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Coexistent systemic mastocytosis and essential thrombocythemia complicated with monoclonal gammopathy and hypocomplementaemia

  • Case Report
  • Published:
Central European Journal of Medicine

Abstract

Hematological neoplasms associated with systemic mast cell disease are most frequently of myeloid origin. There are a few reports, however, of systemic mastocytosis (SM) cases associated with lymphoid or plasma cell neoplasms as well. In this report, the authors present a case of SM (with D816V mutation in the c-KIT gene) associated with JAK2 V617F mutation negative essential thrombocythemia. The leading symptom of the 78-year-old female was recurring hydrothorax that responded only to interferon alpha therapy. During the first year of therapy, the patient developed insulin-dependent diabetes and hypothyroidism. The hematological workup also revealed IgG kappa monoclonal gammopathy that was non-progressive in the following next three years. Low levels of complements without known clinical significance accompanied the entire picture.

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Authors and Affiliations

  1. 3rd Department of Internal Medicine, Semmelweis University, H-1125, Budapest, Hungary

    Judit Varkonyi, Pál Pánczél, Melinda Sperlagh, Lilian Varga, Henriette Farkas & István Karádi

  2. PhD School of Semmelweis University, H-1085, Budapest, Hungary

    Eszter Rausz

  3. 1st Department of Pathology, Semmelweis Unniversity, H-1083, Budapest, Hungary

    Judit Csomor & Tibor Füle

Authors
  1. Judit Varkonyi
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  2. Eszter Rausz
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  3. Pál Pánczél
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  4. Melinda Sperlagh
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  5. Lilian Varga
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  6. Henriette Farkas
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  7. Judit Csomor
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  9. István Karádi
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Correspondence to Judit Varkonyi.

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Varkonyi, J., Rausz, E., Pánczél, P. et al. Coexistent systemic mastocytosis and essential thrombocythemia complicated with monoclonal gammopathy and hypocomplementaemia. cent.eur.j.med 7, 742–746 (2012). https://doi.org/10.2478/s11536-012-0065-1

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  • DOI: https://doi.org/10.2478/s11536-012-0065-1

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