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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.

This article summarizes the clinical, radiological and laboratory features of syndrome, which is known by the acronym "POEMS". POEMS syndrome is a rare multisystemic syndrome with plasma cell dyscrasia. POEMS is characterized by the combination of polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. Other signs are frequently observed in affected patients, such as peripheral edema, arteriopathy, nephropathy, thrombocytosis and osteosclerotic lesions. The plasma cell dyscrasia in POEMS syndrome differs from the dyscrasia found in multiple myeloma. It has been suggested that pleiotropic cytokines which act in synergy on immune, nervous, endocrine and vascular systems could play a pathogenic role in POEMS syndrome.