[The results of patients with essentials thrombocythemia and other myeloproliferation-related thrombocythemia--a report of patients treated with Thromboreductin]. / Výsledky lécby nemocných s esentiální trombocytemií a dalsími myeloproliferacemi provázenými trombocytemií--zpráva z registru pacientu lécených Thromboreductinem.

The registry of patients treated with Thromboreductin (anagrelide) in the Czech Republic contains data concerning patients that have been treated using this drug since 2004. As of June 2009, the total number of patients was 549. The current analysis focused mainly on evaluation of anagrelide dosage needed to achieve a complete response in high-risk patients: reduction in platelet count to below 400 x 10(9)/l, which was also considered as reaching the therapeutic goal. The outcomes of the registry confirm that although anagrelide (Thromboreductin) is a very effective platelet-reducing agent, the administration of which is related to a low incidence of adverse effects and complications, the therapeutic goal is not achieved in all cases and or despite a quick treatment response, the therapeutic goal is achieved more slowly.

Changes of blood count, lymphocyte subpopulations and immunoglobulin levels in nephroblastoma long term survivors.

The aim of this study was to investigate the frequency of blood count, lymphocyte subpopulations, and immunoglobulin levels alterations in a group of healthy nephroblastoma long-term survivors. The group included 122 nephroblastoma longterm survivors who were at least five years post anticancer therapy and free of any sign of recurrence The proportion of lymphocyte subpopulations was analyzed by flow cytometry using antibodies anti CD45 FITC/CD14 PE, anti CD3 FITC/ CD16+CD56 PE, anti CD4 FITC/ CD8 PE and anti CD20 FITC. Immunoglobulin G, A, and M levels were evaluated by immunoturbidimetry. Total blood count was also examined. The occurrence of decreased immunoglobulin levels, leukocytes, lymphocytes, and granulocytes count, proportion of T lymphocytes and their CD4+ subpopulation are not frequent. The most frequently decreased lymphocyte subpopulation was CD8 (15.5%). The most frequent abnormal findings were increased proportion of NK cells (38.5 %), B lymphocytes (38,52 %), decreased number of erythrocytes (25.2 %), hemoglobin levels (41.7 %) and hematocrit (13.9 %). The only significant differences between results of immunological examination and course of the disease were more frequently decreased proportion of CD4+ lymphocytes in recurrent disease survivors and lower IgA levels in survivors after radiotherapy. We found decreased at least one immunological parameter in one fifth of the survivors. The most frequently altered parameter was hemoglobin, which was decreased in 41.7 % of survivors. Decraesed hemoglobin may worsen quality of survivors life. Key words: nephroblastoma long-term survivors, blood count, lymphocyte subpopulations, immunoglobulin G, A, M serum levels.

[What is the current treatment of patients with essential thrombocytopenia and other myeloproliferations accompanied with thrombocythemia [corrected] and what can be the predictive sign of the risk of thrombosis in such patients--a report from the registry of patients treated by Thromboreductine]. / Jak lécíme nemocné s esenciální trombocytemií a dalsími myeloproliferacemi provázenými trombocytemií a co muze být prediktivní známkou rizika trombózy u techto nemocných--zpráva z registru pacientu lécených Thromboreductinem.

The registry of patients treated with Thromboreductine (anagrelid) in the contributing centres in the Czech Republic has been updated with data on the patients receiving this medication since 2004. The original purpose of the registry was to record responses to Thromboreductine therapy and adverse drug reactions in patients with essential thrombocytopenia. However, data on additional Ph negative myeloproliferations, as well as data on cytoreductive therapies other than exclusively that using Thromboreductine has also been recorded in the course of its compilation, including data on combined regimes. At present, the database contains data on 421 patients, and valid conclusions can be drawn if the level of data filling is enhanced. Evaluation has been currently focused on the analysis of the risk of development of clinical symptoms of thrombosis and on the standards of treatment from the viewpoint of the achieved treatment response. Analyses of data from the registry corroborate the special importance of the proof of JAK2 mutation, and of the test for factor V Leiden mutation, and of protein of S for the assessment of the risk of thromboembolic complications. The output of the analysis confirms that anagrelid is a very efficient thromboreductive agent the administration of which is associated with a low incidence of non-serious adverse effects (10.9%). However, in spite of a fast response to therapy, the therapeutic goal consisting in the reduction of the platelet count below 400 (or below 600) x 10(9)/l, i.e. the complete (or partial) treatment response, is relatively slow to achieve. This is likely to be due to lack of radical corrections in the dosage of the drug for different reasons.

[Essential thrombocytemia and other myeloproliferations with thrombocytemia in the data of the register of patients treated with Thromboreductin till the end of 2006]. / Esenciálni trombocytemie a dalsí myeloproliferace s trombocytemií v údajích registru pacientu lécených Thromboreductinem do konce roku 2006.

Since 2005, registers of patients treated with Thromboreductin (anagrelid) kept by some centres in the Czech Republic have been supplied with data concerning patients whose treatment with this preparation started in 2004. The purpose of the register is to record responses to therapy by Thromboreductin and adverse events in patients with essential thrombocytemia and other myeloproliferations, and to subsequently analyse the data. Another objective is to detect predisposition to clinical symptomatology and disease complications. Apart from thrombocyte count, additional risk factors are monitored. The database currently contains data for 336 patients. Initial analyses of data from the register point to the fact that anagrelid is a highly effective thromboreductive agent the administration of which is associated with relatively low incidence of adverse events (11.8 %) of mild and usually transitory nature. The therapeutic objective is attained at a relatively slow rate (according to overall stratification under 400 or under 600 x 10(9)/l thrombocytes), which is probably due to insufficient dose adjustment.

[The contribution of the hematological examination in patients with retinal vein occlusion]. / Prínos hematologického vysetrení u pacientu s venózní retinální okluzí.

OBJECTIVE: The aim of this study is to evaluate outcomes of hematological examinations in patients under 60 years of age affected by central (CRVO) or branch (BRVO) retinal vein occlusion. Furthermore, it also proves possible existence of risk factors resulting in early atherosclerosis and hematological disease. PATIENTS AND METHODS: Eleven patients (5 women and 6 men) of 38 years of age in average (15-56 years) were examined. Eight patients suffered from CRVO, 3 patients from BRVO. All patients were tested for hematological diseases. We focused on the following hematological parameters: presence of paraprotein, homocysteine blood level, lipoprotein A, presence of antiphospholipid antibody, level of activated protein C resistance, protein S, protein C, antithrombin III. The average follow up period was 19 months. RESULTS: hematological screening was positive in 5 patients, 4 patients out of them suffered from CRVO. High homocysteine blood level was detected in 2 patients and more over, an increased level of antiphospholipid antibodies was diagnosed in 1 patient. An increased level of lipoprotein A was determined in 2 remaining patients. BRVO was diagnosed in one case. Outcome of the hematological examination proved the pathological level of activated protein C resistance and lower level of protein S. CONCLUSION: according to these results we assume, the co-existence of more risk factors for hematological disease increase the probability of CRVO.

[Anagrelide in the treatment of essential thrombocythemia (ET) and other myeloproliferative disorders with thrombocythemia based on data from patient register in the CR]. / Anagrelid v lécbe esenciální trombocytemie a dalsích myeloproliferací s trombocytemií sledovaných v registru pacientu v CR.

Anagrelide hydrochloride is an effective drug used in patients with ET and other myeloproliferative disorders with thrombocythemia to selectively decrease the number of thrombocytes. Indications for use of anagrelide were described in detail in Czech medical literature. Since 2005 data concerning treatment with anagrelide in some medical clinics have been collected in patient register showing course of treatment from 2004, when the medicament obtained marketing authorization from State Institute for Drug Control to be used in the treatment of thrombocythemia in myeloproliferative disorders. Aim of patient register is to monitor medical effect of anagrelide therapy and incidence of adverse effects in patients with ET and other myeloproliferative disorders and subsequent analysis of collected data. At the moment patient register contains data from 154 patients.

[Retinopathy as a complication of Waldenstrom's disease]. / M. Waldenström--retinopatie jako komplikace onemocnení.

The authors wish to demonstrate on the case of a 52-year-old female patient who suffered from non-specific symptoms and impaired visual functions, typical changes on the fundus which may be due to some serious haematological diseases. In the case of the mentioned patient the haematological disease (Waldenström's disease) was manifested by hyperviscous and anaemic syndrome. The ophthalmological finding in the general picture was not dominant but on the whole typical and contributed to a more rapid diagnosis of the fundamental disease. Changes on the fundus by which the hyperviscous syndrome is manifested must be known and in the differential diagnosis the possibility of haematological disease should be taken into account as in the majority patients are involved who are at risk of sudden loss of eyesight and sudden death.

[Serious surgical complications associated with chronic anticoagulant therapy]. / Závazné chirurgické komplikace chronické antikoagulacní lécby.

Chronic anticoagulant treatment is administered mostly for cardiological reasons. Cumarin derivatires are used in the majority of cases (Warfarin, Pelentan). It is necessary to monitor this treatment regularly and to control the dose according to the INR value. Different complications can occur; the haemorrhage represents a serious one. The authors discuss several aspects of anticoagulant therapy and possible prevention of the complications. The importance of the problems is demonstrated on the authors' clinical experience--two cases of haemorrhage after Warfarin administration simulating an acute surgical event.

[Perioperative hypothermia and its sequelae]. / Perioperacní hypotermie a její dusledky.

UNLABELLED: Perioperative hypothermia is associated with the development of haemocoagulation, cardiovascular and metabolic disorders leading to an increased morbidity and mortality. The objective of the investigation was to assess the extent of hypothermia and its clinical and laboratory consequences. A group of 30 patients subjected to elective radical laparotomy on account of colorectal carcinoma was divided into to equivalent groups. To the first group heated infusions were administered, to the second group not heated ones. In all patients the central and peripheral temperature, rate of postoperative normalization of the temperature, postoperative thermal comfort, consumption of analgesics and biochemical and haematological parameters were monitored. RESULTS: In patients with non-heated infusions more marked and longer perioperative hypothermia was recorded with a significant alteration of the number of leucocytes and thrombocytes. In the other investigated indicators there was no significant difference between the two groups. Hypothermia did not cause serious complications in any of the patients. CONCLUSION: Although no serious clinical complications induced by hypothermia were recorded, the authors recommend an active approach and provisions for the perioperative maintenance of body temperature as a standard of contemporary perioperative care.

[Secondary hemophagocytic syndrome in a systemic disease]. / Sekundární hemofagocytující syndrom pri systémovém onemocnení.

20 year old man 2 years treated for the seropositive rheumatoid arthritis was admitted for fever accompanied with jaundice, anemia and leukopenia. The underlying disease has been compensated already for long period of time, before his admission only Prednisone (in the dose of 5 mg daily) and Methotrexate (15 mg once a week) was given. His physical examination of admission was without any significant abnormalities, out of the routine laboratory examination the value of leukocytes count was 2.1 x 10(9)/L, erythrocytes 3.7 x 10(12)/L, hemoglobin 95 g/l, hematocrit 0.29, platelets 156 x 10(9)/L. Since admission to hospital the hepatic enzymes ALT, AST, GMT, ALP were about ten times elevated comparing to normal values, the coagulation examination has shown the decrease of Quick test to 55%. With respect to the permanent leukopenia the bone marrow aspiration was taken with the finding of the increase number the RES elements (18.4%) with the signs of hemophagocytosis. The phagocytic reticulum absorbs blood elements erythrocytes, normoblasts, granulocytes, platelets. According to the literature experience we started the combination of the immunosuppressive treatment consisting of corticosteroids and Cyclosporine. Already the day following the application of the high dose of corticosteroids the fever subsided, icterus went away gradually with the normalization of the liver tests. After 20 days of hospitalisation the patient was discharged in good shape. Now, after 4 months the is stabilized on the follow-up treatment of Prednisone a Cyclosporine.

Cyclosporin A therapy in hypoplastic MDS patients and certain refractory anaemias without hypoplastic bone marrow.

We report 17 cytopenic patients with myelodysplastic syndrome (MDS) of refractory anaemia (RA) subtype with hyper-, normo- or hypo-cellular bone marrow (BM), who were treated with cyclosporin A (CyA). Substantial haematological response was observed in 14 patients (82%): their anaemia improved and all transfusion-dependent patients achieved transfusion independence. Complete trilineage recovery was observed in four patients (23%). The CyA therapy has not yet failed in any of the 14 successfully treated patients during follow-up times ranging from 5 to 30 months. CyA was well tolerated in 14 patients; serious side-effects required termination of the therapy in three patients in whom the blood count rapidly deteriorated to former levels upon cessation of therapy. Two patients benefited from a combination therapy of CyA and erythropoietin. Six patients experienced various autoimmune phenomena. CyA could thus offer an alternative treatment for certain MDS patients with RA regardless of hyper-, normo- or hypo-cellularity of bone marrow (BM). The mechanism of the beneficial effect of CyA is discussed and remains the subject of an ongoing study.

[Portal vein thrombosis after splenectomy]. / Trombóza portální vény po splenektomii.

Elective splenectomies in haematologic malignancies are associated with two serious risks: infection and thrombotic complications. The case of a patient operated on for chronic lymphatic leukaemia with subsequent postoperative portal vein thrombosis is described. The complication was detected using abdominal ultrasonography and was treated successfully with anticoagulants (Fraxiparin).

[Can the system for ordering blood for elective surgery be improved?]. / Lze neco zlepsit na systému objednávání krve k elektivním operacím?

The authors investigated the effectiveness of ordering of blood for elective surgery. Analysis of a group of 333 operated patients revealed that blood was administered only in 9% of the operations, i.e when compared with the number ordered the ratio ordered: issued was 13:1. Based on their own experience the authors present the possibility how to improve the blood ordering system from the surgical as well as blood bank activity aspect.