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Aplastic anemia [2] (AA) [3] is a severe hematologic condition in which the body fails to make blood cells in sufficient numbers. Blood cells are produced in the bone marrow by stem cells that reside there. [4] Aplastic anemia causes a deficiency of all blood cell types: red blood cells, white blood cells, and platelets. [5] [6]
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.
Red cell transfusions and corticosteroids are the cornerstones of Diamond-Blackfan anemia treatment since 1951. [ 12 ] Fanconi anemia (FA) is a genetically and phenotypically diverse recessive disorder that is characterized by a variety of congenital malformations , pancytopenia that progresses over time, and a susceptibility to solid tumors as ...
Individuals with TEC have a median age of presentation of 18–26 months; however, the disorder may occur in infants younger than 6 months and in children as old as age 10 years. Because of the gradual onset of the anemia, children are often healthier than expected from their low hemoglobin levels. [citation needed]
Aplastic anemia patients present with symptoms related to a decrease in hematopoietic cell production in the bone marrow. The onset is gradual, and the first symptom is frequently anemia or bleeding, though a high temperature or infections may be present at the onset. The following are examples of specific manifestations: [12]
Candidates for HSCTs include pediatric cases where the patient has an inborn defect such as severe combined immunodeficiency or congenital neutropenia with defective stem cells, and also children or adults with aplastic anemia [17] who have lost their stem cells after birth.
Aplastic anemia, paroxysmal nocturnal hemoglobinuria, myelodysplasia, leukemia Agranulocytosis , also known as agranulosis or granulopenia , is an acute condition involving a severe and dangerous lowered white blood cell count ( leukopenia , most commonly of neutrophils ) and thus causing neutropenia in the circulating blood. [ 1 ]
Other pre-existing bone-marrow disorders such as acquired aplastic anemia following immunosuppressive treatment and Fanconi anemia can evolve into MDS. [15] MDS is thought to arise from mutations in the multipotent bone-marrow stem cell, but the specific defects responsible for these diseases remain poorly understood.