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Correlation between JAK2 allele burden and pulmonary arterial hypertension and hematological parameters in Philadelphia negative JAK2 positive myeloproliferative neoplasms. An Egyptian experience

Annals of Hematology volume 95, pages 1611–1616 (2016)Cite this article

Abstract

Myeloproliferative neoplasms are characterized by a common stem cell-derived clonal proliferation, but are phenotypically diverse. JAK2 is mutated (V617F) in more than 90 % of patients with polycythemia vera (PV) and approximately 60 % of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). Pulmonary arterial hypertension (PAH) is a major complication of several hematological disorders. Chronic myeloproliferative disorders associated with PAH have been included in group five for which the etiology is unclear and/or multifactorial. The aim of this study is to screen Egyptian Philadelphia negative JAK2 positive myeloproliferative neoplasm patients for the presence of PAH and its correlation with JAK2 allele burden. We also made a review for correlation of JAK2 allele with hematological parameters comparing our results to others. We enrolled 60 patients with Philadelphia negative myeloproliferative neoplasms. All patients enrolled in the study were subjected to laboratory and imaging workup in the form of CBC, liver, kidney profile, bone marrow examination, abdominal ultrasonography, and transthoracic echocardiography. Our results revealed that 7 patients out of 60 (11.67 %) had pulmonary arterial hypertension, 3 patients with PMF, 2 patients with PRV, and 2 patients with ET, and its correlation with JAK2 allele burden was not statistically significant. Correlation analysis between JAK2 V617F allele burden and other parameters revealed: statistical significant correlation with age, HB, HCT, PLT, UA, LDH, and splenic diameter but insignificant correlation with WBCs and PAH. Pulmonary arterial hypertension prevalence in our study was 11.67 % and no significant correlation with JAK 2 allele burden. Our study is the largest one up to our knowledge that studies the association between its prevalence and JAK2 burden.

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Acknowledgment

Special thanks to all workers and patients of hematolgy clinic. Mervat Mattar designed the research study and revised the results and the paper. Mohammed Abdel Kader selected the patients and participated in collecting references for paper. Noha Ali did the ECHO to the patients. Doaa El Demerdash aided in writing the paper. Noha El Husseiny performed statistics and wrote the paper.

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Affiliations

  1. Clinical Hematology Unit, Internal Medicine, Cairo, Egypt

    Mervat M. Mattar, Mohammed Abdel Kader Morad, Noha M. El Husseiny & Doaa M. El Demerdash

  2. Cardiology Department, Faculty of Medicine, Cairo University, Cairo, Egypt

    Noha H. Ali

Authors
  1. Mervat M. Mattar
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  2. Mohammed Abdel Kader Morad
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  3. Noha M. El Husseiny
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  4. Noha H. Ali
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  5. Doaa M. El Demerdash
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Correspondence to Noha M. El Husseiny.

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Mattar, M.M., Morad, M.A.K., El Husseiny, N.M. et al. Correlation between JAK2 allele burden and pulmonary arterial hypertension and hematological parameters in Philadelphia negative JAK2 positive myeloproliferative neoplasms. An Egyptian experience. Ann Hematol 95, 1611–1616 (2016). https://doi.org/10.1007/s00277-016-2765-0

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  • DOI: https://doi.org/10.1007/s00277-016-2765-0

Keywords

  • Myeloproliferative neoplasm
  • Pulmonary hypertension
  • JAK2 mutation