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

Judit Varkonyi, Eszter Rausz, Pál Pánczél, Melinda Sperlagh, Lilian Varga, Henriette Farkas, Judit Csomor, Tibor Füle, István Karádi

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)742-746
Number of pages5
JournalCentral European Journal of Medicine
Volume7
Issue number6
DOIs
Publication statusPublished - Dec 1 2012

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Keywords

  • Hypocomplementaemia
  • Interferon alpha
  • Monoclonal gammopathy
  • Systemic mastocytosis

ASJC Scopus subject areas

  • Medicine(all)

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