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We present two cases with a history of acquired haemophilia with massive haemorrhage in the course of the disease. A 74-year-old man presented to the emergency room with an extensive, progressive swelling and haemorrhage in the region of left knee joint, both upper legs and forearms. Laboratory studies revealed the presence of the factor VIII inhibitor in the titer of 115 Bethesda U/mL, low level of factor VIII activity (19.2%) and severe anemia (Hb - 7.0 g%). The patient was treated with FFP transfusions and prednisone with cyclophosphamide to eradicate factor VIII inhibitor. The remission was achieved and lasts for a two years. A 52-year-old woman presented to the emergency room with an extensive subcutaneous haemorrhage in the region of right knee joint and right lower leg. Laboratory studies revealed the presence of the factor VIII inhibitor in the titer of 30 Bethesda U/mL. The factor VIII activity level was only 4%. The patient did not receive the FFP because the severity of the haemorrhage was low. She was treated with steroids. The factor VIII inhibitor disappeared after 2.5 months of therapy.

The aim of this article is to present a case report of Gaucher disease which was diagnosed in patient with hepatosplenomegaly. A 43 years old man has complained of weakness, stomach pain and yellowish skin color for several years. The severity of symptoms has increased during the last 2 years. Laboratory data revealed thrombocytopenia (platelet count 108 G/l) and slightly elevated bilirubin level (1.68 mg/dl). In ultrasound examination hepatosplenomegaly was observed. Computer tomography evaluated the size of the spleen to 20 x 12 cm. Gaucher cells were found in bone marrow. The some type of cells was recognized in liver biopsy. To confirm diagnosis of Gaucher disease enzyme test was performed. Chitotriosidase level in serum was pronouncedly increased (11,540 nmol/mg protein/hr) while normal level is under 150 nmol/mg protein/hr. Glucocerebrosidase activity in leucocytes was within the limit. Thus the enzyme activity was 21.8 nmol/mg protein/hr and was below the normal range which is between 111-455 nmol/mg protein/hr. The last assay, measuring glucolukocerebrosidase activity in cultured fibroblast confirmed the diagnosis of Gaucher disease.

In 16 patients on chronic haemodialysis treatment the platelet activation and function were studied during 2 weeks antiplatelet therapy with indobufen. Followings tests were made: platelet count, platelet aggregation, platelet factor 3 and 4. Indobufen inhibited platelet function, mainly release of platelet factor 4 and improved dialyser regeneration.

18 patients were studied with primary hypercholesterolemia (type II according to Fredricksen) and fasting cholesterol level during low fat diet above 250 mg/dl. Blood samples were taken: I--before lovastatin administration on diet, II--after 4 weeks administration of 20 mg, III--after 4 weeks of 80 mg and IV--4 weeks after the drug discontinued. The following tests were performed: platelets aggregation induced by ADP and adrenaline, PF3 and PF4, platelet MDA, serum triglycerides, total cholesterol, HDL-cholesterol and LDL-cholesterol. Before treatment patients showed significant platelets hyperaggregation and MDA concentration comparing to the control group. During the drug administration significant lowering of platelets MDA concentration, was observed small but significant HDL cholesterol level increase which correlated with platelet MDA concentration. Availability of PF3 and release of PF4 did not change during study.

In 28 patients with plasmocytoma selected parameters of primary haemostasis were studied before treatment and 6-9 months later, in remission. We evaluated spontaneous aggregation, aggregation induced by ADP and adrenaline, platelet factor 3 availability and the realising platelet factor 4. Significant disturbances of primary haemostasis were particularly marked in patients with a high monoclonal protein concentration (greater than 3 g/dl). During remission an increase of platelet aggregation was noted. Its values, however, remained lower than in the control group.

In 18 patients with type I diabetes the effect was studied of 10 days of Indobufen administration in daily doses of 400 mg on the activation and function of platelets. Spontaneous and ADP and adrenaline-induced aggregation, adhesiveness, platelet factors 3 and 4, and generation of malonyldialdehyde (MDA) in platelets were investigated. The results were compared with similar results in the same group prior to the treatment and in a control group of 19 healthy subjects. Highly significant inhibition was noted of spontaneous inhibition and that evoked by adrenaline and ADP, and a considerable reduction of MDA generation by platelets. These results indicate that the new platelet inhibitor--Indobufen, effectively inhibiting platelet activation and function may prevent development of thrombotic complications of diabetes.