• Asian Pacific Journal of Cancer Prevention
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• v.15 no.4
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• pp.1681-1684
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• 2014

Objective: To investigate interferon (IFN) alpha 2 b for treating patients with JAK2V617F positive polycythemia vera (PV) and essential thrombocytosis (ET). Methods: Interferon alpha 2 b was used to treat patients with JAK2V617F positive PV and ET. In control group, hydroxyurea was used. Endpoint of study was to compare rates of hematological and molecular remission. Results: Patients in the interferon alpha 2 b group achieved higher rates of hematologic and molecular remission than patients in the hydroxyurea group, with a lower incidence of thrombosis. Conclusion: Compared with hydroxyurea, interferon alpha 2 b could reduce JAK2V617F load for patients with PV and ET, and achieve higher molecular remission, improve treatment efficacy and reduce complications.

• Journal of Chest Surgery
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• v.49 no.5
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• pp.397-400
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• 2016

Essential thrombocytosis (ET) is a myeloproliferative disorder characterized by an anomalous increase in platelet production. Many patients with ET are asymptomatic. Few studies have reported ET-associated thromboembolism in large vessels such as the aorta. We report a patient with ET who presented with peripheral embolism from an abdominal aortic thrombus and developed acute limb ischemia. The patient underwent aortic replacement successfully. The patient's platelet count was controlled with hydroxyurea, and no recurrence was noted over 2 years of follow-up.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.16
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• pp.7327-7330
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• 2015

Background: Essential thrombocythemia (ET) is a clonal hemopoietic stem cell myeloproliferative neoplasm characterized by persistent thrombocytosis along with megakaryocytic hyperplasia. In the last decade following the identification of an acquired JAK2 V617F mutation, there has been acceleration in our understanding of this disease. The rational of this study was to determine the mutational profile of JAK2 V617F in Pakistan patients with ET. Materials and Methods: In this retrospective cross sectional study, 21 patients with ET were enrolled from January 2011 to December 2014. Patients were diagnosed based on WHO criteria for essential thrombocythemia. Complete blood count was done on an automated hematology analyzer, while JAK2 V617F expression was evaluated by polymerase chain reaction. Results: The mean age was $56.7{\pm}19.0$ years (range 18-87) and the male to female ratio was 1:1.1. The frequency of JAK2 V617F positivity in our ET patients was found to be 61.9%. The mean hemoglobin was $11.7{\pm}2.4$ g/dl with a total leukocyte count of $13.3{\pm}8.1{\times}109/l$ and a platelet count of $1188{\pm}522{\times}109/l$. Positive correlations for JAK2 V617F mutation were established with high TLC count and raised LDH (P<0.05). No correlation of JAK2 V617F could be established with age and gender (P>0.05). Conclusions: JAK2 V617F mutation frequency in our ET patients was similar to those reported previously. Screening for the mutation in all suspected essential thrombocythemia cases could be beneficial in differentiating patients with reactive and clonal thrombocytosis.

• Journal of Yeungnam Medical Science
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• v.28 no.1
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• pp.99-104
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• 2011

Essential thrombocythemia (ET), a subcategory of chronic myeloproliferative disorder, is characterized by absolute thrombocytosis due to excessive clonal proliferation of platelets, hyperaggregability of platelets, and increased incidence of thrombosis and hemorrhage. We consider a diagnosis of ET when an unexplained and persistent thrombocytosis is observed. It is difficult to consider ET first when we meet a patient with esophageal varix bleeding or unusual multiple thromboses like mesenteric vein, splenic vein, and portal vein. This article reports a patient who presented initially with esophageal varix bleeding and unusual multiple thromboses, thereafter, she was diagnosed with ET after testing positive for the Janus Tyrosine Kinase 2 (JAK2) V617F mutation. In conclusion, in patients with varix bleeding and unusual multiple thromboses, myeloproliferative disorders like essential thrombocythemia should be considered as a potential cause and testing for the JAK2 mutation is warranted.

• Journal of Medicine and Life Science
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• v.20 no.2
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• pp.99-102
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• 2023

Patients with essential thrombocythemia (ET) can undergo laparoscopy without international precautions. This case report describes an ET with a mild increase in the platelet count that developed after laparoscopic surgery and prolonged intraperitoneal bleeding. The patient underwent intensive postoperative medical and surgical treatments until cured. Patients with ET should provide informed consent for post-laparoscopic bleeding. Surgeons should perform optimal hemostasis. Further studies are required to provide clear guidelines for both medical and surgical interventions aimed at preventing thrombo-hemorrhagic complications associated with ET. A preoperative study of platelet function and the selection of the best cytotoxic drug for the perioperative period are mandatory.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.17
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• pp.7659-7661
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• 2015

Background: Essential thrombocythemia (ET) is a Philadelphia chromosome-negative myeloproliferative neoplasm characterized by sustained thrombocytosis and megakaryocytic hyperplasia. It is an uncommon hematological malignancy which primarily affects elderly individuals. The rational of this study was to determine its clinico-hematological profile along with risk stratification in Pakistan patients. Materials and Methods: In this retrospective cross sectional study, 21 patients with ET were enrolled from January 2011 to December 2014. Data was analyzed with SPSS version 21. Results: The mean age was $56.7{\pm}19.0years$ (range 18-87) and the male to female ratio was 1:1.1. Of the total, 62% of patients were above 50 years of age. Overall 61.9% were diagnosed incidentally and were asymptomatic. In symptomatic patients, major complaints were weakness (19%); erythromelalgia (14.2%), transit ischemic attack (9.5%) and gastrointestinal bleed (4.7%). The mean hemoglobin count was $11.7{\pm}2.4g/dl$ with a total leukocyte count of $13.3{\pm}8.1{\times}10^9/l$ and platelets count of $1188.8{\pm}522.2{\times}10^9/l$. Serum lactate dehydrogenase, serum creatinine and uric acid were $454.3{\pm}127.8$, $1.2{\pm}0.5$ and $7.4{\pm}3.4$ respectively. According to risk stratification, 57.1% were in high risk; 23.8% in intermediate risk while 19.1% in low risk group. Conclusions: ET in our patients in Pakistan, unlike in the West, is seen in a relatively young population. Primarily patients were asymptomatic and risk stratification revealed predominance of high risk disease in our setting.

• Tuberculosis and Respiratory Diseases
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• v.67 no.4
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• pp.351-355
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• 2009

The incidence of pulmonary thromboembolism increases with age. The risk factors of pulmonary thromboembolism include surgery, malignancy, obesity, lupus anticoagulants, and vascular conditions such as deep vein thrombosis. Thromboembolism in younger patients or in unusual locations, the possibility of primary thrombophilic conditions should be considered. Primary thrombophilic states include myeloproliferative disorders (MPD). JAK2 V617F mutation is found commonly in patients diagnosed with MPD, in 90~95% of polycythemia vera (PV) and in 50~60% of essential thrombocytosis (ET) patients. Sometimes the JAK2 V617F mutation is found in cases without MPD. The relationship between JAK2 V617F mutation and thrombosis has not been defined. Recently, clinical evidence suggests that this mutation may be variably associated with thrombosis. We present one case of pulmonary thromboembolism in a young patient, who was positive for the JAK2 V617F mutation and did not have MPD.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.2
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• pp.443-449
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• 2015

Background: Myeloproliferative disorders (MPDs) are clonal hematologic malignancies originating at the level of the pluripotent hematopoietic stem cell. Matrix metalloproteases (MMPs) are proteolytic enzymes that contribute to all stages of malignancy progression. Genetic variants in the MMP genes may influence the biological function of these enzymes and change their role in carcinogenesis and progression. To our knowledge, this is the first investigation of associations between the -735 C/T and -1562 C/T polymorphisms in the MMP2 and MMP9 genes, respectively, and the risk of essential thrombocytosis (ET), and polycythemia vera (PV). Materials and Methods: The case-control study included JAK2V617F mutation positive 102 ET and PV patients and 111 controls. Polymorphisms were determined by using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) and electrophoresis. Results: No statistically significant differences were detected between patient (ET+PV) and control groups regarding genotype distribution for MMP2 gene-735 C/T and MMP9 gene -1562 C/T polymorphisms and C/T allele frequency (p>0.050). Statistically borderline significance was observed between PV and control groups regarding genotype distribution for the MMP9 gene -1562 C/T polymorphism (p=0.050, OR=2.26, 95%Cl=0.99-5.16). Conclusions: Consequently this study supported that CC genotype of MMP9 gene -1562 C/T polymorphism may be related with PV even if with borderline significance.