aplastic anemia

Pathol.

severe anemia due to destruction or depressed functioning of the bone marrow, usually resulting from bone cancer, radiation, or the toxic effects of certain drugs or chemicals.

[1930-35; A-⁶ + -PLASTIC]

* * *

or anemia of bone-marrow failure

Inadequate blood-cell formation by bone marrow.

Pancytopenia is the lack of all blood-cell types (erythrocytes, leukocytes, and platelets), but any combination may be missing. Drug, chemical, or radiation exposure most often causes the disease, but about half of all cases have no known cause. It may occur at any age. Acute disease may be quickly severe, even fatal; chronic disease has symptoms including weakness, shortness of breath, headache, fever, and pounding heart. There is usually a waxy pallor. Hemorrhages occur in mucous membranes, skin, and other organs. Lack of white blood cells lowers resistance to infection and becomes the major cause of death. Very low platelet count may lead to severe bleeding. The treatment of choice is bone-marrow transplantation. Otherwise treatment involves avoiding any known toxic agent and giving fluids, glucose, and proteins (often intravenously) as well as blood components and antibiotics.

* * *

▪ pathology

      disease in which the bone marrow fails to produce an adequate number of blood cells. There may be a lack of all cell types—white blood cells (leukocytes (leukocyte)), red blood cells (erythrocytes (erythrocyte)), and platelets (platelet)—resulting in a form of the disease called pancytopenia, or there may be a lack of one or more cell types. Rarely, the disease may be congenital (Fanconi anemia); more commonly, it is acquired by exposure to certain drugs (e.g., the antibiotic chloramphenicol) or chemicals (e.g., benzene) or to ionizing radiation. About half of all cases are idiopathic (cause unknown). Aplastic anemia is most common in persons 15 to 30 years of age. Onset of the disease may be abrupt, becoming quickly severe and possibly fatal; more commonly, it is insidious, running a chronic course of several years. Symptoms of chronic aplastic anemia include weakness and fatigue in the early stages, followed by shortness of breath, headache, fever, and pounding heart. There is usually a waxy pallor, and hemorrhages occur in the mucous membranes, skin, and other organs. If white blood cells (specifically, neutrophils) are lacking, resistance to infection is much lowered and infection becomes the major cause of death. When platelets are very low, bleeding may be severe.

      The treatment of choice for severe aplastic anemia is bone marrow transplantation (transplant), provided a compatible donor can be found. If transplantation is not practical, treatment involves avoidance of the toxic agent if known, supportive care (administration of fluids, glucose, and proteins, often intravenously), transfusions of blood components, and administration of antibiotics. Spontaneous recovery occurs occasionally.

* * *