Diagnosis of immune thrombocytopenia, including secondary forms, and selection of second-line treatment

Abstract

This article summarizes our approach to the diagnosis of immune thrombocytopenia (ITP), its secondary forms, and choice of second-line treatment options. We very briefly summarize first-line treatment and then utilize a case-based approach. We first explore persistent, chronic ITP in a younger female. We consider many possibilities beyond primary ITP e.g., hypogammaglobulinemia, chronic infection, and anemia, and how to approach their diagnosis and management. The journey continues throughout pregnancy and the post-partum period and eventually includes fourth-line treatment after a late relapse. We then consider an older male, emphasizing differences in diagnostic considerations and management. The focus is on initiation and continuation of second-line treatment, the pros and cons of each option, and briefly the impact of treatment choices related to the endemic presence of severe acute respiratory syndrome coronavirus 2. During the review of potential second-line treatment options, we also briefly touch upon novel treatments. Finally, there is a short section on refractory disease drawn from our previous extensive review published in February 2020.1 The clinical nature of the discussions, replete with figures and tables and with the interspersion of pearls regarding efficacy and toxicity at different ages and genders, will serve the reader in the management of "typical" adult patients who develop persistent and chronic ITP.

Figure 1.

Treatment options for immune thrombocytopenia during pregnancy, leading up to delivery, and in the post-partum period. IVIG: intravenous immunoglobulin; TPO-RA: thrombopoietin receptor agonist; ITP: immune thrombocytopenia.

Figure 2.

Second-line treatment options for immune thrombocytopenia and possible treatment courses. TPO: thrombopoietin; R1, R2, R3, R4: first, second, third and fourth weekly dose of rituximab; COVID: coronavirus disease 2019; ITP: immune thrombocytopenia; BID: bis in die.