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Journal

2012 | 7 | 6 | 742-746

Article title

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

Content

Title variants

Languages of publication

EN

Abstracts

EN
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.

Publisher

Journal

Year

Volume

7

Issue

6

Pages

742-746

Physical description

Dates

published
1 - 12 - 2012
online
11 - 10 - 2012

Contributors

  • 3rd Department of Internal Medicine, Semmelweis University, H-1125, Budapest, Hungary
author
  • PhD School of Semmelweis University, H-1085, Budapest, Hungary
  • 3rd Department of Internal Medicine, Semmelweis University, H-1125, Budapest, Hungary
  • 3rd Department of Internal Medicine, Semmelweis University, H-1125, Budapest, Hungary
author
  • 3rd Department of Internal Medicine, Semmelweis University, H-1125, Budapest, Hungary
  • 3rd Department of Internal Medicine, Semmelweis University, H-1125, Budapest, Hungary
author
  • 1st Department of Pathology, Semmelweis Unniversity, H-1083, Budapest, Hungary
author
  • 1st Department of Pathology, Semmelweis Unniversity, H-1083, Budapest, Hungary
  • 3rd Department of Internal Medicine, Semmelweis University, H-1125, Budapest, Hungary

References

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Document Type

Publication order reference

Identifiers

YADDA identifier

bwmeta1.element.-psjd-doi-10_2478_s11536-012-0065-1
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