[Autoimmune thrombocitopenic purpura in pregnancy].

The author deals with haematologists' and obstetricians' current views on acquired ATP in children and adults, characterised by a transient, acute or chronic decrease in platelets count (<50.109/l) due to premature destruction by the reticuloendothelial system. The most common questions arising in connection with this disease are: what is autoimmune thrombocytopenic purpura; is there any correlation between pregnancy and ATP; what are its symptoms; does pregnancy itself affect the autoimmune disease. If is of utter importance for women with ATP to be aware of the risks these symptoms pose both on the health of the mother and the foetus. Obstetricians and gynaecologists seldom object to pregnancy in women with ATP. Nevertheless, it is essential to point out that additional monitoring and therapy are needed. There is no medical evidence that supports the notion of terminating pregnancy due to ATP. Assessment is made only by an obstetrician, haematologist and pediatrician working in close collaboration. This collaborative work must be present throughout the whole pregnancy, delivery and puerperium. The treatment necessary for women with ATP aims to establish platelet count over 50.000 ppm when approaching the end of pregnancy, preferably between 80.000 ppm - 100.000 ppm taking into account vagina or surgical delivery as well as the administration of anaesthetic. Delivery management must be decided entirely on obstetrics consideration, but not on ATP ones. Pregnant women with ATP must be monitored and treated with caution by a highly specialised medical team.

[Therapeutic approach in pregnant women with an autoimmune thrombocytopenic purpura].

The case presented herein aim to update the existing information about the common diagnostic problems and therapeutic approach in pregnant women that have autoimmune thrombocytopenic purpura (ATP).

[A case of pseudotumor in a patient with hemophilia A]. / Sluchai s psevdotumor u bolen s khemofiliia A.

A case of a patient with proved hemophilia A and a pseudotumor of the iliac bone is presented. The diagnostic importance of sonography and computed tomography is pointed out. Obligatory control and follow-up by physical, ultrasound and computed tomographic examinations of hemophilic patients with muscular hematomas and subperiosteal hemorrhages are recommended for the prophylaxis of pseudotumors. Their persistence in spite of proper treatment with coagulative bioproducts should be considered as an indication for surgical treatment in order to prevent the development of hemophilic pseudotumors.

[Thrombocyte aggregation and the secretion reaction in hemophilia patients]. / Prouchvane na trombotsitnata agregatsiia i reaktsiiata na sekretsiia pri bolni s khemofilii.

The action of platelet aggregation and release reaction on the tendency toward bleeding in patients with hemophilia was studied. Were examined 43 patients with hemophilia A, 14 of them with hemorrhages, 5 patients with hemophilia B, 5 patients with hemophilia A and factor VIII inhibitor and 2 patients with congenital factor VII deficit. Disturbed aggregation with ADP was found in 2 patients and with adrenalin also in 2 patients with hemophilia A without hemorrhages, in 1 patient with hemophilia A with hemorrhages and in 1 patient with hemophilia B. In the remaining patients the examined indices were normal. No data for spontaneous aggregation were found. The results of the study indicate that no connection was established between the tendency toward bleeding in the hemophiliac patients and the platelet aggregation and release reaction. No data for platelet hyperactivation by the hemorrhages were found.

[Diagnostic problems in disorders of thrombocyte functions]. / Diagnostichni problemi pri narusheniiata na trombotsitnite funktsii.

Numerous specific methods have been introduced lately in the clinical practice allowing the determination of the exact defect in platelet functions of the patient. Those methods are divided in five groups: global methods in vivo and in vitro, methods reflecting the platelet adhesiveness, platelet aggregation, platelet secretion, platelet participation in coagulation. The author is one of the first that introduced the study on platelet aggregation and secretion by aggregometer as well as the study of factor VIII antigen and von Willebrand factor. The author, based on his experience, proposes a diagnostic constellation of tests for establishing the nature of platelet defect.

[Thrombocyte functions in malignant blood diseases]. / Prouchvane na trombotsitnite funktsii pri niakoi zlokachestveni kruvni zaboliavaniia.

Platelet aggregation with various inductors and platelet secretion were studied in chronic lymphoid leukemia, myeloma, chronic myeloid leukemia and acute leukemia. It was established that disturbed aggregation with adrenaline and disturbed secretion are found more often in these malignant blood diseases. An interesting fact is that there is no correlation between the disturbed platelet functions in vitro and the clinical manifestations of disturbed hemostasis (hemorrhages or thrombosis).

[Changes in the factor VIII molecular complex in Hodgkin's disease and non-Hodgkin's lymphoma]. / Promeni vuv faktor VIII molekulniia kompleks pri bolestta na khodzhkin i nekhodzhkinovi limfomi.

Factor VIII molecular complex is included in the proteins of "acute inflammation", being changed not only in the congenital diseases - hemophilia A and Willebrand disease but also in a series of acquired diseases as various inflammatory processes, hypercoagulability, DIC syndrome, neoplasms. The studies carried out on Hodgkin's disease and non-Hodgkin lymphomas reveal that the changes in its separate activities (factor VIIIK and Willebrand antigen) are not unidirectional with the changes of fibrinogen level and cannot serve as an index of the activity of the process.

[Patient with factor V deficiency (parahemophilia) diagnosed and treated as hemophilia A]. / Bolen s defitsit na faktor V (parakhemofiliia), diagnostitsiran i lekuvan kato khemofiliia A.

The authors observed the patient T.P.D., aged 36, admitted to the clinic of RIHBT with melena and hematemesis. Since early childhood the patient has been treated on the occasions of hemorrhagic incidences for hemophilia A. The hemostatic investigations however, revealed normal level of factor VIII and the level of factor V--1%, providing grounds to revise the initial diagnosis and to admit that the rarely found parahemophilia is concerned. The treatment with freshly-frozen plasma, fresh blood, velin and iron preparations led to coping with hemorrhagic syndrome and posthemorrhagic anemia. The diagnostic possibilities are interpreted for establishing the deficiency of the factors of coagulation and of the specific substitution therapy.

[Thrombotic thrombocytopenic purpura (Moschkowitz's disease)--a review of the literature with a case report]. / Trombotichna trombotsitopenichna purpura (bolest na Moshkovits)--pregled na literaturata s opisanie na edin sluchai.

A case with thrombotic thrombocytopenic purpura (TTP) in a young female is described. The clinical picture is described as well as the pathologoanatomical finding, the characteristic and pathognomonic sins of the disease being mentioned. A survey of the literature is made, covering data about the thorough characteristic of the disease--clinical-laboratory, pathomorphological picture, etiological and pathogenetic aspects. Attention is paid to the laboratory discrepancies with the syndrome of disseminated intravascular coagulation, included in the differential diagnosis when making the clinical diagnosis of TTP. The success in the treatment of TTP during the last several years is reported with the introduction of combined therapy with corticosteroids, splenectomy, antiaggregating thrombocyte agents, exsanguination transfusion and, with the most reliable effect for the present--plasmapheresis and plasmainfusions.