Immune Thrombocytopenia (ITP): Enigma of an Old Disease

Case Report

Ann Hematol Oncol. 2020; 7(8): 1315.

Immune Thrombocytopenia (ITP): Enigma of an Old Disease

Caplan A1, Ahlstrom S2, Sharma A3* and Mollick J4

1Internal Medicine Resident, St. Mary’s Medical Center, San Francisco, USA

2Physician Assistant, Tucson, USA

3Assistant Clinical Professor of Medicine, University of California, San Francisco, USA

4Associate Clinical Professor of Medicine, University of California, San Francisco, USA

*Corresponding author: Atul Sharma, Assistant Clinical Professor of Medicine, University of California, San Francisco, USA. E-mail: atul.sharma@ucsf.edu

Received: September 14, 2020; Accepted: October 12, 2020; Published: October 19, 2020

Abstract

“Immune thrombocytopenia,” formerly Idiopathic Thrombocytopenic Purpura (ITP), an autoimmune disorder, is characterized by isolated thrombocytopenia, with platelet count less than 10 x 10 to the 9th /L [1,2]. Estimated ITP incidence is 100 cases, per million persons per year. Approximately 50% of cases occur in children [3]. In adults, it tends to follow a chronic course, with mucocutaneous bleeding being the most common manifestation [4]. A rare, though major cause of fatal bleeding in patients with ITP, is intracerebral hemorrhage; less than 1% of all cases [5]. Here we discuss a case of a 26-year-old Asian female patient, with refractory ITP and intracerebral hemorrhage, leading to brain death. We explore current treatment recommendations and suggested approaches to therapy as well as challenges.

Keywords: ITP; Immune thrombocytopenia; Idiopathic thrombocytopenic purpura; Intracerebral hemorrhage

Case Presentation

A 26 y/o Asian female, with a three year history of ITP, who had been previously treated with steroids (prednisone and dexamethasone), IVIG, and Eltrombopag (oral TPO analog) for intractable mucocutaneous bleeding with poor response to steroids and IVIG. She had also recently been started on rituximab approximately two weeks prior to presentation. Of note, despite multiple attempts, we were unable to obtain records from the patient’s outpatient hematologist to confirm her diagnostic and treatment history. We were, however able, to confirm that the patient had never been previously hospitalized for ITP symptoms, and thus we postulate that she likely did achieve remission previously.

On this occasion, the patient was sent to the ED, by her hematologist, for progressive LLE pain and worsening oral mucosal bleeding. Her LLE pain was described by the patient as a “tingling sensation moving from the calf to hip, with worsening pain at the hip and knee joints”. She denied recent falls or trauma. She additionally reported the presence of oral hemorrhagic bullae, causing “discomfort with eating and persistent taste of blood.”

She had been hospitalized two weeks prior to this admission with multiple petechiae of the hard and soft palate, spontaneous epistaxis, and ecchymosis of lateral left thigh. She was noted to have a platelet count <1 and received 50 grams IVIG and platelet transfusion, with subsequent improvement of platelets to 41k/ul. Spontaneous bleeding symptoms successively resolved and she was discharged home in stable condition with close outpatient follow up.

When she represented, her vital signs were normal with physical exam notable for bullae of buccal mucosa, and tip and R lateral tongue. Petechiae of bilateral LEs, and ecchymosis of the R lateral thigh and L lateral calf was also found on exam. No joint edema or effusions were noted. The neurological exam was benign. Labs were notable for platelet count of 5K/ul.

The patient was given 50mg IVIG without steroids, however, unlike during previous admission, she did not exhibit decrease in bleeding symptoms. She developed epistaxis shortly after admission. After consult with hematology/oncology, she was given additional IVIG and steroids; methylprednisolone 60 mg twice daily for 4 days. She was not transfused with platelets. The epistaxis ceased.

Approximately 24 hours into her hospital stay a rapid response was called when patient was noted to have an episode of emesis followed by new onset right sided facial droop, with right upper and lower extremity weakness. A Stroke Alert was called. A noncontrast head CT demonstrated a large left basal ganglia bleed, hemorrhage into the adjacent ventricle, and early trapping of the left temporal horn (Figure A). She was immediately transferred to ICU and intubated, due to worsening neurologic deficits and mentation. Immediately after intubation, the patient began to seize, and was given lorazepam IV and a loading dose of Levatiracetem. She was subsequently transferred to a tertiary care center with neurosurgery services.