PL EN


Preferences help
enabled [disable] Abstract
Number of results
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, varkjud@kut.sote.hu
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
  • [1] Valent P., Horny H.P., Escribano L., Longley B.J., Li C.Y., Schwartz L.B., Marone G., Nunez R., Akin C., Sotlar K., Sperr W.R., Wolff K., Brunning R.D., Parwaresch R.M., Austen K.F., Lennert K., Vardiman J.W., Bennett J.M. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leukemia Research 2001, 25, 603–625. http://dx.doi.org/10.1016/S0145-2126(01)00038-8[Crossref]
  • [2] Valent P., Akin C., Escribano L. Födinger M., Hartmann K., Brockow K., Castells M., Sperr W. R., Kluin-Nelemans H. C., Hamdy N. A. T., Lortholary O., Robyn J., van Doormaal J., Sotlar K., Hauswirth A.W., Arock M., Hermine O., Hellmann A., Triggiani M., Niedoszytko M. Standards and standardization in mastocytosis: Consensus Statements on Diagnostics, Treatment Recommendations and Response Criteria. European Journal of Clinical Investigation 2007,37, 435–453. http://dx.doi.org/10.1111/j.1365-2362.2007.01807.x[Crossref][WoS]
  • [3] Hagen W., Schwarzmeier J., Walchshofer S., et al. A case of bone marrow mastocytosis associated with multiple myeloma. Ann. Haematol. 1998,76, 167–74. http://dx.doi.org/10.1007/s002770050382[Crossref]
  • [4] Stellmacher F., Sotlar K., Balleisen L., et al. Bone marrow mastocytosis associated with IgM Kappa plasma cell myeloma. Leukemia Lymphoma 2004, 45, 801–805. http://dx.doi.org/10.1080/10428190310001615693[Crossref]
  • [5] Sotlar K., Saeger W., Stellmacher F., Stahmer J., Jäckle S., Valent P., Horny H-P. “Occult” mastocytosis with activating c-kit point mutation evolving into systemic mastocytosis associated with plasma cell myeloma and secondary amyloidosis J. Clin. Pathol. 2006,59, 875–878. http://dx.doi.org/10.1136/jcp.2005.028837[Crossref]
  • [6] Pullarkat S.T., Sedarat F., Paquette R. et al. Systemic Mastocytosis with Plasma. Cell Dyscrasia: report of a case. Leuk. Res. 2008, 32, 1160–1163 http://dx.doi.org/10.1016/j.leukres.2007.10.017[Crossref][WoS]
  • [7] Motwani P., Kocoglu M., Lorsbach R.B. Systemic mastocytosis in association with plasma cell dyscrasias: report of a case and review of the literature. Leuk. Res. 2009, 33, 856–859 http://dx.doi.org/10.1016/j.leukres.2008.11.010[Crossref][WoS]
  • [8] Kluin-Nelemans H.C., Ferenc V., van Doormaal J.J., van Iperen C., Peters W.G., Akin C., Valent P. Lenalidomide therapy in systemic mastocytosis. Leuk. Res. 2009, 33, 19–22 http://dx.doi.org/10.1016/j.leukres.2008.06.013[Crossref]
  • [9] Giclas P.C. Classical pathway evaluation. Curr. Protoc. Immunol. 2001, Chapter 13, Unit 13.1 [Crossref]
  • [10] Minh D.Q., Czink E., Mod A., Fust G., Hollan S.R. Serial complement measurements in patients with leukaemia. Clin. Lab. Haematol. 1983, 5, 23–34. http://dx.doi.org/10.1111/j.1365-2257.1983.tb00493.x[Crossref]
  • [11] Delamarche C., Berger F., Pouplard A., Emile J. An ELISA technique for the measurement of C1q in cerebrospinal fluid. J. Immunol Methods 1988, 114, 101–106 http://dx.doi.org/10.1016/0022-1759(88)90160-3[Crossref]
  • [12] Swerdlow S.H., Campo E., Harris N.L., et al, editors. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC; 2008
  • [13] Raanani P., Ben Bassat I. Immune-mediated complications during interferon therapy in hematological patients. Acta Haemtol. 2002,107, 133–144 http://dx.doi.org/10.1159/000057631[Crossref]
  • [14] Vardiman J.W., Harris N.L., Brunning R.D. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood, 2002,100, 2292–2302 http://dx.doi.org/10.1182/blood-2002-04-1199[Crossref]
  • [15] Cugno M., Castelli R., Cicardi M. Angioedema due to acquired C1 - inhibitor deficiency: A bridging condition between autoimmunity and lymphoproliferation. Autoimmunity Reviews 2008, 8, 156–159 http://dx.doi.org/10.1016/j.autrev.2008.05.003[Crossref][WoS]
  • [16] van Spronsen D.J., Hoorntje S.J., Hannema A.J., Hack C.E. Acquired angio-oedema caused by IgA paraprotein. Neth. J. Med. 1998, 52, 22–25 http://dx.doi.org/10.1016/S0300-2977(97)00066-1[Crossref]
  • [17] Gordon E.H., Beall G.N., Klaustermeyer W.B. Angioedema and multiple myeloma. Ann. Allergy 1983, 51, 378–380
Document Type
Publication order reference
Identifiers
YADDA identifier
bwmeta1.element.-psjd-doi-10_2478_s11536-012-0065-1
JavaScript is turned off in your web browser. Turn it on to take full advantage of this site, then refresh the page.