Atypische Manifestation einer primären systemischen Amyloidose: wiederkehrende Leberblutung und Hämoperitoneum
| Autoři | |
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| Rok publikování | 2011 |
| Druh | Konferenční abstrakty |
| Fakulta / Pracoviště MU | |
| Citace | |
| Popis | Introduction: Primary systemic amyloidosis is a protein misfolding disorder characterized by deposition of insoluble fibrils, derived from monoclonal light chains, in the extracellular matrix. Although the liver is a common site of amyloid deposition, clinically significant deteriorations of hepatic functions are rare and reports on spontaneous liver bleeding are exceptional. Herein we describe the case of a female, born 1950, repeatedly admitted to hospital with hemoperitoneum due to amyloidosis. Case Report: Dyspeptic symptoms and subfebrile temperatures were the first manifestations appearing in our patient in 2008. After finding a liver infiltration suspected of hemangioma, an embolization was performed in June 2009. This procedure was complicated by a liver rupture and the developement of a chronic subhepatal and retroperitoneal hematoma. However, the diagnosis of lambda-AL amyloidosis was determined from a liver biopsy as late as in April 2010. Due to the overall condition of the patient and advanced state of the disease, aggressive chemotherapy and liver or hematopoietic stem cell transplantations were contraindicated. In May 2010, the patient was started on low-intensity palliative chemotherapy with cyclophosphamide and dexamethasone. Based on radiological evaluations (CT, MRI, PET/CT), progressive hepatomegaly and formation of new subcapsular and intraparenchymal hematomas (maximum 16 x 6 cm in size) were confirmed. Two more epizodes of hemoperitoneum had occured in this patient (6/2010 and 10/2010) and the woman died of multiorgan failure in November 2010. Autopsy showed AL-amyloidosis with massive infiltration of the liver (weight 3820 g), spleen, kidneys, intraabdominal lymph nodes and suprarenal glands. The liver was extremely fragile and disintegrating at a touch. Conclusions: The cause behind ruptured and bleeding liver doesn't always need to be a hemangioma but also amyloidosis may be the case. The outlook for a complete haematological and organ treatment response is very limited in cases of advanced disease and in patients with contraindications for aggressive treatment. Thus an early diagnosis is of utmost importance. |