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...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Case Reports in Hepatology |
| Online Access: | http://dx.doi.org/10.1155/2012/728172 |
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| _version_ | 1849410956080185344 |
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| author | Stefanie Adolf Gunda Millonig Helmut Karl Seitz Andreas Reiter Peter Schirmacher Thomas Longerich Sebastian Mueller |
| author_facet | Stefanie Adolf Gunda Millonig Helmut Karl Seitz Andreas Reiter Peter Schirmacher Thomas Longerich Sebastian Mueller |
| author_sort | Stefanie Adolf |
| collection | DOAJ |
| 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 | Article |
| id | doaj-art-60b91fa7fd50435296d5b9d91a043740 |
| institution | Kabale University |
| issn | 2090-6587 2090-6595 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
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| series | Case Reports in Hepatology |
| spelling | doaj-art-60b91fa7fd50435296d5b9d91a0437402025-08-20T03:34:56ZengWileyCase Reports in Hepatology2090-65872090-65952012-01-01201210.1155/2012/728172728172Systemic Mastocytosis: A Rare Case of Increased Liver StiffnessStefanie Adolf0Gunda Millonig1Helmut Karl Seitz2Andreas Reiter3Peter Schirmacher4Thomas Longerich5Sebastian Mueller6Department of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, GermanyDepartment of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, GermanyDepartment of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, GermanyDepartment of Medicine III, Mannheim Hospital, University of Heidelberg, Wiesbadener Straße 7-11, 68305 Mannheim, GermanyInstitute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220/221, 69120 Heidelberg, GermanyInstitute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220/221, 69120 Heidelberg, GermanyDepartment of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, GermanyAssessment 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.http://dx.doi.org/10.1155/2012/728172 |
| spellingShingle | Stefanie Adolf Gunda Millonig Helmut Karl Seitz Andreas Reiter Peter Schirmacher Thomas Longerich Sebastian Mueller Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness Case Reports in Hepatology |
| 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 |
| url | http://dx.doi.org/10.1155/2012/728172 |
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