Volume 1, Issue 3 (Summer 1997)
J Arak Uni Med Sci 1997, 1(3): 34-36 |
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Hashemiyeh M. Tar Syndrome A Case Report. J Arak Uni Med Sci 1997; 1 (3) :34-36
URL: http://jams.arakmu.ac.ir/article-1-6531-en.html
URL: http://jams.arakmu.ac.ir/article-1-6531-en.html
Department of Pediatrics, Arak University of Medical Sciences, Arak, Iran.
Abstract: (1060 Views)
TAR syndromic is a congenital amegakoryecytic thrombocytopenia with Bilateral Absence of radii. Its inheritance is autosomal – recessive, Bilateral absence of radii manifests as a shortening of the forearms and flexion at the elbows; occasionally other limb abnormalities, such as phocomelia and radial deviation, are present. Other congenital anomalies are occasionally present, such as deformity of the digities, micrognathia, disocation of the hip and congenital heart disease. Purpura may present in first few days of life or may be delayed for weeks. Hemorrhagic manifestations range from a few petechiae to severe and even fatal intracranial hemorrhage. Platelet counts are usually in range of 10,000 – 30,000/mm3. Bone marrow examination reveals myeloid hyperplasia and almost total absence of megakaryocytes. Fifty percent of patients survive to 3 years. If patients survive beyond the first year, the platelet count stabilizes and the prognosis is such better. This condition is not premalignant. Treatment consists of transfusion of red cells for anemia and transfusion of platelet concentrates for severe bleeding from thrombocytopenia. Corticosteroids and splenectomy are of no constant benefit.
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