Essential Thrombocythaemia A Single Institution E

Authors: Sunny Joseph Varghese Mohamed Bahey El Din Mona Al Hendi Ramesh Kumar

Publish Date: 2012/06/26

Volume: 29, Issue: 3, Pages: 139-146

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Abstract

This presentation is a clinical narrative and long term follow up 6–16 years of 21 prospectively studied patients with essential thrombocythaemia ET in Kuwait The median age 559 years is younger than reported by others Two patients were below the age of 40 years with one of them presenting as postpolycytheamia ET at 16 years of age Twelve patients 571  remained asymptomatic throughout the period of follow up Four patients complained of erythromelalgia three 19  suffered from thrombotic episodes and only one 43  had excessive bleeding Four patients presented with splenomegaly Intensity of thrombocytosis or duration of very high platelet count had no relationship with these complications Two patients transformed to postET myelofibrosis and one patient developed chronic myeloid leukaemia CML None transitioned to acute leukaemia All patients are still alive after follow up for 6–16 years Janus kinase 2 mutation was positive in eight 38  patients It had no bearing on transition of our ET patients to postET myelofibrosis or CML Platelet aggregation tests were performed in 14 patients Six 429  showed defective response to ADP Only one of these patients suffered from bleeding All patients were given aspirin 81 mg/day Cytoreductive therapy with hydroxyurea was taken by six 429  subjects Two patients who were treated with anagrelide and one with alphainterferon did not continue treatment for long

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