Aplastic anemia is a normochromic normocytic anemia, which is caused by the loss of progenitor cells and the resulting hypoplasia of the bone marrow, erythrocytes, leukocytes and platelets. The symptoms are an expression of severe anemia, thrombocytopenia (petechiae, bleeding) or leukopenia (infections). For the diagnosis of peripheral pancytopenia and hypoplasia / aplasia must be detected in the bone marrow. The treatment consists of the administration of antithymocyte the horse and cyclosporine. Note / Editor’s correction: granulocyte colony-stimulating factor and bone marrow transplantation provide further treatment options are!
The term “aplastic anemia” usually refers to a tri-linear hypoplasia in the bone marrow with concomitant neutropenia and thrombocytopenia. In contrast, the isolated aplastic anemia (pure red cell aplasia = PRCA) relates exclusively to the red cell series. Both diseases are rare, but aplastic anemia is more common.
Aplastic anemia is a normochromic normocytic anemia, which is caused by the loss of progenitor cells and the resulting hypoplasia of the bone marrow, erythrocytes, leukocytes and platelets. The symptoms are an expression of severe anemia, thrombocytopenia (petechiae, bleeding) or leukopenia (infections). For the diagnosis of peripheral pancytopenia and hypoplasia / aplasia must be detected in the bone marrow. The treatment consists of the administration of antithymocyte the horse and cyclosporine. Note / Editor’s correction: granulocyte colony-stimulating factor and bone marrow transplantation provide further treatment options are! The term “aplastic anemia” usually refers to a tri-linear hypoplasia in the bone marrow with concomitant neutropenia and thrombocytopenia. In contrast, the isolated aplastic anemia (pure red cell aplasia = PRCA) relates exclusively to the red cell series. Both diseases are rare, but aplastic anemia is more common. Etiology About half of the cases of aplastic anemia (most common in adolescents and young adults) is idiopathic. Known triggers include chemicals (e.g., benzene, inorganic arsenic), ionizing radiation and drug (eg. As cytostatics, antibiotics, NSAIDs, anti-convulsants, acetazolamide, gold salts, penicillamine, quinacrine). The underlying mechanism is not known, but a selective hypersensitivity appears to be, which is probably genetic. Fanconi anemia is a rare, familial form of aplastic anemia with bone abnormalities, microcephaly, hypogonadism, and brown pigmentation of the skin. It occurs in children with chromosomal abnormalities. The Fanconi anemia runs often clinically unapparent. Only by the occurrence of other diseases (in particular acute infections or inflammatory diseases) in the formation of a peripheral cytopenia. After healing of the disease, the peripheral levels return to normal, even though the bone marrow mass is further reduced. The isolated aplastic anemia (PRCA) may occur as an acute and reversible event. In acute aplastic anemia isolated is a short-term disappearance of erythrocyte precursors in the bone marrow during various acute viral infections, in particular infections with human parvovirus. It is especially common in children. The anemia usually lasts longer than the acute infection. Chronic isolated aplastic anemia may be associated with hemolytic disease, thymoma and autoimmune diseases. Rare it is drug-induced (tranquilizers, anticonvulsants), toxins (organic phosphates), riboflavin or chronic lymphocytic leukemia caused. A rare congenital form is the so-called Diamond Blackfan anemia. They usually occurs in young children, but has been described already in adulthood. Often found in the Diamond Blackfan anemia short stature and bone abnormalities on the thumb and fingers. Symptoms and signs The onset of aplastic anemia is usually insidious and often manifests weeks or months after exposure to the pollutant. But occasionally acute progressive forms occur. The clinical signs vary depending on the severity of the pancytopenia. In most cases, severe symptoms and clinical signs of anemia show (z. B. pallor). Severe thrombocytopenia can petechiae and bruising and bleeding of mucous membranes, leading into the conjunctiva or other tissues. Agranulocytosis often causes life-threatening infections. Splenomegaly usually does not exist, unless it is caused by transfusional hemochromatosis. The symptoms of aplastic anemia are usually insulated weaker and depend on the extent of anemia or the underlying disease. Diagnostic blood bone marrow examination Suspected aplastic anemia must in patients, especially young patients with pancytopenia (eg., Leukocytes <1500 / uL, platelets <50,000 / ul) are provided. In patients with bone abnormalities and normozytärer anemia, but normal white blood cell and platelet counts an isolated aplastic anemia may be suspected (including Diamond-Blackfan anemia). There is a suspicion of this diagnosis, a bone marrow examination should be performed. In aplastic anemia, the red blood cells normochromic normocytic (sometimes geringgradig macrocytic) are. The leukopenia is primarily due to a decrease of the neutrophils. The platelet levels are well below 50,000 / ul. The reticulocytes are decreased or absent. The serum iron is increased. The bone marrow is acellular. At isolated aplastic anemia normocytic anemia with reticulocytopenia and elevated serum iron is present, however, the leukocyte and platelet counts are normal. Cellularity and maturity levels in the bone marrow can - apart from the absence of red cell progenitors - be normal. Therapy equine antithymocyte globulin, corticosteroids and cyclosporin Occasionally, hematopoietic stem cell transplantation Occasionally cytokines Occasionally surgery for thymoma-associated aplastic anemia iisolierter The treatment of choice for aplastic anemia is administration of equine antithymocyte globulin (ATG) in combination with cyclosporine. (Editor's note: A dose should not be provided here, as this will change depending on the preparation, and is usually administered in specialized clinics Thus, for example, is currently not treated for 10 days...) (Editor's note: A dose should not be here be given as this will change depending on the preparation and is applied in specialized clinics. So) is z. B. not currently over 10 days treated. about 60% of patients respond to the administration of antithymocyte on. Allergic reactions and serum sickness may occur. Therefore, skin testing is recommended in order to prevent allergies to horse serum and an accompanying corticosteroid (prednisone 40 mg / m2 p.o. once daily, starting on day 7 for 10 days or until remission of symptoms). Ciclosporin (5-10 mg / kg p.o. once daily) has a similar efficacy as anti-thymocyte globulin. It is at about 50% of patients who do not respond to anti-thymocyte globulin is effective, which is why a different mechanism of action is adopted. The combination therapy of anti-thymocyte globulin and cyclosporine has been shown to be effective. In very severe cases of aplastic anemia or failure of treatment with anti-thymocyte globulin and cyclosporine a bone marrow transplant can be effective. Hematopoietic stem cell transplantation is a treatment option for younger patients (especially <30 years), but requires an identical twin or HLA-compatible donor. Therefore, brothers and sisters are already being tested for HLA compatibility at diagnosis. Since transfusions increase the risk of a subsequent transplantation, blood products are used only where essential. The isolated aplastic anemia with immunosuppressive drugs (eg. As prednisone, cyclosporine or cyclophosphamide) can be successfully treated, especially if an autoimmune mechanism is suspected. Because patients benefit with a thymoma-associated aplastic anemia isolated from a thymectomy, should be sought by computed tomography after a Thymusvergrößerung and optionally performed an operation. Summary aplastic anemia includes a tri-linear hypoplasia in the bone marrow with neutropenia and thrombocytopenia. Many cases are idiopathic, but triggers can also chemicals, drugs or ionizing radiation to be. Manifestations include marked pallor and weakness, petechiae, bruising and mucosal bleeding and serious infections. When carried out usually bone marrow aplasia investigation shows. Administration of equine antithymocyte globulin (ATG) and / or cyclosporin; Corticosteroids may be necessary to control allergic reactions to anti-thymocyte globulin. Bone marrow transplantation may be necessary.