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[Systemic mastocytosis with progressive disease course].

Authors
  • Várkonyi, Judit1
  • Szombath, Gergely1
  • Vályi-Nagy, Anna1
  • Csomor, Judit2
  • Egedi, Krisztina2
  • Kovalszky, Ilona2
  • Tölgyesi, Katalin3
  • Szerafin, László3
  • Tóth, László4
  • Soós, Györgyi4
  • Masszi, Tamás1
  • 1 III. Belgyógyászati Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest, Kútvölgyi út 4., 1125.
  • 2 I. Patológiai Intézet, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.
  • 3 Hematológiai Osztály és Patológia Osztály, Jósa András Oktató Kórház Nyíregyháza.
  • 4 Patológiai Intézet, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen.
Type
Published Article
Journal
Orvosi hetilap
Publication Date
Feb 01, 2018
Volume
159
Issue
5
Pages
192–196
Identifiers
DOI: 10.1556/650.2018.30978
PMID: 29376426
Source
Medline
Keywords
License
Unknown

Abstract

Authors report on a case of a male patient of systemic mastocytosis that was associated with extensive cutaneous lesions. Chronic diarrhoea worsening his quality of life was well managed by the administration of antihistamines. The pleural fluid recurrence soon after drainage has been controlled by the administration of alpha interferon. 40 years after the onset of the first skin signs progression has been manifested in the development of "B" (bone marrow infiltration rate >30%, dysmyelopoiesis, serum tryptase >20 μg/L, hepato- and splenomegaly) and "C" symptoms (liver function test abnormalities, cytopenia, malabsorption, osteoporosis). The patient died at age of 87. The authors' aim was to attract attention on this rare disease and emphasize that symptomatic therapy with antihistamines and drugs available based on customised rights by the National Health Insurance Fund might provide good quality of life. Orv Hetil. 2018; 159(5): 192-196.

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