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Submitted: 27. 2. 2016.

BACKGROUND: Idiopathic portal hypertension (IPH) is a disease of unknown etiology which manifests itself in hypercirculation and hypertension in the portal venous system and splenomegaly with hypersplenism. METHODS: We describe a case of IPH in a young sportsman with the characteristic clinical, sonographic, and computed-tomography picture. Open splenectomy was performed. RESULTS: Preoperative examination revealed an interesting elevation of endothelin-1 (ET-1) and vasoadhesive molecule-1 (VCAM-1) in the serum. Postoperative course was without complications. One year after operation the patient is fine without any symptomatology of IPH. CONCLUSIONS: ET-1 and VCAM-1 will probably have a significant influence on the development of IPH. ET-1 causes hypercirculation and hypertension in the portal venous system, and VCAM-1 causes adhesion of lymphocytes to the endothelium of the portal tracts and their migration into the wall, mainly in a presinusoidal way, followed by fibrotization of the venous wall. Splenectomy was performed in this case, after which the clinical symptomatology of IPH disappeared.

Multiple myeloma is the second most prevalent and mostly fatal hematologic cancer. Further advances have been made in understanding the mechanisms involved in the myeloma pathogenesis and elucidation of critical signalling pathways as therapeutical targets. Proteasome inhibitors are the example of this new approach and bortezomib is the first agent in this class to enter clinical trials.In 6 hematological centers in Czech Republic 29 patients with refractory/relapsed myeloma had been treated with bortezomib (Velcade, Millennium Pharmaceuticals) in 2004. The initial dose 1.3 mg/m2 of Velcade was given, in 1 case the dose was adjusted due to pre-existing renal failure to 1 mg/m2. The response was achieved in 17 patients (59%). Four patients had complete, 11 partial and two minor responses. In 5 cases stabilization of disease was observed and 6 patients progressed during the therapy.Unfortunately, one patient died immediately after the start of therapy due to sepsis. The most common adverse events were thrombocytopenia, anaemia, neuropathy, gastrointestinal complication, renal failure and fatigue. Grade 4 adverse events occurred in 37.9% of patients (4x thrombocytopenia, 2x gastrointestinal, 2x renal failure, 1x sepsis, leucopenia, hepatopathy and anaemia, respectively). Peripheral neuropathic pain of grade 3 was reported in 4 cases, in one patient therapy had to be interrupted due to this complication. We confirmed promising results of phase II trials.