A 21 year old female with a several month history of easy bruising and increased menstrual flow is evaluated for a bleeding disorder. Her platelet count is 31,000/mm3. Subsequent investigations determine that she has idiopathic thrombocytopenic purpura (ITP). In this disorder, the low platelet count is due to which of the following?
ITP is a chronic autoimmune disorder in which antibodies against platelet glycoproteins cause platelet destruction and removal by the reticuloendothelial system. Secondary thrombocytopenia can also be produced by lupus, viral infections, and drugs. Only when secondary thrombocytopenia has been ruled out, can the diagnosis of ITP be made. Defective platelet aggregation is responsible for thrombasthenia, an autosomal dominant disease that causes prolonged bleeding time but normal numbers of platelets. Hypersplenism causes thrombocytopenia when an enlarged spleen traps normal platelets in the absence of other specific platelet disorders. This type of thrombocytopenia can be cured with splenectomy. Although the thrombocytopenia in ITP often improves with splenectomy, ITP does not cause splenomegaly. Megakaryopoiesis is disturbed in any disorder that causes bone marrow failure, including drug toxicity, leukemia, and infections. In this setting, thrombocytopenia is often pa of a pancytopenia. Ref: Diz-Kucukkaya R., Chen J., Geddis A., Lopez J.A. (2010). Chapter 119. Thrombocytopenia. In J.T. Prchal, K. Kaushansky, M.A. Lichtman, T.J. Kipps, U. Seligsohn (Eds), Williams Hematology, 8e.
Antiplatelet antibodies
Defective platelet aggregation
Hypersplenism
Ineffective megakaryopoiesis