Essential Thrombocythaemia: A Single Institution Experience of 16 Years

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 (55.9 years) is younger than reported by others. Two patients were below the age of 40 years with one of them presenting as post-polycytheamia ET at 16 years of age. Twelve patients (57.1 %) remained asymptomatic throughout the period of follow up. Four patients complained of erythromelalgia, three (19 %) suffered from thrombotic episodes and only one (4.3 %) 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 post-ET 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 post-ET myelofibrosis or CML. Platelet aggregation tests were performed in 14 patients. Six (42.9 %) showed defective response to ADP. Only one of these patients suffered from bleeding. All patients were given aspirin (81 mg/day). Cyto-reductive therapy with hydroxyurea was taken by six (42.9 %) subjects. Two patients who were treated with anagrelide and one with alpha-interferon did not continue treatment for long.

Acquired JAK-2 V617F Mutational Analysis in Pakistani Patients with Essential Thrombocythemia

“…JAK2 V617F positivity in ET has been observed from various racial backgrounds ranging from 25% to 70% (Sazawal et al, 2010;Alshemmari et al, 2014). When compared with earlier reports, our results are intermediate with regional studies reported from India; ranging 38% to as high as 70% for JAK2V617F mutational expression (Sazawal et al, 2010;Varghese et al, 2013). (Hamidah et al, 2012;Alshemmari et al, 2014).…”

Clinico-Hematological Profile and Risk Stratification in Patients with Essential Thrombocythemia: Experience from Pakistan

“…DOI:http://dx.doi.org/10.7314/APJCP.2015.16.17.7659 Clinico-Hematological Profile in Essential Thrombocythemia: Experience from Pakistan. Most of our patients were asymptomatic (61.9%) and diagnosed inadvertently. This is more or less similar to studies reported from Kuwait (57.1%) and Thailand (54.2%) (Varghese et al, 2013;Duangnapasatit et al, 2015). Nevertheless, symptomatic cases were found to be 38.1% in our study.…”

“…The most common symptoms in our patients were weakness (19%); painful burning sensationerythromelalgia (14.2%) and transit limbs ischemia (9.5%). Varghese had reported 19 % of patients presented with erythromelalgia, while 14.1 % had thrombotic episodes and 4.5 % developed bleeding (Varghese et al, 2013).…”

Essential Thrombocythemia among Patients with Myeloproliferative Neoplasms in Haematology Unit of a Tertiary Care Centre: A Descriptive Cross-sectional Study

“…This finding is different from the study of Kuwait and Thailand where 57.1% and 54.2% of the patient were asymptomatic respectively. [11][12][13] Despite this, 11.76% of the cases in our study were asymptomatic. According to a study, 19% of the patients suffered erythromelalgia, 14.1% had thrombotic events, and 4.5% had bleeding.…”

“…According to a study, 19% of the patients suffered erythromelalgia, 14.1% had thrombotic events, and 4.5% had bleeding. 13 The most likely reason for the low number of asymptomatic patients is that they only come to the clinic when they acquire symptoms in our settings. Splenomegaly has been documented in 25% to 50% of ET patients.…”