Title: Primary immune thrombocytopenia and essential thrombocythemia: Cases series

Abstract

We have identified in the literature five cases of primary immune thrombocytopenic purpura (ITP) with subsequent development of essential thrombocythemia (ET) [1-5]. This surprising clinical condition is possible, but very rare and not easy to diagnose and manage. Clinical characteristic is shown in Table 1. ITP. JAK2V617F mutation was present in three and CALR mutation in one patient. Three out of five ITP patients undergone splenectomy and had a good response. Moreover, a new case from our Department, that has not yet been published. In the 51-year-old female ITP was diagnosed in the age of 27 (1997). The normalization of platelet count has been achieved after splenectomy performed in 2004. In 2013 the diagnosis of systemic lupus erythematosus (SLE) was confirmed. At that time, her platelet count increased up to 900 G/L, and the diagnosis of ET-TN was made on the basis of bone marrow histopathological examination and WHO criteria. We have made an attempt to analyse the possible causes of the sequential appearance of ITP and ET taking into consideration the following: alteration of thrombopoietin receptor (TPO-R), the role of autoimmunity and inflammation, and cytokine modulation. A better understanding of these interactions may provide the opportunities for determination of predisposing factors and may aid in finding a new treatment modalities both for ITP and ET patients.