Erdheim-Chester-betegség

A histiocytosis ritka formája

Translated title of the contribution: Erdheim-Chester disease: A rare form of histiocytosis

Németh István, G. Bogáts, Kaiser László, L. Tiszlavicz

Research output: Contribution to journalArticle

Abstract

INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT- Histiocytosis presented in a 57-year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim-Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.

Original languageHungarian
Pages (from-to)317-320
Number of pages4
JournalLege Artis Medicinae
Volume17
Issue number4-5
Publication statusPublished - Apr 2007

Fingerprint

Erdheim-Chester Disease
Histiocytosis
Non-Langerhans-Cell Histiocytosis
Histiocytes
Pericardial Effusion
Autopsy
Bone and Bones
Therapeutics

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Erdheim-Chester-betegség : A histiocytosis ritka formája. / István, Németh; Bogáts, G.; László, Kaiser; Tiszlavicz, L.

In: Lege Artis Medicinae, Vol. 17, No. 4-5, 04.2007, p. 317-320.

Research output: Contribution to journalArticle

István, Németh ; Bogáts, G. ; László, Kaiser ; Tiszlavicz, L. / Erdheim-Chester-betegség : A histiocytosis ritka formája. In: Lege Artis Medicinae. 2007 ; Vol. 17, No. 4-5. pp. 317-320.
@article{541a07962e0c4d209c9ed90f6052c77e,
title = "Erdheim-Chester-betegs{\'e}g: A histiocytosis ritka form{\'a}ja",
abstract = "INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT- Histiocytosis presented in a 57-year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim-Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.",
keywords = "Erdheim-chester disease, Histiocytosis",
author = "N{\'e}meth Istv{\'a}n and G. Bog{\'a}ts and Kaiser L{\'a}szl{\'o} and L. Tiszlavicz",
year = "2007",
month = "4",
language = "Hungarian",
volume = "17",
pages = "317--320",
journal = "Lege Artis Medicinae",
issn = "0866-4811",
publisher = "Literatura Medica Publishing House",
number = "4-5",

}

TY - JOUR

T1 - Erdheim-Chester-betegség

T2 - A histiocytosis ritka formája

AU - István, Németh

AU - Bogáts, G.

AU - László, Kaiser

AU - Tiszlavicz, L.

PY - 2007/4

Y1 - 2007/4

N2 - INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT- Histiocytosis presented in a 57-year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim-Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.

AB - INTRODUCTION - Erdheim-Chester disease is rare non-Langerhans cell histiocytosis with multisystem involvement. The histological hallmark of the disease is CD1a negative histiocyte proliferation. CASE REPORT- Histiocytosis presented in a 57-year-old Caucasian man with exophtalmus, which was after two years followed by progressive pericardial infiltration and effusion leading to cardiac compression and consequent functional disturbance. It was the histological analysis of a surgical sample removed during pericardial fenestration that revealed Erdheim-Chester disease. The patient died from multisystem involvement one year after the initial diagnosis. Postmortem examination showed long bone, orbital cavity and cranial cavity involvement as well as extensive retroperitoneal and mediastinal xanthogranulomatous infiltration. CONCLUSIONS - The clinical diagnosis of Erdheim-Chester disease is difficult because the symptoms are insidious and non-specific. The histological report is often only descriptive. The prognosis of the disease is poor due to the lack of targeted therapy and to the advanced stage at diagnosis.

KW - Erdheim-chester disease

KW - Histiocytosis

UR - http://www.scopus.com/inward/record.url?scp=34249861809&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=34249861809&partnerID=8YFLogxK

M3 - Article

VL - 17

SP - 317

EP - 320

JO - Lege Artis Medicinae

JF - Lege Artis Medicinae

SN - 0866-4811

IS - 4-5

ER -