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New paraneoplastic syndrome in chronic basophilic leukemia

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Abstract

Chronic basophilic leukemia (CBL) is an extremely rare disorder. A female patient presented with recurrent attacks of chills, fever and abdominal pain was found to have simultaneous cyclic oscillation in leukocyte counts and C-reactive protein values. She was initially diagnosed with familial Mediterranean fever and treated with colchicine. Diagnosis of CBL was established by morphologic studies of peripheral blood and bone marrow. Her febrile attacks recurred with marked elevation in serum interleukin-6 (IL-6) level when basophil counts climbed to peak levels during cyclic oscillation. Molecular studies by real-time PCR showed IL-6 gene expression in neoplastic basophils separated by magnetic-activated cell sorting infiltrating the bone marrow, suggesting that IL-6 is released by neoplastic basophils of an underlying CBL, resulting in a new paraneoplastic syndrome that mimics autoinflammatory disorders.

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References

  1. Pardanani AD, Morice WG, Hoyer JD, Tefferi A. Chronic basophilic leukemia: a distinct clinico-pathologic entity. Eur J Haematol. 2003;71:18–22.

    Article  PubMed  Google Scholar 

  2. Tang G, Woods LJ, Wang SA, Brettler D, Andersen M, Miron PM, Peched L, Woda BA, Hao S. Chronic basophilic leukemia: a rare form of chronic myeloproliferative neoplasm. Hum Pathol. 2009;40:1194–9.

    Article  PubMed  Google Scholar 

  3. Lahortiga I, Akin C, Cools J, Wilson TM, Mentens N, Arthur DC, Maric I, Noel P, Kocabas C, Marynen P, Lessin LS, Wlodarska I, Robyn C, Metcalfe DD. Activity of imatinib in systemic mastocytosis with chronic basophilic leukemia and a PRKG2–PDGFRB fusion. Haematol Hematol J. 2008;93:49–56.

    Article  CAS  Google Scholar 

  4. Pfaffl MW. A new mathematical model for relative quantification in real time RT-PCR. Nucleic Acids Res. 2001;29:2002–7.

    Article  Google Scholar 

  5. Pardanani A. Systemic mastocytosis in adults: 2012 update on diagnosis, risk stratification and management. Am J Hematol. 2012;87:402–11.

    Article  Google Scholar 

  6. Gatti RA, Robinson WA, Deinard AS, Nesbit M, McCullough JJ, Ballow M, Good RA. Cyclic leukocytosis in chronic myelogenous leukemia: new perspectives on pathogenesis and therapy. Blood. 1973;41:771–82.

    PubMed  CAS  Google Scholar 

  7. Mehta BC, Agarwal MB. Cyclic oscillations in leukocyte count in chronic myeloid leukemia. Acta Haematol. 1980;63:68–70.

    Article  PubMed  CAS  Google Scholar 

  8. Sechet B, Meseri-Delwail A, Arock M, Wijdenes J, Lecron J-C, Sarrouilhe D, et al. Immunoglobulin D enhances interleukin-6 release from the KU812 human prebasophil cell line. Gen Physiol Biophys. 2003;22:255–63.

    PubMed  CAS  Google Scholar 

  9. Han X, Jorgensen JL, Brahmandam A, Schlette E, Huh YO, Shi Y, Awagu S, Chen W. Immunophenotypic study of basophils by multiparameter flow cytometry. Arc Pathol Lab Med. 2008;132:813–9.

    Google Scholar 

  10. Toba K, Koike T, Shibata A, Hashimato S, Takahashi M, Masuka M, Azegami T, Takahashi H, Alzawa Y. Novel technique for the direct flow cytofluorometric analysis of human basophils in unseperated blood and bone marrow, and the characterization of phenotype and peroxidase of human basophils. Cytometry. 1999;35:249–59.

    Article  PubMed  CAS  Google Scholar 

  11. Chen K, Cerutti A. The function and regulation of immunoglobulin D. Curr Opin Immunol. 2011;23:345–52.

    Article  PubMed  Google Scholar 

  12. Rosenthal S, Schwartz JH, Canellos GP. Basophilic chronic granulocytic leukemia with hyperhistaminemia. Br J Haematol. 1977;36:367–72.

    Article  PubMed  CAS  Google Scholar 

  13. Guzmán C, Hallal-Caleros C, Lopez-Griego L, Morales-Montor J. Interleukin-6: a cytokine with a pleiotropic role in the neuroimmunoendocrine network. Open Neuroendocrinol J. 2010;3:152–60.

    Google Scholar 

  14. Li JT, Hou FF, Gou ZJ, Shan YX, Zhang X, Liu ZQ. Advanced glycation end products upregulate C-reactive protein synthesis by human hepatocytes through stimulation of monocyte IL-6 and IL-1β production. Scand J Immunol. 2007;66:555–62.

    Article  CAS  Google Scholar 

  15. Dinerello CA, Cannon JG, Mancilla J, Bishai I, Lees J, Coceani F. Interleukin-6 as an endogenous pyrogeN induction of prostaglandin E2 in brain, but not in peripheral blood mononuclear cells. Brain Res. 1991;562:199–206.

    Article  Google Scholar 

  16. Touitou S. Autoinflammatory diseases. IN 2008 Best Practice & Research Clinical Rheumatology. vol. 22. 2008. p. 811–829.

  17. El-Osta HE, Kurzrock R. Castleman’s disease: from basic mechanisms to molecular therapies. Oncologist. 2011;16:497–511.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

The authors are greatly indebted to the Dokuz Eylul University Supportive Society of The Department of Hematology & Oncology in Izmir Turkey for the grant support.

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Authors and Affiliations

  1. Division of Hematology, Dokuz Eylul University School of Medicine, 35330, Inciralti, Izmir, Turkey

    Cavit Cehreli & Fatih Demirkan

  2. Division of Hematology, Dokuz Eylul University School of Medicine, Izmir, Turkey

    Halil Ates

  3. Institute of Oncology, Dokuz Eylul University, Izmir, Turkey

    Ruksan Cehreli

  4. Departments of Medical Biology and Genetics, Dokuz Eylul University School of Medicine, Izmir, Turkey

    Zeynep Sercan

Authors
  1. Cavit Cehreli
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  2. Halil Ates
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  3. Ruksan Cehreli
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  4. Zeynep Sercan
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  5. Fatih Demirkan
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Correspondence to Cavit Cehreli.

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Cehreli, C., Ates, H., Cehreli, R. et al. New paraneoplastic syndrome in chronic basophilic leukemia. Int J Hematol 97, 498–504 (2013). https://doi.org/10.1007/s12185-013-1281-4

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  • DOI: https://doi.org/10.1007/s12185-013-1281-4

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