The patient is a 55-year-old woman with known chronic idiopathic thrombocytopenic purpura. The patient has been treated for this autoimmune disorder for approximately 1 year. The patient was previously seen in the office of her internal medicine specialist to discuss an elective splenectomy. The patient has failed to maintain a normal platelet count after a course of prednisone therapy. She also had major side-effect reactions to the prednisone medication. After hearing of the risks and benefits of the splenectomy versus another course of steroid therapy, the patient is admitted and consents to the surgery, a laparoscopic partial splenectomy. Assign diagnosis and procedure codes as needed.
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Immune thrombocytopenia (ITP) is a common hematologic disorder. Its pathogenesis involves both accelerated platelet destruction and impaired platelet production. First-line agents are usually effective initially but do not provide long-term responses. Splenectomy remains an effective long-term therapy, as does rituximab (Rituxan) in a subset of patients. Thrombopoietic agents offer a new alternative, although their place in the overall management of ITP remains uncertain.
Once regarded as idiopathic, immune thrombocytopenia (ITP) is now understood to have a complex pathogenesis, involving the evolution of antibodies against multiple platelet antigens leading to reduced platelet survival as well as impaired platelet production. For this reason, multiple therapies with different mechanisms of action are available to treat ITP, though not all of them are effective for individual patients.
In this article, I discuss the pathogenesis, demographics, manifestations, diagnosis, and management of ITP.
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