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PubMed2001

[Anesthetic management of two patients with essential thrombocythemia].

We report different methods of anesthetic management in two patients with essential thrombocythemia. Case 1 is a 69-year-old male scheduled for cholecystectomy. His blood platelet counts were maintained between 10 to 40 x 10(4).microliters-1 after myelosuppression therapy. His preoperative blood tests were within normal limits. Since he had no signs of hemorrhage or thrombus preoperatively, an epidural catheter was inserted for intraoperative analgesia and postoperative pain relief. Anesthesia was induced with propofol and fentanyl, and maintained with N2O-O2-sevoflurane. Mepivacaine 1% was injected through the epidural catheter for intraoperative analgesia and buprenorphine was injected through the catheter for postoperative pain relief. His perioperative course was uneventful. Case 2 is an 88-year-old female scheduled for emergency enterectomy. She had had recurrent bouts of thrombosis. Her blood platelet counts were 89.1 x 10(4).microliters-1. Since her preoperative management of thrombocythemia had been poor, epidural anesthesia was not performed. Anesthesia was induced with propofol, and maintained with N2O-O2-sevoflurane. Her perioperative course was uneventful. We conclude that spinal or epidural anesthesia is not contraindicated when preoperative platelet counts and aggregation test are within normal limits in a patient with essential thrombocythemia.

Aged↗
PubMed2005

Anagrelide is effective in treating patients with hydroxyurea-resistant thrombocytosis in patients with chronic myeloid leukemia.

We report phase II trial results of the use of oral anagrelide hydrochloride for treating 38 patients with hydroxyurea (HU)-resistant thrombocytosis accompanying chronic myeloid leukemia (CML). Anagrelide's efficacy was well established during a phase II study of more than 400 patients with one of the four myeloproliferative disorders: essential thrombocythemia, polycythemia, idiopathic myelofibrosis, and CML. In the last subgroup, there were 114 CML patients with significant thrombocytosis treated with anagrelide. Out of these patients, 38 had symptoms of thrombosis or hemorrhage and had thrombocytosis resistant to HU. They were then treated with anagrelide at an initial dose of 2.0 mg/day, followed by modifications based upon response and toxicity. In all, 71% of these patients responded with platelet reductions of more than 50% in a median time of approximately 4 weeks. The response rate was not influenced by age, gender, or prior thrombosis or hemorrhage. Importantly, the response rate to anagrelide in patients refractory to prior HU was essentially the same as that of the other 76 CML patients. Treatment with anagrelide was well tolerated and without undue toxicity. Reduction of excessive platelet counts by anagrelide sometimes occurring in CML may lead to the prevention of thrombohemorrhagic complications occurring in this clinical setting and is relevant even in those patients in whom imatinib mesylate is primary therapy.

Adult↗
PubMed1996

Treatment of essential thrombocythemia during pregnancy with interferon-alpha.

BACKGROUND: Only a few cases of essential thrombocythemia in pregnant women have been reported, and the management of this myeloproliferative disorder during pregnancy remains uncertain. We report a successful pregnancy in a patient who had essential thrombocythemia and who was treated with interferon-alpha, and we review the literature for the outcome of similar patients. CASE: A 32-year-old woman, gravida 4, para 3, aborta 0, presented at 18 weeks' gestation with two episodes of amaurosis fugax and an elevated platelet count of 2300 x 10(9)/L. The initiation of interferon-alpha led to a progressive fall of the platelet level, with no occurrence of thrombotic or hemorrhagic manifestations. Serial ultrasound examinations revealed normal fetal and placental development. The patient was delivered of a male infant at 37 weeks. Both child and placenta were normal on examination. CONCLUSION: Our case and the current available data suggest that interferon-alpha may be the best therapeutic option for pregnant patients with essential thrombocythemia in whom myelosuppression is required.

Adult↗
PubMed1997

Aspirin in essential thrombocythemia: status quo and quo vadis.

Aspirin has a well established role in the prevention of arterial thrombosis. Discussion on the efficacy and safety of aspirin in the treatment and prophylaxis of thrombosis in essential thrombocythemia (ET) has become an important issue. The rationale for its use in ET comes from the observation that arterial thrombosis and platelet-mediated microcirculatory disturbances are the major causes of morbidity and mortality in ET. Experimental data have shown persistently elevated levels of thromboxane A2 (TXA2) in ET patients probably reflecting an enhanced in vivo platelet activation. Increased TXA2 biosynthesis and platelet activation in vivo in ET are selectively suppressed by repeated low doses of aspirin. ET-related symptoms such as erythromelalgia, transient neurologic and ocular disturbances are sensitive to aspirin. However, the benefit of low-dose aspirin is still uncertain in the primary prevention of thrombosis in ET. Furthermore, aspirin may unmask a latent bleeding diathesis frequently present in ET which may result in severe hemorrhagic complications. Thus, aspirin is contraindicated in ET patients with a bleeding history or a very high platelet count (> 1500 x 10(9)/L) leading to the acquisition of von Willebrand factor deficiency. If indicated, aspirin is presently used in the widely accepted low-dose regimen of 100 mg daily. However, an optimal effective dose has not yet been established. To further evaluate the efficacy and safety of aspirin in ET, prospective clinical trials are needed.

Abortion, Habitual↗
PubMed1991

Thrombosis and hemorrhage in thrombocytosis: evaluation of a large cohort of patients (357 cases).

We report clinical and laboratory findings in a large cohort of patients with thrombocytosis (357 cases). At the time of study, the patients showed a platelet number greater than 500 x 10(9)/L. The follow-up of patients ranged between 3 and 16 years. 123 patients were affected by polycythemia vera (PV), 97 by essential thrombocythemia (ET), 13 by chronic myeloid leukemia (CML) and 31 by myelofibrosis (MF). In 93 subjects, the thrombocytosis was reactive (ST). We found the highest incidence of thrombosis in PV patients, especially concerning the cerebro-vascular system; thrombosis, but in a lower percentage, was recognized in MF patients. Also, ET patients showed a number of thrombosis in peripheral arteries. Thrombosis of the coronary arteries were quite rare while 25% of MPD subjects showed peripheral vein thrombosis. We take into account that many patients showed thrombocytosis associated to one or more atherosclerotic risk factors. Hemorrhages were present especially in CML and in ET, but were not as frequent as thrombosis. The most common bleeding manifestations affected skin and mucosa. Hemorrhages after surgical procedures were also frequent. Gastro-intestinal bleeding was not strictly related to anti-aggregating therapy and occurred not only in PV but also in ET patients.

Adult↗
PubMed2004

Practice guidelines for the therapy of essential thrombocythemia. A statement from the Italian Society of Hematology, the Italian Society of Experimental Hematology and the Italian Group for Bone Marrow Transplantation.

BACKGROUND AND OBJECTIVES: The Italian Society of Hematology (SIE) and the two affiliated Societies (SIES and GITMO) commissioned a project to develop guidelines for the therapy of essential thrombocythemia (ET) using evidence-based knowledge and consensus formation techniques. DESIGN AND METHODS: Key questions on the optimal management of ET patients were formulated by an Advisory Council (AC) and approved by an Expert Panel (EP) composed of 7 senior hematologists. The AC systematically reviewed the published literature from 1980 to August 2002, and articles were graded according to their internal validity and quality. Using the Delphi technique, the EP was asked to answer the key questions according to the available evidence. From September 2002 to March 2003, four Consensus Conferences were held in accordance with the Nominal Group Technique with the goal of solving residual disagreement on recommendations. RESULTS: The EP provided recommendations on when to start platelet-lowering therapy, the most appropriate platelet-lowering agent, the use of anti-platelet therapy, and the management of women in childbearing age and of pregnant women. INTERPRETATION AND CONCLUSIONS: By using evidence and consensus, recommendations for the treatment of key problems in ET have been issued. Statements are graded according to the strength of the supporting evidence and uncertainty is explicitly declared.

Acute Disease↗
PubMed2006

Coronary artery intervention after cytostatics treatment in unstable angina patient with essential thrombocythemia. A case report and literature review.

Essential thrombocythemia (ET) is a clonal disorder of myeloid stem cells that causes thrombocytosis. As a result, ET can lead to vascular thrombosis and tissue ischemia; the association of coronary artery abnormalities such as myocardial infarction or unstable angina is rare. Here we describe a 45,-year-old male patient with essential thrombocythemia who presented with unstable angina. Elective coronary angiography showed total occlusion of mid right coronary artery and mid left anterior descending coronary artery. ET was confirmed by a bone marrow biopsy; treatment was started with antiplatelet therapy including aspirin and clopidogrel along with cytostatic therapy with hydroxyurea and anagrelide. After the initiation of the treatment, the platelet count decreased to 20 x 10(4)/microL. In addition, percutaneous coronary angioplasty was successfully performed with stent placement at the right coronary artery without hemorrhagic or thrombotic complications.

Angina, Unstable↗
PubMed1992

Relationship of thrombohemorrhagic complications to endothelial cell function in patients with chronic myeloproliferative disorders.

Thrombotic and hemorrhagic disorders are common complications of the myeloproliferative disorders. Endothelial cells release both procoagulant and profibrinolytic factors, which may contribute to these hemorrhagic or thrombotic complications. The pre- and postvenous stasis levels of the procoagulant and profibrinolytic factors produced by endothelial cells were correlated with the occurrence of complications in polycythemia rubra vera (PRV) patients (n = 29) and essential thrombocythemia (ET) patients (n = 17) compared with normal patients (n = 17). Tissue plasminogen activator (tPA) activity, plasminogen activator inhibitor (PAI) activity, von Willebrand's factor (vWF) activity and antigen (vWF:Ag), and factor VIII activity were measured. The resting tPA activity was significantly higher in the two disease groups compared with normal controls, but no difference between the levels of tPA and either complication within the disease groups was observed. Significantly elevated tPA following venous stasis was observed in the patients of both disease groups who had bleeding complications. Significant decreases, compared with the normal group, in both resting and postvenous stasis levels of PAI were observed in the disease groups regardless of complication history. The subjects from both disease groups with thrombotic complications had significantly elevated resting vWF and both resting and postvenous stasis vWF:Ag levels compared with normal controls. The endothelial cell is likely to be responding to abnormal hemostasis rather than being primarily involved in the genesis of a hyper- or hypocoaguable state.

Adult↗
PubMedDate not supplied

Essential thrombocythemia in pregnancy: report of four cases.

We report four pregnancies in four patients with essential thrombocythemia (ET). In two cases pregnancy resulted in spontaneous abortion at 6 and 11 weeks of gestation, respectively. The other two had a full-term, normal delivery; one of them received no therapy during the pregnancy, whereas the other was treated with acetylsalicylic acid from 11 weeks for signs of threatened abortion. No thrombotic or hemorrhagic complications were observed in any of the patients during pregnancy or the post-partum period. We believe that pregnancy in patients with ET represents a risk. Close monitoring is therefore indispensable and antiaggregant therapy might be of benefit.

Adult↗
PubMed1989

[Essential thrombocytosis].

Essential thrombocythemia (ET) belongs to the group of clonal myeloproliferative disorders such as polycythemia vera (PV), chronic myelogenous leukemia (CML), and idiopathic myelofibrosis (MF). This rare disorder, characterized by an important thrombocytosis, includes a mucocutaneous hemorrhagic diathesis and thromboembolic events. Neurologic manifestations are frequent in ET and are due to obstruction of the cerebral microvasculature. Both thrombocytosis and platelet dysfunction can be responsible for the thrombo-hemorrhagic phenomena in ET. First symptoms of ET in our patient was thrombosis of the vertebral artery with a secondary embolic event in the thalamus region although the platelet count was below 600.10(9)/l, the classic diagnostic limit for ET. These data strongly suggest that qualitative platelet abnormalities rather than thrombocytosis are the main cause for thrombo-embolic events in ET.

Cerebral Angiography↗
PubMed1993

Essential thrombocythemia: a relative benign long-term course.

The disease manifestations, response to therapy, and follow-up course of 35 patients with essential thrombocythemia seen in a single center were evaluated. The median age was 53 years (range 17-81). Twelve patients were asymptomatic on presentation and the diagnosis was incidental. Twenty-three patients presented with thrombocytosis-related symptoms, most commonly neurological, microcirculatory, or thromboembolic. In no patient did hemorrhage occur as a presenting symptom. The mean platelet count (+/- SD) at diagnosis was 858 +/- 223 x 10(9)/l and the mean maximal count during the follow-up period was 989 +/- 269 x 10(9)/l. The median follow-up of the entire group was 54 months (range 12-172). Ten of the 12 patients who were asymptomatic on presentation remained so during the observation period, while 10 patients who presented with thrombocytosis-related symptoms became asymptomatic following a single course of treatment with no recurrence of symptoms. Thirty of the 35 patients were treated with busulfan or hydroxyurea or both. Response to cytoreductive therapy was prompt and continuous in most patients. Essential thrombocythemia might be a disease of grave consequence but it seems that in general its course under treatment is more benign than previously thought.

Adolescent↗
PubMed2005

[Diagnosis and treatment of primary thrombocythemia].

Primary thrombocythemia is a frequent myeloproliferative disorder that may be asymptomatic for a long period of time. Once the usual reactive causes have been excluded, further diagnosis requires specialized exploration. The severity level of the disease is not necessarily related to reduce survival rates, but to thrombotic or hemorrhagic complications that can affect quality of life. Myelofibrotic and acute leukemic transformations can also be long-term complications, possibly influenced by treatment strategy. Cytoreductive treatment is required in patients older than 60, or with a history of thrombosis or hemorrhage, or with a platelet count above 1 500 - 109/L. In cases where these indicators do not exist, there are no guidelines for treatment in France, and all investigators try to discriminate patients with a low vascular risk in order to avoid cytoreductive treatment. In fact, in the absence of curative treatment, it seems reasonable to avoid potentially leukemogenic drugs in younger patients.

Acute Disease↗
PubMed1993

[Essential thrombocythemia in pregnancy].

A 30-year-old woman was admitted to our hospital because of thrombocythemia during pregnancy. Her leukocyte count was 10,000/microliters, Hb was 11.7 g/dl, and platelet count 181.9 x 10(4)/microliter. Bone marrow aspirate showed an increase in megakaryocytes (255/microliters). Both Ph1 chromosome and bcr rearrangement were negative. She was diagnosed as having essential thrombocythemia (ET) with pregnancy, and was treated with aspirin (150 mg/day). Her pregnancy was uneventful, but she was readmitted because of overterm pregnancy. A caesarean section was performed, and a healthy male infant weighing 3,672 g was delivered, with a platelet count of 25.5 x 10(4)/microliter. However, the uterine was atonic, and atonic hemorrhage occurred. Supravaginal hysterectomy was performed. Subsequently, intrabdominal gross hemorrhage occurred, but the bleeding was halved by platelet transfusion. Microscopic examination showed uterine infarction. We suggest that platelet count should be reduced by means of plateletpheresis or interferon-alpha throughout pregnancy with ET.

Adult↗
PubMed2004

Hemostatic gene polymorphisms and the prevalence of thrombotic complications in polycythemia vera and essential thrombocythemia.

Patients with polycythemia vera and essential thrombocythemia are at risk for thrombotic and bleeding complications. Currently, no diagnostic test can predict thrombohemorrhagic complications. In a prospective study of 86 patients with polycythemia vera (43 patients) or essential thrombocythemia (43 patients), we examined the possible role of polymorphisms of platelet adhesion receptors [glycoprotein (GP) Ibalpha, GPIa, GPIIIa) and clotting factor II (prothrombin's G20210A mutation) and clotting factor V (Leiden mutation) in determining the risk of thrombotic or bleeding complications. Except for an association between vasomotor symptoms and prothrombin mutation (P < 0.001), no significant correlation between polymorphism of clotting factors and thrombohemorrhagic complications was identified. When the entire patient cohort was considered, the polymorphisms of platelet adhesion receptors were not associated with the risk for thrombotic or bleeding complications. However, among patients with polycythemia vera, the presence of the PlA2 allele of GPIIIa was associated with an increased risk of arterial thrombosis. In view of previous studies linking the presence of the PlA2 allele of GPIIIa to a higher risk for coronary artery thrombosis, our data have physiologic relevance. However, they need to be confirmed in a larger study.

Adult↗