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

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Varkonyi J., Rausz E., Pánczél P., Sperlagh M., Varga L., Farkas H., Csomor J., Füle T., Karádi I.

Open Medicine

2012

vol. 7

issue 6

742-746

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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1 Open Medicine

1 2012

1 Csomor J.

1 Farkas H.

1 Füle T.

1 Karádi I.

1 Pánczél P.

1 Rausz E.

1 Sperlagh M.

1 Varga L.

1 Varkonyi J.

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