Essential thrombocythaemia

Baillière's Clinical Haematology
G Tobelem

Abstract

Essential thrombocythaemia (ET) is a rare clonal myeloproliferative disorder characterized by a persistent increase in platelet count. The commonly used criteria for the diagnosis, except for the level of the increase in platelet count, are usually those fixed by the Polycythemia Vera Study Group. The average age of onset is around 60 years, both sexes being affected. The symptoms frequently present at diagnosis are related to microcirculatory disturbances (palms, soles, fingers). Neurological symptoms are often observed. Thrombotic complications of the large vessels are less frequent. Haemorrhagic problems are present in about 30% of patients. Bleeding time is normal in most cases, whereas platelet aggregation abnormalities are frequently found. Nil adrenaline aggregation is the most discriminative test. The clinical course is characterized by long intervals without any symptoms; thromboembolic or haemorrhagic episodes can, however, occur, mainly in uncontrolled ET. Development of terminal acute leukaemia has been reported in 34 cases. The expression of the influence of the treatments, 32P or alkylating agents, is very strong. The treatment of ET has to take in consideration the difficult compromise between balancing the neces...Continue Reading

Citations

Oct 1, 1991·Annals of Hematology·S SacchiM F Martelli
Jan 1, 1990·Pediatric Hematology and Oncology·E Fernandez-RoblesG Cornu
Apr 1, 1995·British Journal of Haematology·M KikuchiA Ohsaka
Mar 1, 1994·Annals of Hematology·R J SchlemperJ C Eikenboom
Feb 1, 1990·European Journal of Haematology·J Kutti
Jul 1, 1995·Stem Cells·T I Mughal
Dec 12, 2002·Hematology·Apostolia-Maria Tsimberidou, Francis J Giles
Sep 1, 1994·Baillière's Clinical Haematology·S D Wright, E G Tuddenham

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