 2022 Jan 6;139(1):34-43. [ 16 ]
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