Clinical manifestations of subleukemic myelosis, which debuted with Budd-Chiari syndrome in a young female patient, are presented. The cytoreductive therapy should be spent simultaneously with anticoagulant therapy.

AIM:To assess incidence of hyperhomocysteinemia (HHC) in patients with chronic myeloproliferative diseases (CMPD) and to analyse possible correlation between an elevated concentration of plasma homocystein (HC) and thrombotic complications.MATERIAL AND METHODS:The trial enrolled 61 patients: 39 CMPD patients with thrombotic complications and free of them, 22 nonhematological patients with thrombosis. The control group consisted of 40 healthy donors. The examination protocol included determination with standard methods of HC plasma concentration, platelet and plasma components of hemostasis, mutation of factor V Leiden gene, prothrombin and methylenetetrahydrofolate reductase (MTHFR).RESULTS:Mean HC concentration in the serum in CMPD patients was 19 +/- 1.7 mcmol/l which appeared higher than in healthy donors (12 +/- 1.3 mcmol/l). The highest HC was in patients with subleukemic myelosis (SLM)--23 +/- 2.3 mcmol). No difference in HC concentration in plasma was observed in CMPD carriers of homo- or heteroxygous mutation of C667T gene or CMPD patients without the mutation. In CMPD content of factor VIII was higher in HHC than in normal HC (222 +/- 26.5 and 116 +/- 20%, respectively, p = 0.002). For von Willebrand factor 202 +/- 15.6 and 120 +/- 14.6%, respectively (p < 0.003). HC reduction in response to vitamin therapy was the greater the higher its initial level was.CONCLUSION:There is correlation between HHC and thrombosis in CMPD patients. HC concentration may depend on the proliferative stage of CMPD. As HC is a significant independent factor of thrombotic complications risk, it is necessary to detect and treat HHC.

AIM:To analyse the course of pregnancy in chronic myeloproliferative diseases (CMPD) with hyperthrombocytosis, primarily, essential thrombocytemia.MATERIAL AND METHODS:The analysis of thrombogenic risk factors covered literature data and 8 cases observed by the authors.RESULTS:Six pregnant women received long-term treatment with preparations of interferon-alpha in a dose 9-20 million IU a week (both before and during pregnancy). Rapid reduction of hyperthrombocytosis (1100-4000 x 10(9) l) and the absence of a negative effect on development of the fetus were seen in all the cases. Normal delivery on week 37-39 was in 4 patients, spontaneous abortion on week 24 was provoked by a car accident. Three gravidas (gestational week 28, 33 and 34) are still under observation. Lupus anticoagulant or elevation of anticardiolipin antibodies level was detected in 4 of 8 patients, 2 patients had heterozygous mutation of methylentetrahydrofolatereductase genes and factor V (Leiden). These patients were given lannacher, faxiparine, folic acid and discrete plasmapheresis (in 2 cases).CONCLUSION:Gravidas with hyperthrombocytosis, if not contraindicated, must be treated with aspirin and interferon-alpha preparations at any gestational term. Moreover, it is necessary to exclude additional most prevalent causes of thrombophilia for adequate prevention of thromboses.

Aim To ascertain risk factors of thromboembolism of the pulmonary artery (TPA) in Willebrand's disease (WD). Material and methods We made a retrospective analysis of hospitalizations of WD patients for 10 years. We analysed causes of the patients' admission, interventions, registered maximal levels of factor VIII (FVIII) and Willebrand's factor (FW) in which the interventions were made, cases of TPA. Results Thirty four patients with WD were hospitalized 45 times. Three patients were treated conservatively because of gastrointestinal bleeding, the rest patients received surgical therapy. All the patients were given FVIII concentrates, cryoprecipitate, fresh-frozen plasma. In the course of the treatment, FW and FVIII levels were determined in 38% cases, FW--in 23%, FVIII--in 27%, coagulation was studied in 12% without test for FVIII and FW levels. Maximal concentration of FW was 72.1 +/- 11.8%, FVIII--125 +/- 15.8%. TPA developed in 2 (4.4%) of 45 patients. In both cases we observed a marked rise of plasmic concentration of FVIII due to therapy (250 and 240%). Conclusion In patients with WD thromboembolic complications risk factors are age, obesity, surgical interventions, immobilization, etc. Simultaneous administration of several drugs containing FW and FVIII was also among the risk factors. Overdosage of FVIII is one of the causes of thrombotic complications in WD. FW and FVIII correlations in FVIII preparations must be considered. Prophylactic heparin therapy is recommended in patients with a high risk of thrombotic complications upon achievement of normal hemostasis.

AIM:To specify indications to plasmapheresis (PA) and to assess its efficiency in patients with hypercoagulatory syndrome in hematogenic thrombophilia (HT).MATERIAL AND METHODS:18 patients (11 males, 7 females, age 26-50 years) with various forms of HT received standard antiaggregation, anticoagulatory therapy plus therapeutic PA. By PA technique the patients were divided into two groups.RESULTS:A positive trend in clinical, instrumental and laboratory indices was observed in all the patients.CONCLUSION:Therapeutic PA in hypercoagulation syndrome in HT patients leads to fast normalization of the clinical picture and reestablishment of patency of thrombus-affected vessels.