Microangiopathic hemolytic anemia medical therapy
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Associate Editor(s)-in-Chief: Mydah Sajid, MD[1]
- Microangiopathic hemolytic anemia is a medical emergency and requires prompt treatment.
- Therapeutic plasmapheresis is the treatment of choice. It should be instituted immediately in patients with high clinical suspicion of microangiopathic hemolytic anemic before the results of confirmatory diagnostic tests like ADAM TS13 assay[1].
- If plasmapheresis is unavailable, patients can be administered large volume plasma or FFPs. This treatment is not as efficacious as plasmapheresis, but it can be suitable alternative[2] .
- Patients should be given folic acid (oral as patients can develop folate deficiency due to ongoing hemolysis [3].
- Platelets transfusion is indicated for patients with serious internal bleeding, hemorrhage or neurological complications as there is a high incidence of thrombosis with platelet transfusion [4] [5].
- Patients are given adjunct corticosteroids along with therapeutic plasmapheresis. The dose of prednisolone (oral) depends upon severity of clinical symptoms with higher doses for clinically more severe disease. The staring dose of prednisolone (oral) is 1mg/kg. This dose is maintained until platelet count comes back to normal range. Then the dose is rapidly tapered over a period of three to four weeks[6].
- In thrombotic thrombocytopenic purpura, there is increased platelet aggregation with formation of microthrombi in blood vessels. Antiplatelet therapy results in increased survival rate in these patients[7].
- Eculizumab, a monoclonal antibody that directly inhibits terminal part of C5 complement system protein is the drug of choice for atypical hemolytic uremic syndrome[8].
- Immunosuppressive therapy including Rituximab, a monoclonal antibody directed against CD20 protein and high dose prednisolone (oral) is administered to patients with refractory thrombotic microangiopathy[9][10].
References
- ↑ Arnold DM, Patriquin CJ, Nazy I (2017). "Thrombotic microangiopathies: a general approach to diagnosis and management". CMAJ. 189 (4): E153–E159. doi:10.1503/cmaj.160142. PMC 5266569. PMID 27754896.
- ↑ Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC; et al. (1991). "Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group". N Engl J Med. 325 (6): 393–7. doi:10.1056/NEJM199108083250604. PMID 2062330.
- ↑ Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F; et al. (2012). "Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies". Br J Haematol. 158 (3): 323–35. doi:10.1111/j.1365-2141.2012.09167.x. PMID 22624596.
- ↑ Swisher KK, Terrell DR, Vesely SK, Kremer Hovinga JA, Lämmle B, George JN (2009). "Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura". Transfusion. 49 (5): 873–87. doi:10.1111/j.1537-2995.2008.02082.x. PMID 19210323.
- ↑ Goel R, Ness PM, Takemoto CM, Krishnamurti L, King KE, Tobian AA (2015). "Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality". Blood. 125 (9): 1470–6. doi:10.1182/blood-2014-10-605493. PMC 4342358. PMID 25588677.
- ↑ George JN (2010). "How I treat patients with thrombotic thrombocytopenic purpura: 2010". Blood. 116 (20): 4060–9. doi:10.1182/blood-2010-07-271445. PMID 20686117.
- ↑ Bobbio-Pallavicini E, Gugliotta L, Centurioni R, Porta C, Vianelli N, Billio A; et al. (1997). "Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP". Haematologica. 82 (4): 429–35. PMID 9299856.
- ↑ Fakhouri F, Hourmant M, Campistol JM, Cataland SR, Espinosa M, Gaber AO; et al. (2016). "Terminal Complement Inhibitor Eculizumab in Adult Patients With Atypical Hemolytic Uremic Syndrome: A Single-Arm, Open-Label Trial". Am J Kidney Dis. 68 (1): 84–93. doi:10.1053/j.ajkd.2015.12.034. PMID 27012908.
- ↑ Prior DJ (1975). "A study of the electrophysiological properties of the incurrent siphonal valve muscle of the surf clam, Spisula solidissima". Comp Biochem Physiol A Comp Physiol. 52 (4): 607–10. doi:10.1016/s0300-9629(75)80009-0. PMID 0.1182/blood-2014-10-559211 Check
|pmid=value (help). - ↑ Balduini CL, Gugliotta L, Luppi M, Laurenti L, Klersy C, Pieresca C; et al. (2010). "High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study". Ann Hematol. 89 (6): 591–6. doi:10.1007/s00277-009-0877-5. PMID 20033409.