C32-23 A Refractory Case of Immune Thrombocytopenia Complicated by Cmv Pneumonitis

A 20-year-old African-American female with a past medical history of granulomatosis with polyangiitis, Crohn’s disease, type 1 diabetes mellitus, and chronic hepatitis B presented with acute-on-chronic vaginal bleeding and severe thrombocytopenia with a platelet count of 2,000. Immune thrombocytopenic purpura (ITP) is an acquired autoimmune disorder characterized by thrombocytopenia due to autoantibodies against platelet antigens. The estimated incidence is 2 to 5 per 100,000 people.The patient was evaluated for secondary causes of immune thrombocytopenic purpura (ITP). Potential triggers included chronic infections, such as HIV (which was negative in her case), systemic lupus erythematosus, Epstein-Barr virus (EBV), and Helicobacter pylori.During this admission, the patient was receiving chronic steroids and subsequently developed CMV infection. Notably, a high CMV viral load is associated with more severe thrombocytopenia and an increased risk of bleeding complications. The patient received appropriate treatment for CMV her platelet count remained suboptimal despite these interventions.The patient’s bone marrow biopsy revealed trilineage hematopoiesis with increased megakaryocytes and iron deficiency anemia, findings supportive of a diagnosis of immune thrombocytopenia (ITP). Six months after initial presentation, she underwent splenectomy, which is considered an effective intervention for achieving durable remission in ITP by removing the primary site of platelet clearance and autoantibody production. She was also treated with intravenous immunoglobulin (IVIG), which temporarily increased her platelet count to approximately 50,000 by modulating the immune response—primarily through blockade of Fc receptors on macrophages, thereby reducing platelet destruction. She was then started on eltrombopag, a thrombopoietin receptor agonist, and is recommended as a second-line therapy for patients with insufficient response to corticosteroids, IVIG, or splenectomy.In patients with immune thrombocytopenic purpura (ITP) who fail to achieve sustained remission or experience relapse following glucocorticoid therapy, several alternative therapeutic modalities have been validated in randomized clinical trials. Thrombopoietin receptor agonists (TPO-RAs) act on the c-Mpl receptor expressed on megakaryocyte precursors, thereby promoting megakaryocyte proliferation and differentiation. Rituximab, a monoclonal antibody targeting the CD20 antigen on B lymphocytes, induces B-cell depletion.Given the persistent and refractory nature of the patient’s disease, rilzabrutinib—an oral Bruton tyrosine kinase inhibitor—was initiated. Evidence from multiple clinical trials demonstrates that rilzabrutinib induces rapid and sustained platelet responses, reduces the incidence of bleeding events, and the need for rescue therapy.This case exemplifies a refractory case of ITP with unique diagnostic and therapeutic challenges that persist despite the application of maximal immunomodulatory treatment. This abstract is funded by: NA