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 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.
Department of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, Germany.
Stefanie
S
Adolf
Department of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, Germany.
Gunda
G
Millonig
Department of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, Germany.
Helmut Karl
HK
Seitz
Department of Medicine III, Mannheim Hospital, University of Heidelberg, Wiesbadener Straße 7-11, 68305 Mannheim, Germany.
Andreas
A
Reiter
Institute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220/221, 69120 Heidelberg, Germany.
Peter
P
Schirmacher
Institute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220/221, 69120 Heidelberg, Germany.
Thomas
T
Longerich
Department of Medicine, Salem Medical Center and Alcohol Research Center, University of Heidelberg, Zeppelinstraße 11-33, 69121 Heidelberg, Germany.
Sebastian
S
Mueller