Introduction
The management of adult (≥18 years) immune thrombocytopenia patients relies on platelet count, the risk of bleeding and presence of bleeding.
Objective
Confirming the diagnosis of immune thrombocytopenia and the start of therapy, our hematology service, a referral center, favors the establishment of this algorithm to treat those patients.
Results
Presentation, recently diagnosed or recurrence – group 1: life-threatening bleeding: high-dose intravenous immunoglobulins with methylprednisolone or dexamethasone. Hospitalization and platelet transfusion are considered. Group 2: Platelets <30 × 10
9
/L with bleeding or risk factor for bleeding, or platelets <20 × 10
9
/L: prednisone or dexamethasone. No response, platelets <20 × 10
9
/L: replace corticoid or increase doses. If platelets continue <20 × 10
9
/L: immunization and splenectomy. Investigation of
Helicobacter pylori
, if positive: treatment for
H. pylori.
Chronic immune thrombocytopenia with platelets <20 × 10
9
/L we propose two new groups (A and B): Group A: <65 years, no or low surgical risk, patient declines maintenance therapy or patient intends to get pregnant: immunization and splenectomy. Group B: failure of splenectomy (refractory) or no splenectomy indication or history of exposure to malaria or babesiosis and no response to corticoids or corticoid dependence: choose thrombopoietin receptor agonists: eltrombopag or romiplostim. Patient at high risk for arterial or venous thrombosis: recommend rituximab. After rituximab or thrombopoietin receptor agonists, if platelets continue <20 × 10
9
/L: indicate immunosuppressants (azathioprine or cyclophosphamide), dapsone or mycophenolate mofetil or vinca alkaloids. The goals of treatment for chronic or refractory immune thrombocytopenia are to keep platelets >20 × 10
9
/L and stop bleeding.
Hemophilia A (HA) is a rare bleeding disorder caused by reduced or absent clotting factor VIII (FVIII) activity due to mutation in its coding gene (F8). [1] FVIII replacement is required lifelong to treat bleeding, especially in severe cases (FVIII activity below 0.01 international units [IU]/mL). [1] Besides that, up to 44% of people with HA (PwHA) may develop neutralizing antibodies against exogenous FVIII (inhibitors). [2] The presence of inhibitors predispose PwHA to severe bleeding, [3] and increased morbidity and mortality. [4,5] The treatment of choice for persistent, high-responding inhibitors is immune tolerance induction (ITI), which consists of frequent and long-term infusions of FVIII. [1] Success rates among different ITI regimens range from 60%-90%. [6]
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