[Combination of heterozygote blood coagulation factor V Leiden carriage with erythrocytosis as a cause of the hip deep vein thrombosis]. / Sochetanie geterozigotnogo nositel'stva leidenskoi mutatsii faktora svertyvaniia krovi s éritrotsitozom kak prichina tromboza glubokikh ven bedra.

AIM: To reject one of the variants of inherited thrombophylia in a 64-year-old patient with deep thrombosis of leg veins and high hemoglobin and red cell levels. MATERIAL AND METHODS: The study was made of antithrombin III and protein C, protein S levels; resistance to activated protein C; molecular structure of DNA coding factor 5; methylenetetrahydrofolate reductase. RESULTS: The patient was diagnosed to have heterozygote factor V Leiden mutation. The replacement of arginine by glutamine in position 506 of factor V molecule raises the risk of thrombosis. This risk was aggravated by high hemoglobin, red cells, hematocrit, low volume of circulating plasma, smoking. The patient had normal levels of leukocytes and platelets, normal spleen size, slightly lowered level of erythropoietin. CONCLUSION: The presence of thrombosis in patients with erythremia or erythrocytosis rejects one of the thrombophilia forms.

Mechanisms of thrombocytopenia in patients with lymphoproliferative diseases.

Different factors are involved in the development of thrombocytopenia in patients with lymphoproliferative disorders. Significant correlation was detected between the number of megakaryocytes in bone marrow and platelet count (r = 0.485, p = 0.002, n = 37) and significant difference between the number of megakaryocyte in patients with normal platelet count (> 200,000/microliters) and patients with marked thrombocytopenia (platelet count < 100,000/microliters). All patients in the latter group (n = 15) had a relatively low number of megakaryocytes. Low but significant reverse correlation was found between the level of platelet-associated IgG (PA-IgG) and platelet count (r = -0.249, p = 0.024, n = 82) and significant difference between the mean levels of PA-IgG in the groups of patients with platelet count > 200,000/microliters and < 100,000/microliters. PA-IgG were increased in 46% of patients in the total group and in 65% of patients with platelet count < 100,000/microliters. The correlation between platelet count and PA-IgG was about 2 times higher in splenectomized (r = -0.549, p = 0.005, n = 24) than nonsplenectomized patients. All splenectomized patients with platelet count < 100,000/microliters (n = 8) had a significant increase in PA-IgG. Serum antibodies were detected in only 7% of tested patients. This group was characterized by severe thrombocytopenia (in 6 of 10 patients--platelet count < 50,000/microliters) and a high incidence of haemorrhages (in 5 of 10 patients). Thus the depression of platelet production was suggested to be the basic cause of thrombocytopenia in lymphoproliferative disorders. Involvement of immune mechanisms was revealed in a large number of patients and correlated with a deeper and more complicated thrombocytopenia.

Application of a high sensitivity aggregate-haemagglutination test for the diagnosis of autoimmune haemolytic anaemia with a negative direct antiglobulin test.

An aggregate-haemagglutination test has been used for determining antierythrocyte autoantibodies. The first antiglobulin variant of the test allows us to establish the presence of antibodies in 33 cases with a negative direct Coombs' test. The test II (antiantiglobulin variant) proved to be positive in 88 cases of auto-immune haemolytic anaemia (AIHA) that showed a negative direct Coombs' test and a negative antiglobulin variant. Immunoglobulin G has been revealed in the majority of AIHA patients. IgM has been determined in 1 case of a symptomatic form associated with chronic lymphocytic leukaemia. IgA has been recorded in 3 cases with an idiopathic AIHA form and in 6 cases of chronic lymphocytic leukaemia. Both types of light chains were found on the surface of erythrocytes in all cases of AIHA.