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

J. Várkonyi, Eszter Rausz, P. Pánczél, Melinda Sperlagh, L. Varga, H. Farkas, Judit Csomor, T. Füle, I. 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 2012

Fingerprint

Systemic Mastocytosis
Essential Thrombocythemia
Paraproteinemias
Plasma Cell Neoplasms
Hydrothorax
Mutation
Hematologic Neoplasms
Hypothyroidism
Interferon-alpha
Immunoglobulin G
Lymphocytes
Insulin
Therapeutics
Genes

Keywords

  • Hypocomplementaemia
  • Interferon alpha
  • Monoclonal gammopathy
  • Systemic mastocytosis

ASJC Scopus subject areas

  • Medicine(all)

Cite this

@article{bc163ac96b6f4ba4b3685dae0030a808,
title = "Coexistent systemic mastocytosis and essential thrombocythemia complicated with monoclonal gammopathy and hypocomplementaemia",
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.",
keywords = "Hypocomplementaemia, Interferon alpha, Monoclonal gammopathy, Systemic mastocytosis",
author = "J. V{\'a}rkonyi and Eszter Rausz and P. P{\'a}ncz{\'e}l and Melinda Sperlagh and L. Varga and H. Farkas and Judit Csomor and T. F{\"u}le and I. Kar{\'a}di",
year = "2012",
month = "12",
doi = "10.2478/s11536-012-0065-1",
language = "English",
volume = "7",
pages = "742--746",
journal = "Central European Journal of Medicine",
issn = "1895-1058",
publisher = "Springer Verlag",
number = "6",

}

TY - JOUR

T1 - Coexistent systemic mastocytosis and essential thrombocythemia complicated with monoclonal gammopathy and hypocomplementaemia

AU - Várkonyi, J.

AU - Rausz, Eszter

AU - Pánczél, P.

AU - Sperlagh, Melinda

AU - Varga, L.

AU - Farkas, H.

AU - Csomor, Judit

AU - Füle, T.

AU - Karádi, I.

PY - 2012/12

Y1 - 2012/12

N2 - 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.

AB - 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.

KW - Hypocomplementaemia

KW - Interferon alpha

KW - Monoclonal gammopathy

KW - Systemic mastocytosis

UR - http://www.scopus.com/inward/record.url?scp=84872687698&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84872687698&partnerID=8YFLogxK

U2 - 10.2478/s11536-012-0065-1

DO - 10.2478/s11536-012-0065-1

M3 - Article

VL - 7

SP - 742

EP - 746

JO - Central European Journal of Medicine

JF - Central European Journal of Medicine

SN - 1895-1058

IS - 6

ER -