Microangiopathic hemolytic anemia medical therapy

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].

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