Proposal of treatment algorithm for immune thromocytopenia in adult patients of a hematology service at a referral center in Northeastern Brazil

Abstract

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.

Short-term dose-escalated romiplostim for preparing an adult patient with persistent newly diagnosed primary immune thrombocytopenia for splenectomy

“…Treatment is recommended for patients with a platelet count of <30,000 platelets/mm 3 , associated with bleeding or <20,000 platelets. 1 …”

“… 2 Platelet transfusions for life-threatening bleeding have a better response if used after IVIG infusion due to increased platelet survival. 2 For patients who do not respond to steroids/IVIG, intravenous dexamethasone alone, 1 or in combination with rituximab, is recommended. 3 The combination provides a sustained response rate of 58% at six months and is superior to mono-therapy.…”

“… 4 Splenectomy is indicated for patients failing first- and second-line treatment. 1 IVIG plus platelet transfusions may increase the platelet count sufficiently to decrease the risk of hemorrhage and permit surgery. 1 The use of thrombopoietin receptor agonists (TPO-RAs) has been used in chronic ITP to increase the platelet count, permitting the splenectomy.…”

“… 1 IVIG plus platelet transfusions may increase the platelet count sufficiently to decrease the risk of hemorrhage and permit surgery. 1 The use of thrombopoietin receptor agonists (TPO-RAs) has been used in chronic ITP to increase the platelet count, permitting the splenectomy. There is little published data on the short-term use of TPO-RAs in patients refractory to the initial treatment for the subsequent splenectomy.…”