TTP, ET, and ITP Sequential Occurrence in a Single Patient: a Case Report

INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP), essential thrombocythemia (ET) and idiopathic thrombocytopenic purpura (ITP) are three separate disease entities that affect the platelets. Each has a different pathophysiology clinical presentation and consequences. We present a case where TTP, ET, and ITP were diagnosed in the same patient at different times of her clinical course with the aim of drawing the attention to this undocumented occurrence which remains under investigation.

CASE REPORT: A 42 year old African American female was diagnosed in 1994 with TTP after she presented with slurring of speech and left upper extremity weakness. Her hemoglobin was 9.7 g/dL, creatinine of 1.3 mg/dL, LDH of 448 IU/L and her peripheral smear showed schistocytes. She was diagnosed with TTP and was treated with plasma exchange with improvement in the platelet count. In March 2001, she presented with purple toe syndrome and a platelet count of 877,000. Bone marrow biopsy revealed panmyeloid hyperplasia, clustering of megakaryocytes, and stainable iron. She was then diagnosed to have essential thrombocythemia and was started on Anagrelide, which was later switched to Hydroxyurea. In October 2001, Hydroxurea was stopped and two weeks later, her platelet count dropped to 12,000. Investigations including a bone marrow biopsy were done which showed megakaryocytes in atypical clusters. No schisocytes were seen on peripheral smear. The patient’s platelet count dropped to 7,000 and she was transfused with 12 units of platelets. Post transfusion her count was 90,000 but dropped to 25,000 in less than 12 hours. She was then started on IV immunoglobulins and steroids for a presumptive diagnosis of ITP and her platelet count subsequently improved. Subsequent outpatient evaluation was negative for ANA, C3, C4, and HIV

CONCLUSION: The fact that both immune and thrombotic thrombocytopenic purpura occur with increased frequency among persons with systemic lupus erythematosus, HIV disease, or pregnancy supports the hypothesis that some pathophysiologic factors are shared. As the three conditions have different natural histories and require different treatment modalities, it is important to recognize that these diseases may be seen sequentially and here comes the importance of reviewing peripheral blood smears for evaluation of thrombocytopenia and bone marrow aspirations for diagnosis of thrombocythemia in order to reach an accurate diagnosis and tailor therapy accordingly. This case demonstrates the variability and complexity of platelet disorders. Platelets may go one way or another and can even go in opposite directions in the same person. After reviewing the literature, there are no reported cases of TTP, ET, and ITP diagnosed in the same patient. Our aim is to draw the attention to this rare case which is still under investigation.