[Acute myeloid leukemia developing in the course of essential thrombocythemia].
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The course of primary thrombocythemia has been observed in 22 patients over a period of 1-19 years. In contrast to experience with primary thrombocythemia in the literature, thrombotic complications were far more common than hemorrhage. 20 out of 22 patients complained of pain in toes and fingers due to disturbances of microcirculation, whereas bleeding disorders were present only in 5. Studies on spontaneous aggregation of platelets revealed a close association between platelet hyperaggregability and ischemic attacks. 500 mg acetylsalicylic acid every second day normalized the hyperaggregability in vitro and removed pain completely. The indication for antiaggregating substances in the therapy of primary thrombocythemia is discussed.
Essential thrombocythemia is a myeloproliferative disorder with an increased amount of abnormal platelets, causing both hemorrhagic and thrombotic pathology. Some of its systemic complications include deep vein thrombosis, pulmonary emboli, myocardial infarcts, and renal vessel thrombosis among others. We present the rare case of a woman suffering from essential thrombocythemia with the sudden loss of vision in her right eye, caused by central retinal artery occlusion. A possible connection between these two disorders is discussed.
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The clinical course of essential thrombocythemia (ET) is variable, ranging from microvascular circulation disturbances to severe thromboembolic or hemorrhagic complications in patients who do not have any symptoms for many years. The identification of patients at risk for major thrombosis who need platelet-lowering therapy is important. During the last two decades, several risk factors for the development of ET-related thrombotic and bleeding complications have been identified. These include platelet counts, previous thrombotic events, older age, cardiovascular risk factors, hereditary thrombophilia, clonality, and the presence of molecular markers such as PRV-1 or the Janus kinase 2 (JAK2) mutation. According to the presence or absence of these risk factors, individual patients with ET are currently stratified as low-, intermediate-, or high-risk patients. The influence of these risk factors on therapeutic decisions in patients with ET is critically reviewed and discussed in detail.
Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by marked thrombocytosis with associated hemorrhagic, thrombotic and embolic complications caused by platelet dysfunction. In this report we describe two cases of ET and moderate to severe hypertension associated with renal artery stenosis and renal microvascular lesions. In both cases treatment of the hematologic disorder resulted in reduction of blood pressure and improvement of clinical symptoms.
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Polycythemia vera and essential thrombocythemia are chronic myeloproliferative disorders, the benign clinical course of which can be complicated by both thrombotic and hemorrhagic diatheses. Thrombotic diathesis is characterized by microcirculatory disturbances and by an increased risk of arterial and venous thromboses. Thrombotic accidents often manifest at diagnosis or in the preclinical phase of the disease so that the search for a latent myeloproliferative disorder has become widely recommended in screenings for acquired thrombophilia, particularly when venous thromboses manifest at an unusual site. Hemorrhagic diathesis is more rare, less ominous and mostly affects patients with a very high platelet count. In these subjects, an altered degradation and function of von Willebrand factor can cause minor mucocutaneous hemorrhages, which are sometimes a prelude to major gastrointestinal bleedings. The bleeding tendency can be effectively treated by cytoreduction. Pathogenesis and treatment of thrombotic diathesis are still controversial. The nature of disease-related hemostatic abnormalities and the role of common risk factors are far from being elucidated. In polycythemic subjects, treatment of blood hyperviscosity is essential and low-dose aspirin, which has an established antithrombotic efficacy, should always be used in the absence of contraindications. These are mostly constituted by conditions of increased bleeding risk, which, in particular, have to be evaluated when considering aspirin use in patients with essential thrombocythemia. Future clinical research should primarily aim to assess the risk/benefit ratio of aspirin use in this disease, to better characterize the determinants of vascular risk and to reduce the high incidence of leukemias in patients with these diseases. This might require either the availability of safer cytoreductive agents or, alternatively, the use of more aggressive antiplatelet regimens in patients at high thrombotic risk.