Thrombotic thrombocytopenic purpura medical therapy: Difference between revisions
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==Medical Therapy== | ==Medical Therapy== | ||
===Plasmapheresis=== | ===Plasmapheresis=== | ||
Since the early 1990s, [[plasmapheresis]] has become the treatment of choice for TTP.<ref>{{cite journal |author=Zheng XL, Kaufman RM, Goodnough LT, Sadler JE |title=Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura |journal=Blood |volume=103 |issue=11 |pages=4043–9 |year=2004 |pmid=14982878 |doi=10.1182/blood-2003-11-4035}}</ref> This is an [[exchange transfusion]] involving removal of the patient's [[blood plasma]] through [[apheresis]] and replacement with donor plasma ([[fresh frozen plasma]] or cryosupernatant); the procedure has to be repeated daily to eliminate the inhibitor and ablate the symptoms. Plasma infusion may be necessary and preferred if plasma exchange is not readily available. Exchange tranfusion has a complete response rate of 76%; plasma exchange allows ~80% of acquired ADAMTS13 autoantibody mediated TTP patients to survive an episode(compared to ~80% mortality without it), usually with minimal organ damage. FFP is the replacement fluid of choice in TTP and an exchange of a single plasma volume is the standard initial treatment. All patients should receive folate. [[Lactate dehydrogenase]] levels are generally used to monitor disease activity. The serum level increases as erythrocytes are destroyed. Plasmapheresis may need to be continued for 1-8 weeks before patients with idiopathic TTP cease to consume platelets and begin to normalize their hemoglobin. No single laboratory test (platelet count, LDH, ADAMTS13 level, or inhibitory factor) is indicative of recovery; research protocols have used improvement or normalization of LDH as a measure for ending plasmapheresis. Although patients may be critically ill with failure of multiple organ systems during the acute illness, including renal failure, myocardial ischemia, and neurologic symptoms, recovery over several months may be complete in the absence of a frank myocardial infarct, stroke, or CNS hemorrhage. Platelet transfusions are contraindicated unless there is life-threatening hemorrhage. | Since the early 1990s, [[plasmapheresis]] has become the treatment of choice for TTP.<ref>{{cite journal |author=Zheng XL, Kaufman RM, Goodnough LT, Sadler JE |title=Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura |journal=Blood |volume=103 |issue=11 |pages=4043–9 |year=2004 |pmid=14982878 |doi=10.1182/blood-2003-11-4035}}</ref> This is an [[exchange transfusion]] involving removal of the patient's [[blood plasma]] through [[apheresis]] and replacement with donor plasma ([[fresh frozen plasma]] or cryosupernatant); the procedure has to be repeated daily to eliminate the inhibitor and ablate the symptoms. Plasma infusion may be necessary and preferred if plasma exchange is not readily available. Exchange tranfusion has a complete response rate of 76%; plasma exchange allows ~80% of acquired ADAMTS13 autoantibody mediated TTP patients to survive an episode(compared to ~80% mortality without it), usually with minimal organ damage. FFP is the replacement fluid of choice in TTP and an exchange of a single plasma volume is the standard initial treatment. All patients should receive folate. [[Lactate dehydrogenase]] levels are generally used to monitor disease activity. The serum level increases as erythrocytes are destroyed. Plasmapheresis may need to be continued for 1-8 weeks before patients with idiopathic TTP cease to consume platelets and begin to normalize their hemoglobin. No single laboratory test (platelet count, LDH, ADAMTS13 level, or inhibitory factor) is indicative of recovery; research protocols have used improvement or normalization of LDH as a measure for ending plasmapheresis. Although patients may be critically ill with failure of multiple organ systems during the acute illness, including renal failure, myocardial ischemia, and neurologic symptoms, recovery over several months may be complete in the absence of a frank myocardial infarct, stroke, or CNS hemorrhage. Platelet transfusions are contraindicated unless there is life-threatening hemorrhage. | ||
Children with Upshaw-Schulman syndrome receive plasma every three weeks prophylactically; this maintains adequate levels of functioning ADAMTS13. | |||
===Pharmacotherapy=== | ===Pharmacotherapy=== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical Therapy
Plasmapheresis
Since the early 1990s, plasmapheresis has become the treatment of choice for TTP.[1] This is an exchange transfusion involving removal of the patient's blood plasma through apheresis and replacement with donor plasma (fresh frozen plasma or cryosupernatant); the procedure has to be repeated daily to eliminate the inhibitor and ablate the symptoms. Plasma infusion may be necessary and preferred if plasma exchange is not readily available. Exchange tranfusion has a complete response rate of 76%; plasma exchange allows ~80% of acquired ADAMTS13 autoantibody mediated TTP patients to survive an episode(compared to ~80% mortality without it), usually with minimal organ damage. FFP is the replacement fluid of choice in TTP and an exchange of a single plasma volume is the standard initial treatment. All patients should receive folate. Lactate dehydrogenase levels are generally used to monitor disease activity. The serum level increases as erythrocytes are destroyed. Plasmapheresis may need to be continued for 1-8 weeks before patients with idiopathic TTP cease to consume platelets and begin to normalize their hemoglobin. No single laboratory test (platelet count, LDH, ADAMTS13 level, or inhibitory factor) is indicative of recovery; research protocols have used improvement or normalization of LDH as a measure for ending plasmapheresis. Although patients may be critically ill with failure of multiple organ systems during the acute illness, including renal failure, myocardial ischemia, and neurologic symptoms, recovery over several months may be complete in the absence of a frank myocardial infarct, stroke, or CNS hemorrhage. Platelet transfusions are contraindicated unless there is life-threatening hemorrhage.
Children with Upshaw-Schulman syndrome receive plasma every three weeks prophylactically; this maintains adequate levels of functioning ADAMTS13.
Pharmacotherapy
Many TTP patients need additional immunosuppressive therapy, with glucocorticoid steroids (e.g. prednisolone or prednisone), vincristine, cyclophosphamide, splenectomy or a combination of the above. Rituximab, a monoclonal antibody targeting CD20 on B cells, has been successfully used to treat patients with refractory disease.
References
- ↑ Zheng XL, Kaufman RM, Goodnough LT, Sadler JE (2004). "Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura". Blood. 103 (11): 4043–9. doi:10.1182/blood-2003-11-4035. PMID 14982878.