Aplastic anemia once a uniformly fatal disease, aplastic anemia is now curable with allogeneic transplantation in 80% of children and 40% of adults, and. Aplastic anemia is a haemotopoietic stemcell disorder that results in pancitopenia and hypocellular bone marrow. The pathophysiology of acquired aplastic anemia is immune mediated. Bone marrow transplantation compared with inmunosuppressive therapy. Fanconis anemia is a rare, inherited disease that leads to aplastic anemia. Anemia aplasica hematologia y oncologia manual msd version. We propose a diagnostic approach based on an algorithm from the basic hematologic data. According to its etiology, it can be congenital or acquired. This condition can lead to aplastic anemia, or aplastic anemia can evolve into paroxysmal nocturnal hemoglobinuria. Anemia aplastica, pancitopenia, transplante medula osea.
Abstract the basic pathophysiology of the hemolytic anemias is a reduced erythrocyte lifespan, then the bone marrow increases its output of erythrocytes and as a result the reticulocyte count often exceeds 2 percent. Pdf the diagnosis and treatment of aplastic anemia. Aplastic anemia is a rare disorder characterized by suppression of bone marrow function resulting in progressive pancytopenia. A triggerrelated abnormal t cell response facilitated by some genetic predisposition has been postulated as the pathogenetic mechanism leading to the overproduction of bone marrowinhibiting cytokines. Environmental exposures, such as drugs, toxins or viruses, are thought to trigger. Finally, it provides a short list of basic references. Anemia aplasica genetic and rare diseases information. Anemia aplasica hematologia y oncologia manual msd. The european group for blood and bone marrow transplantation experience.