Von-Willebrand factor as a predictor of three-month mortality in patients with liver cirrhosis compared to MELD score.

INTRODUCTION AND AIM: Endothelial dysfunction is involved in the pathogenesis of portal hypertension and in the progression of liver disease. As an indicator of endothelial dysfunction, von Willebrand factor (vWF-Ag) can be a useful mortality predictor in patients with liver cirrhosis. The aim of the study is to compare the predictive value of vWF-Ag with the predictive value of MELD score regarding the three-month mortality in patients with liver cirrhosis. MATERIALS AND METHODS: In 70 patients with cirrhosis and portal hypertension we measured the vWF-Ag concentration and we followed the patients for 90 days. We registered all manifestations and complications of liver cirrhosis and the three-month mortality was the main end-point. RESULTS: We registered mean vWF-Ag of 341.9±155.8%, median 312%, IQR (214-410), vWF-Ag significantly correlated with MELD score (R=0.3713 ; p<0.05) and vWF-Ag median was higher in the uncensored compared to the median in the censored patients (p<0.0067). vWF-Ag and MELD score were significantly associated with three-month mortality, with no significant difference in the diagnostic performance between the two parameters [AUC=0.735, p=0.007 for vWF-Ag ; AUC=0.885, p=0.000 for MELD score], (Z=1.473, p=0.1407). CONCLUSION: In patients with liver cirrhosis vWF-Ag is a relevant predictor of three-month mortality that equals the MELD score.

NonHodgkin's Lymphoma with Peritoneal Localization.

The gastrointestinal tract is the most common extranodal site involved with lymphoma accounting for 5-20% of all cases. Lymphoma can occur at any site of the body, but diffuse and extensive involvement of the peritoneal cavity is unusual and rare. We report a case of diffuse large B-cell lymphoma in a 57-year-old female infiltrating the peritoneum and omentum and presenting with ascites and pleural effusion. The performed examinations did not discover any pathological findings affecting the digestive tract or parenchymal organs, except for diffuse thickening of the peritoneum and omentum. Peripheral, mediastinal, or retroperitoneal lymphadenopathy was not registered. The blood count revealed only elevated leukocytes and on examination there were no immature blood cells in the peripheral blood. The cytology from the ascites and pleural effusion did not detect any malignant cells. Due to the rapid disease progression the patient died after twenty-two days of admission. The diagnosis was discovered postmortem with the histological examination and immunohistochemical study of the material taken during the surgical laparoscopy performed four days before the lethal outcome. Although cytology is diagnostic in most cases, laparoscopy with peritoneal biopsy is the only procedure which can establish the definitive diagnosis of peritoneal lymphomatosis.

Choledochal cysts: diagnosis and treatment.

The aim of this study is to show the different diagnostic procedures and treatment in patients diagnosed with congenital choledochal cysts. Choledochal cysts are congenital anomalies of the bile ducts and include cystic dilatation of the extrahepatic and intrahepatic biliary ducts or both. The study shows ten patients diagnosed as having choledochal cysts. Diagnosis was established by clinical and radiographic findings including: ultrasound (US), magnetic resonance cholangiopancreatograpy (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC) and cytological examination of the bile juice. In the study choledochal cysts were classified according to the Todani classification. Most common cysts were type I (six cases); type III (one case), type IVa (one case) and two patients were type V cysts (Caroli disease). The most frequent symptoms were abdominal pain, jaundice and cholangitis. US findings were sensitive for the preliminary diagnosis of choledochal cysts in all the patients. MRCP accurately defined the cyst anatomy and the site of the biliary origin in all the cases with extrahepatic cysts. In three cases ERCP clearly demonstrated the cyst and by PTC smaller cysts were well defined. Cytological examination of the bile juice obtained during the PTC procedure showed malignant cells in one case. Therefore pancreaticoduodenectomy was performed and pathological examination showed associated cholangiocarcinoma. Five years after the operation the patient was well and free of the disease. Five patients underwent surgical treatment with a total cyst excision and Roux-en-Y hepaticojejunostomy while the surgical approach in two patients was partial cyst excision and cystojejunostomy. Patients with Caroli disease were conservatively treated and 3 with interventional endoscopic procedures. Despite US evidence suggesting choledochal cyst diagnosis, other supportive radiographic imaging modalities such as MRCP, ERCP and PTC are required to define the precise cyst anatomy and are essential for the preoperative assessment. Total cyst excision is recommended for reducing cyst-related complications and risk of cholangiocarcinoma.

Iron overload in patients with transfusion dependent myelodisplastic syndrome.

The myelodisplastic syndrome is a heterogeneous group of diseases, characterised by ineffective and dysplastic haematopoesis and pancytopenia in the peripheral blood, followed by progressive disturbance of differentiation of the haematopoetic stem cell, resulting in evolution of the disease towards acute leukaemia. According to the latest WHO classification, the term myelodisplastic syndrome includes diseases with an indolent course, as well as diseases with a fast evolution towards acute leukaemia. Because of this diversity, haematologists base their therapeutic decisions on prognostic scoring systems which incorporate all the significant factors with an influence on survival in this group of patients with myelodisplastic syndrome. Bearing in mind that anaemia is the most frequent form of cytopenia in patients with myelodisplastic syndrome, it is common that at some point of the disease almost every patient with myelodisplastic syndrome is transfusion-dependent. Frequently applied transfusions secure the correction of anaemia in these patients, giving them a good quality of life, but at the same time endangering them with the potential threat of iron overload, when the physiological mechanisms of iron excretion from the organism become insufficient. There is a clear correlation between transfusion dependence and the overall survival in patients with myelodisplastic syndrome. Chelators secure the lowering of the iron surfeit and are indicated in transfusion-dependant patients with myelodisplastic syndrome ( need for two blood units monthly, during one year ), when the ferritin level increases over 1000, in patients who are candidates for transplantation as well as in patients from good prognostic groups with median survival over one year. The therapy with chelators lasts as long as the patient is transfusion-dependant.