Seltene Ursache einer Fundusvarizenblutung

J Naderi; A W Lohse; P R Galle; C Schramm

doi:10.1055/s-0029-1245465

Seltene Ursache einer Fundusvarizenblutung

Standard

Seltene Ursache einer Fundusvarizenblutung. / Naderi, J; Lohse, A W; Galle, P R; Schramm, C.

In: Z GASTROENTEROL, Vol. 48, No. 10, 10.2010, p. 1215-8.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Naderi, J, Lohse, AW, Galle, PR & Schramm, C 2010, 'Seltene Ursache einer Fundusvarizenblutung', Z GASTROENTEROL, vol. 48, no. 10, pp. 1215-8. https://doi.org/10.1055/s-0029-1245465

APA

Naderi, J., Lohse, A. W., Galle, P. R., & Schramm, C. (2010). Seltene Ursache einer Fundusvarizenblutung. Z GASTROENTEROL, 48(10), 1215-8. https://doi.org/10.1055/s-0029-1245465

Vancouver

Naderi J, Lohse AW, Galle PR, Schramm C. Seltene Ursache einer Fundusvarizenblutung. Z GASTROENTEROL. 2010 Oct;48(10):1215-8. https://doi.org/10.1055/s-0029-1245465

Bibtex

@article{1c368a7bde8e422ead384426863a70ca,

title = "Seltene Ursache einer Fundusvarizenblutung",

abstract = "BACKGROUND: Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disease, which is characterized by myelofibrosis, osteosclerosis and pancytopenia. Affected patients frequently develop portal hypertension secondary to extramedullary hematopoiesis, which rarely becomes clinically relevant.CASE REPORT: We here report on a 63-year-old patient with a first presentation of variceal bleeding. The patient was diagnosed with portal hypertension and due to marked splenomegaly, liver cirrhosis was suspected. Subsequently, an extramedullary hematopoiesis and AMM could be diagnosed. The variceal bleeding was initially treated endoscopically. The patient has received cytoreductive therapy and a splenorenal shunt, which has led to remission and long-term survival.CONCLUSION: Agnogenic myeloid metaplasia is a rare cause of portal hypertension and may present with variceal bleeding. Clinicians should be aware of this rare cause of portal hypertension, as treatment of the underlying disease with cytoreductive therapy and appropriate management of portal hypertension may lead to long-term survival.",

keywords = "Diagnosis, Differential, Esophageal and Gastric Varices, Female, Gastrointestinal Hemorrhage, Humans, Middle Aged, Primary Myelofibrosis, Rare Diseases, Case Reports, English Abstract, Journal Article",

author = "J Naderi and Lohse, {A W} and Galle, {P R} and C Schramm",

note = "{\textcopyright} Georg Thieme Verlag KG Stuttgart · New York.",

year = "2010",

month = oct,

doi = "10.1055/s-0029-1245465",

language = "Deutsch",

volume = "48",

pages = "1215--8",

journal = "Z GASTROENTEROL",

issn = "0044-2771",

publisher = "Karl Demeter Verlag GmbH",

number = "10",

}

RIS

TY - JOUR

T1 - Seltene Ursache einer Fundusvarizenblutung

AU - Naderi, J

AU - Lohse, A W

AU - Galle, P R

AU - Schramm, C

N1 - © Georg Thieme Verlag KG Stuttgart · New York.

PY - 2010/10

Y1 - 2010/10

N2 - BACKGROUND: Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disease, which is characterized by myelofibrosis, osteosclerosis and pancytopenia. Affected patients frequently develop portal hypertension secondary to extramedullary hematopoiesis, which rarely becomes clinically relevant.CASE REPORT: We here report on a 63-year-old patient with a first presentation of variceal bleeding. The patient was diagnosed with portal hypertension and due to marked splenomegaly, liver cirrhosis was suspected. Subsequently, an extramedullary hematopoiesis and AMM could be diagnosed. The variceal bleeding was initially treated endoscopically. The patient has received cytoreductive therapy and a splenorenal shunt, which has led to remission and long-term survival.CONCLUSION: Agnogenic myeloid metaplasia is a rare cause of portal hypertension and may present with variceal bleeding. Clinicians should be aware of this rare cause of portal hypertension, as treatment of the underlying disease with cytoreductive therapy and appropriate management of portal hypertension may lead to long-term survival.

AB - BACKGROUND: Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disease, which is characterized by myelofibrosis, osteosclerosis and pancytopenia. Affected patients frequently develop portal hypertension secondary to extramedullary hematopoiesis, which rarely becomes clinically relevant.CASE REPORT: We here report on a 63-year-old patient with a first presentation of variceal bleeding. The patient was diagnosed with portal hypertension and due to marked splenomegaly, liver cirrhosis was suspected. Subsequently, an extramedullary hematopoiesis and AMM could be diagnosed. The variceal bleeding was initially treated endoscopically. The patient has received cytoreductive therapy and a splenorenal shunt, which has led to remission and long-term survival.CONCLUSION: Agnogenic myeloid metaplasia is a rare cause of portal hypertension and may present with variceal bleeding. Clinicians should be aware of this rare cause of portal hypertension, as treatment of the underlying disease with cytoreductive therapy and appropriate management of portal hypertension may lead to long-term survival.

KW - Diagnosis, Differential

KW - Esophageal and Gastric Varices

KW - Female

KW - Gastrointestinal Hemorrhage

KW - Humans

KW - Middle Aged

KW - Primary Myelofibrosis

KW - Rare Diseases

KW - Case Reports

KW - English Abstract

KW - Journal Article

U2 - 10.1055/s-0029-1245465

DO - 10.1055/s-0029-1245465

M3 - SCORING: Zeitschriftenaufsatz

C2 - 20886427

VL - 48

SP - 1215

EP - 1218

JO - Z GASTROENTEROL

JF - Z GASTROENTEROL

SN - 0044-2771

IS - 10

ER -