Cargando…
Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness
Assessment of liver stiffness (LS) by transient elastography (Fibroscan) has significantly improved the noninvasive diagnosis of liver fibrosis. We here report on a 55-year-old patient with drastically increased LS due to previously unknown systemic mastocytosis. The patient initially presented with...
Autores principales: | , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2012
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4208443/ https://www.ncbi.nlm.nih.gov/pubmed/25374708 http://dx.doi.org/10.1155/2012/728172 |
_version_ | 1782341133469220864 |
---|---|
author | Adolf, Stefanie Millonig, Gunda Seitz, Helmut Karl Reiter, Andreas Schirmacher, Peter Longerich, Thomas Mueller, Sebastian |
author_facet | Adolf, Stefanie Millonig, Gunda Seitz, Helmut Karl Reiter, Andreas Schirmacher, Peter Longerich, Thomas Mueller, Sebastian |
author_sort | Adolf, Stefanie |
collection | PubMed |
description | Assessment of liver stiffness (LS) by transient elastography (Fibroscan) has significantly improved the noninvasive diagnosis of liver fibrosis. We here report on a 55-year-old patient with drastically increased LS due to previously unknown systemic mastocytosis. The patient initially presented with increased weight loss, nocturnal pruritus, increased transaminases, bilirubinemia, and thrombocytopenia. Abdominal ultrasound showed ascites, hepatomegaly, and splenomegaly. In addition, LS was 75 kPa (IQR 0 kPa) clearly exceeding the cut-off value for F4 cirrhosis of 12.5 kPa. However, histological analysis of the liver specimen indicated liver involvement by systemic mastocytosis and excluded liver cirrhosis. An additional CT scan detected disseminated bone lesions. After three months of treatment with Midostaurin, LS slightly decreased down to 31.9 kPa (IQR 8.3 kPa). This case illustrates that diffused sinusoidal neoplastic infiltrates are a pitfall in the non-invasive diagnosis of liver cirrhosis. In conclusion, refined clinical algorithms for increased LS should also include mastocytosis in addition to inflammation, congestion, and biliary obstruction. |
format | Online Article Text |
id | pubmed-4208443 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-42084432014-11-05 Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness Adolf, Stefanie Millonig, Gunda Seitz, Helmut Karl Reiter, Andreas Schirmacher, Peter Longerich, Thomas Mueller, Sebastian Case Reports Hepatol Case Report Assessment of liver stiffness (LS) by transient elastography (Fibroscan) has significantly improved the noninvasive diagnosis of liver fibrosis. We here report on a 55-year-old patient with drastically increased LS due to previously unknown systemic mastocytosis. The patient initially presented with increased weight loss, nocturnal pruritus, increased transaminases, bilirubinemia, and thrombocytopenia. Abdominal ultrasound showed ascites, hepatomegaly, and splenomegaly. In addition, LS was 75 kPa (IQR 0 kPa) clearly exceeding the cut-off value for F4 cirrhosis of 12.5 kPa. However, histological analysis of the liver specimen indicated liver involvement by systemic mastocytosis and excluded liver cirrhosis. An additional CT scan detected disseminated bone lesions. After three months of treatment with Midostaurin, LS slightly decreased down to 31.9 kPa (IQR 8.3 kPa). This case illustrates that diffused sinusoidal neoplastic infiltrates are a pitfall in the non-invasive diagnosis of liver cirrhosis. In conclusion, refined clinical algorithms for increased LS should also include mastocytosis in addition to inflammation, congestion, and biliary obstruction. Hindawi Publishing Corporation 2012 2012-10-11 /pmc/articles/PMC4208443/ /pubmed/25374708 http://dx.doi.org/10.1155/2012/728172 Text en Copyright © 2012 Stefanie Adolf et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Adolf, Stefanie Millonig, Gunda Seitz, Helmut Karl Reiter, Andreas Schirmacher, Peter Longerich, Thomas Mueller, Sebastian Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness |
title | Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness |
title_full | Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness |
title_fullStr | Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness |
title_full_unstemmed | Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness |
title_short | Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness |
title_sort | systemic mastocytosis: a rare case of increased liver stiffness |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4208443/ https://www.ncbi.nlm.nih.gov/pubmed/25374708 http://dx.doi.org/10.1155/2012/728172 |
work_keys_str_mv | AT adolfstefanie systemicmastocytosisararecaseofincreasedliverstiffness AT milloniggunda systemicmastocytosisararecaseofincreasedliverstiffness AT seitzhelmutkarl systemicmastocytosisararecaseofincreasedliverstiffness AT reiterandreas systemicmastocytosisararecaseofincreasedliverstiffness AT schirmacherpeter systemicmastocytosisararecaseofincreasedliverstiffness AT longerichthomas systemicmastocytosisararecaseofincreasedliverstiffness AT muellersebastian systemicmastocytosisararecaseofincreasedliverstiffness |