[Hereditary hemorrhagic thrombocytoasthenia].
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Adenosine Triphosphate↗
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Results for “HEMORRHAGIC THROMBOCYTHEMIA”
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[Possibilities of the prognosis of a hemorrhagic syndrome in patients with hepatitis B].
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Blood Platelets↗
What is the standard treatment in essential thrombocythemia.
The treatment of patients with essential thrombocythemia (ET) should be based primarily on the expected risk of major thrombotic complications. Although the specific values chosen for separating different risk categories are in part arbitrary, the following recomendations can be made. Young asymptomatic subjects with platelet counts below 1,500x10(9)/L are at lower risk and can be followed untreated. However, it should be emphasized that thrombotic events can also occur in a small percentage of these lower-risk cases. Low-dose aspirin (100-300 mg/day) should be given to patients with symptoms of microvascular occlusion, such as erythromelalgia or transient neurological attacks, and avoided in those with bleeding manifestations. The risk/benefit of low-dose aspirin in the primary prevention of thrombosis in asymptomatic patients remains uncertain. For high-risk patients (age >60 years, or platelet count >1,500x10(9)/L, or previous thrombosis), hydroxyurea, plus aspirin in the case of thrombosis, is the treatment of choice because its efficacy in preventing thrombotic complications has been proven in a randomized clinical trial. However, the possible long-term leukemogenicity of this drug, as well as that of other effective cytoreductive agents such as busulphan and pipobroman, remains a major concern. Anagrelide and interferon could overcome this worry but their efficacy has been hitherto demonstrated only in lowering the platelet count. Controlled clinical studies showing a benefit in preventing thrombotic and hemorrhagic complications are urgently needed.
Antineoplastic Agents↗
Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia.
BACKGROUND: We conducted a randomized comparison of hydroxyurea with anagrelide in the treatment of essential thrombocythemia. METHODS: A total of 809 patients with essential thrombocythemia who were at high risk for vascular events received low-dose aspirin plus either anagrelide or hydroxyurea. The composite primary end point was the actuarial risk of arterial thrombosis (myocardial infarction, unstable angina, cerebrovascular accident, transient ischemic attack, or peripheral arterial thrombosis), venous thrombosis (deep-vein thrombosis, splanchnic-vein thrombosis, or pulmonary embolism), serious hemorrhage, or death from thrombotic or hemorrhagic causes. RESULTS: After a median follow-up of 39 months, patients in the anagrelide group were significantly more likely than those in the hydroxyurea group to have reached the primary end point (odds ratio, 1.57; 95 percent confidence interval, 1.04 to 2.37; P=0.03). As compared with hydroxyurea plus aspirin, anagrelide plus aspirin was associated with increased rates of arterial thrombosis (P=0.004), serious hemorrhage (P=0.008), and transformation to myelofibrosis (P=0.01) but with a decreased rate of venous thromboembolism (P=0.006). Patients receiving anagrelide were more likely to withdraw from their assigned treatment (P<0.001). Equivalent long-term control of the platelet count was achieved in both groups. CONCLUSIONS: Hydroxyurea plus low-dose aspirin is superior to anagrelide plus low-dose aspirin for patients with essential thrombocythemia at high risk for vascular events.
Adult↗
Quantitative platelet disorders.
Thrombocytopenia may be caused by abnormal platelet production, accelerated removal owing to immunologic or nonimmunologic reasons, or sequestration of platelets in the spleen. Bleeding associated with thrombocytopenia usually presents as petechial or ecchymotic hemorrhages or epistaxis. Immunologic and nonimmunologic cases of thrombocytopenia may be diagnosed with routine hematology, bone marrow cytology, and platelet specific tests. Thrombocythemia may also be associated with platelet functional abnormalities, contrasting the normal platelet function noted in reactive thrombocytosis.
Animals↗
The pathogenesis and management of essential thrombocythaemia.
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Acute Disease↗
Long-term use of anagrelide in young patients with essential thrombocythemia.
Anagrelide is a novel platelet-lowering agent that has recently been approved for use in essential thrombocythemia (ET) and related disorders. Short-term drug efficacy and toxicity data have previously been presented. The purpose of this study was to obtain additional information regarding long-term anagrelide use. This is a retrospective series of 35 young patients (17 to 48 years) with ET who received anagrelide treatment before 1992. Initial drug dosage ranged between 1 and 10 mg/d, and the median maintenance dosage was 2.5 mg/d. The overall initial response rate of 94% included 74% complete remissions and 20% partial remissions. Of the 33 responding patients, 27 (82%) remained on anagrelide therapy for a median of 10.8 years (range, 7 to 15.5). Of these, 66% maintained a complete and 34% a partial remission over the study period. In general, the reporting of somatic side effects decreased over time, and anemia was the only new side effect that emerged after long-term therapy. Eight patients (24%) experienced a more than 3 g/dL decrease in hemoglobin level. Despite active therapy, 20% of the patients experienced a total of 10 thrombotic episodes, and a similar proportion experienced major hemorrhagic events. All thrombohemorrhagic complications occurred at a platelet count of more than 400 x 10(9)/L. It is concluded that long-term treatment of ET with anagrelide is associated with decreased reporting of initial side effects and the development of mild-to-moderate anemia. Complete normalization of platelet counts may be needed to minimize residual thrombohemorrhagic risk during therapy. (Blood. 2001;97:863-866)
Adolescent↗
[Prevention of vascular complications in polycythemia vera and primary thrombocythemia treated with low doses of acetylsalicylic acid].
22 patients (13 with polycythaemia vera and 9 with primary thrombocythemia) were treated with 250 mg acetylsalicylic acid (ASA) daily for an average of 25 months. Before therapy was started, 3 patients had arterial thromboses, 3 had venous thromboses, 3 had spontaneous hemorrhage, 3 had acral circulatory disorders and 13 had dizziness, whereas under ASA treatment neither arterial nor venous thromboses occurred and only 4 mild spontaneous hemorrhages were recorded. Under ASA the circulatory disorders of the extremities disappeared completely in 11 patients and recurred intermittently in milder form in 2 patients. Dizziness was completely abolished in 12 of the 13 patients. Discontinuation of therapy was followed by prompt recurrence of symptoms. No correlation could be established between symptoms and extent of platelet disease either before or during ASA therapy. Low-dose salicylates are highly effective in the prevention and treatment of vascular complications in polycythaemia vera and primary thrombocythemia. Thanks to ASA, potentially leukemogenic cytostatic agents and radiophosphorus can be used more sparingly.
Aged↗
[Diagnosis and treatment of essential thrombocythemia].
Essential thrombocythemia (ET) is one of the chronic myeloproliferative disorders (MPD), clonal disorders of hematopoietic stem cell. The differential diagnosis from the other MPD and reactive thrombocytosis was described. The treatment of this disease was then stated with emphasis on the importance of control of platelet count. For the control, drugs to suppress the megakaryocytic proliferation are used, including busulfan, hydroxyurea, interferon-alpha and Anagrelide. The therapy for asymptomatic patients and pregnant women as well as patients with acute hemorrhage and thrombosis was also discussed.
Adolescent↗
Abnormalities of von Willebrand factor in myeloproliferative disease: a relationship with bleeding diathesis.
We studied factor VIII related properties in 24 patients with increased platelet number. Twenty-one were affected by myeloproliferative disorders (eight had polycythaemia vera, 13 had essential thrombocythaemia) and three had secondary thrombocytosis. Normal levels of VIII:C and VIIIR:Ag were found while a significant (P less than 0.05) decrease of VIIIR:RCOF (43 +/- 13%) related to a lack of larger multimers of VWF (39 +/- 12%) was observed in 57% of patients with myeloproliferative disorders. A normal VWF pattern was found in the three patients with secondary thrombocytosis. The highest incidence of VWF abnormalities occurred in patients with essential thrombocythaemia (70%) in comparison with polycythaemic patients (38%). A significant (P less than 0.03) correlation between platelet count and the values of both VIIIR:RCOF and VWF multimeric pattern was observed only in patients with polycythaemia vera. The lowest levels of VIIIR:RCOF and the greatest loss of larger VWF multimers (less than 30%) were observed in two patients who presented bleeding symptoms at the time of study and a prolonged bleeding time. In addition, the relationship between VWF pattern and bleeding diathesis was supported by the fact that 75% of the patients with VWF abnormalities had bleeding history.
Adult↗
Vascular complications of essential thrombocythaemia: a link to cardiovascular risk factors.
Essential thrombocythaemia (ET) is a myeloproliferative disorder characterized by absolute thrombocytosis and increased incidence of thrombosis and haemorrhage. We report higher morbidity in patients with ET due to arterial ischaemic complications when cardiovascular risk factors are present. In this retrospective analysis of 46 patients, arterial complications occurred in 20/46 patients (43.4%); patients with cardiovascular risk factors, especially cigarette smoking, had more than twice as many arterial complications than patients without risk factors (62.5% v 22.7%, P < 0.05). Neither age, gender nor degree of thrombocytosis were linked to the number of complications. In contrast, we observed no association between cardiovascular risk factors and venous thrombosis or haemorrhagic complications of ET.
Adult↗
The significance of thrombocytosis in old age.
We performed a retrospective study on 21 patients, aged 70 years or more, affected by polycythemia vera (PV) or essential thrombocythemia (ET). As controls, we evaluated 10 younger ET patients. The results indicate that in older ET subjects there was a lower incidence of hemorrhagic and thrombotic complications than in younger patients and PV patients. However, platelet number and platelet function tests were similar in all the patients studied. We suppose that an increase in hematocrit as seen in PV is much more dangerous as compared to an isolated increase in platelet count, and that thrombocytosis alone in old age can be an isolated expression of a natural involution of blood marrow similar to myelofibrosis.
Adult↗
[Hemorrhagic thrombocytosis treated with triethyliminobenzoquinone].
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Adult↗
Therapeutic thrombocytapheresis: a review of 132 patients.
Therapeutic thrombocytapheresis has been used is an attempt to obtain a rapid cytoreduction in thrombocythemic patients, in order to prevent or reduce thrombosis or bleeding complications. Long-term cytoreduction has to be performed with chemotherapy. We have treated with thrombocytapheresis 132 patients with increased platelet count (76 with thrombosis and/or bleeding) due to splenectomy, essential thrombocythemia and other myeloproliferative disorders: a comparative study has been performed to find a platelet count that could be helpful in preventing thrombotic and hemorrhagic complications and the role of diagnosis in predicting patient's risk.
Humans↗
[A clinical and biological study of 11 cases of essential thrombocythemia].
11 patients diagnosed as suffering from essential thrombocytosis at "Hospital Xeral de Lugo" between 1978 and 1986, are presented. 7 patients were female and 4 were male with a median age of 63 years. 6 patients had thrombotic manifestation, 6 had hemorrhagic symptoms, 2 had both manifestations and only one had no thrombo-hemorrhagic clinical symptoms. Spleen enlargement was found in 6 cases and liver enlargement in 5. The median count of platelets were 1.585.000 ml; there was no relationship between the amount and their function with the major or minor complications incidence. There was a good response to treatment with hydroxyurea and/or busulfan associated to platelets aggregation inhibitor in some cases. 4 patients died, all of them due to thrombotic complications.
Adult↗
Essential thrombocythemia. Clinical characteristics and course of 61 cases.
Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.
Bone Marrow↗
Essential thrombocythemia associated with recurrent abortions and fetal growth retardation.
Essential thrombocythemia (ET) is one of the myeloproliferative disorders characterized by an elevated platelet count, usually greater than 1,000 X 10(9)/L. It may be associated with either hemorrhagic or thrombotic tendencies. Although it usually affects older people, we have recently encountered two young women with ET who had a total of six spontaneous abortions before the end of the first trimester. During her most recent pregnancy, one of the patients underwent plateletpheresis on four separate occasions before the 12th week. She reached 34 weeks but demonstrated evidence of fetal growth retardation and pre-eclampsia. At delivery there were scattered villus infarcts in the placenta. Based on this patient and previous reports in the literature, we postulate that the spontaneous abortions, fetal growth retardation, and pre-eclampsia may be related to the thrombocytosis and suggest that the use of antiplatelet agents throughout pregnancy should be seriously considered in such cases.
Abortion, Habitual↗
Occurrence of haemorrhagic and thrombotic events in myeloproliferative disorders: a retrospective study of 108 patients.
Raised platelet count, impaired platelet function, and high PCV values are all considered risk factors for the haemorrhagic and/or thrombotic events frequently complicating the course of myeloproliferative disorders. Nevertheless, the outcome in young patients is reported as being frequently uncomplicated and, in these cases, treatment could be deferred. We examined retrospectively the occurrence of haemorrhagic and/or thrombotic events at the onset of myeloproliferative disorders in 108 patients and found that 67.4% of patients, with either raised platelet count or isolated high PCV, were symptomatic at the onset and the rate was similar regardless of disease, sex, age, platelet number and PCV values. We were unable to locate a patient group with a lower rate of complications and, in light of the frequency of symptoms, it seems reasonable to attempt a normalization of blood values in every case.
Adult↗