Authors: Andrew Brown, MS4; Devin Madenberg, DO; Cory Ganshert, DO; Sara Dunbar, DO
Introduction: Immune thrombocytopenia (ITP) is an acquired autoimmune thrombocytopenia caused by autoantibodies against platelet antigens. Inciting factors can vary widely, and range from infection, autoimmune disease, medication, and hematologic malignancy. Clinical manifestations of the disease most commonly include bleeding and thrombocytopenia. Evaluation and treatment can prove challenging, especially when patients do not respond to initial treatment. This patient also presented with oral lesions, which have a broad differential including malignancy, bleeding disorders, and autoimmune disease. In this case, we display the differential for these issues and the treatment of refractory ITP.
Case Presentation: A 64 year old female with a history of hypertension, type 2 diabetes, CVA, and GERD. She initially presented with a three day history of oral blisters after biting the inside of her cheek. Physical exam showed petechia of her neck and upper chest and bilateral fibrinous lesions of the buccal mucosa without active bleeding. Initial evaluation revealed a new thrombocytopenia with a platelet count of less than 3 k/uL and a hemoglobin of 9.1 g/dL. Peripheral smear was without schistocytes. Negative workup results included CMV, HSV, EBV, Hepatitis B and C, HIV, Parvovirus, ANA and anti-DS DNA. She was recently diagnosed with H pylori and was on triple therapy, which was considered as a potential cause. She was treated for suspected ITP with four day course of 40 mg IV dexamethasone, four doses of IVIG 400 mg/kg, 8 units of platelets as well as one dose of romiplostim 250 mg; however, her platelets count remained less than 5 k/uL. Due to concern for lymphoproliferative disorder as a cause, a bone marrow biopsy was obtained and showed atypical megakaryocyte hyperplasia which was thought to be from romiplostim administration with no signs of malignancy. On the sixth day of admission, platelet count recovered to 77 k/uL. She was transitioned to daily prednisone with continued improvement in her platelet count to 514 k/uL. She was discharged after 9 days with daily prednisone and weekly romiplostim. At an outpatient hematology appointment 2 weeks after discharge, her skin and oral lesions had resolved and platelet count was 381 k/uL.
Discussion: This case was particularly challenging to determine an etiology and treatment plan for her ITP. Primary, secondary and drug-induced causes were all considered. Treatment of refractory ITP has special considerations: IVIG dosage must be adjusted in patients with a history of CVA, and use of rituximab has the potential for Hepatitis B reactivation. Thrombopoietin receptor agonist therapy can be considered for patients failing to respond to earlier line treatments. Finally, this case demonstrates the broad differential for oral lesions which include infection, malignancy, and other autoimmune phenomena.
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- Neunert C, et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost. 2015 Mar;13(3):457-64. doi: 10.1111/jth.12813. Epub 2015 Jan 14.