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At least 109 records · Page 6Linked to original sources
PubMed1996

Erythromelalgic, thrombotic and hemorrhagic manifestations in 50 cases of thrombocythemia.

Fifty consecutive patients with thrombocythemia (35 men and 15 women) were diagnosed as primary thrombocythemia (PT) in 30 and thrombocythemia associated with polycythemia vera (PV) in 20. The symptoms were platelet-mediated erythromelalgia in 16 PT and 15 PV, coronary artery disease in 3 PT and 2 PV, atypical cerebral ischemic attacks in 8 PT and 3 PV, paradoxical thrombosis and bleeding in 3 PT and 2 PV and hemorrhages alone in 6 PT and 2 PV patients. Erythromelalgia was localized in the forefoot sole and toes in 28, the fingertips in 9, the handpalm in 2. Untreated erythromelalgia progressed to acrocyanosis or peripheral ischemia with necrosis in a toe or fingertip in 14 cases. Painful red, warm and indurated erythromelalgic hot spots in the skin of the upper legs were misdiagnosed as superficial thrombophelebitis in 5 PT and 2 PV patients. Erythromelalgia in thrombocythemia already occurred at slightly increased platelet counts above 400 x 10(9)/l. The curative effect of aspirin on erythromelalgia in thrombocythemia was consistently accompanied by a significant increase of platelet counts. Erythromelalgia and bleeding paradoxically occurred in 5 patients at platelet counts between 1000 and 2000 x 10(9)/l. In this situation aspirin prevents erythromelalgic and microcirculatory circulation disturbances, but further increases the risk of serious bleeding complications. Presenting hemorrhagic manifestations in thrombocythemia were observed at platelet counts in excess of 1000 x 10(9)/l in 9 PT and 4 PV patients as severe epistaxis in 5, atypical ecchymoses in 3, gastrointestinal bleeding in 2 and secondary bleeding in 3. The concept of platelet-mediated erythromelalgia, thrombosis and hemorrhages in thrombocythemia is discussed.

Adult↗
PubMed1982

Coexistent hereditary haemorrhagic telangiectasia and primary thrombocythaemia--coincidence or syndrome?

Two patients with coexistent hereditary haemorrhagic telangiectasia (HHT) and primary thrombocythaemia (PT) are presented. In one of them, both diseases were detected at the same time. In the other, signs of HHT were present 20 years prior to the diagnosis of PT. Both patients had recurrent gastrointestinal haemorrhage with consequent anaemia, and multiple gastric telangiectases were demonstrated endoscopically. Persistent and severe haemorrhage from gastric telangiectases in one of the patients was successfully controlled by endoscopic monopolar electrocoagulation. The very high platelet counts were reduced with melphalan in one patient and with busulfan in the other. The degree of severity of both disorders was parallel in both cases. We suggest that the coexistence of these two rare disorders in one and the same patient may represent a syndrome that could be more common than hitherto known.

Aged↗
PubMed1985

[Massive hemorrhage following multiple epidural punctures as a late complication in thrombocythemia].

We present a female patient in whom numerous lumbar punctures for epidural anaesthesia were followed by a delayed development of a large subcutaneous haematoma in the lumbar region. The outcome was fatal. The patient's basic disease was found to be chronic myeloic leukaemia that had caused an excessive elevation of platelet count. We, therefore, suggest that lumbar punctures for epidural anaesthesia should be approached with extreme care in cases of thrombocytaemia. If possible, repeated punctures for the placement of epidural catheters should be avoided.

Aged↗
PubMed1993

Fibrinolytic imbalance in essential thrombocythemia: role of platelets.

Thrombotic and hemorrhagic complications are frequent in patients with essential thrombocythemia (ET), a myeloproliferative syndrome with an increased number of circulating platelets. Since platelets are a physiological reservoir for the plasminogen activator inhibitor (PAI-1) contained in plasma, we evaluated plasma and platelet tissue plasminogen activator (tPA) and PAI-1 in 20 ET patients with and without thrombotic complications and in 13 control subjects. In ET patients with thrombotic complications there was a significantly greater platelet PAI-1 functional activity than in ET patients without thrombotic complications and in the control group (p < 0.05 and p < 0.025, respectively). Moreover, platelet tPA activity was significantly low in all ET patients (p < 0.001). This fibrinolytic imbalance (increased plasminogen inhibitor and lowered activator) might be a critical cofactor in the thrombotic complications in ET patients.

Adult↗
PubMed2003

The rate of progression to polycythemia vera or essential thrombocythemia in patients with erythrocytosis or thrombocytosis.

BACKGROUND: The clinical relevance of mild erythrocytosis (hematocrit > 0.48 in women or > 0.51 in men) or thrombocytosis (platelet count > 400 x 10(9) cells/L) in asymptomatic persons is uncertain. OBJECTIVE: To estimate the frequency of polycythemia vera or essential thrombocythemia in persons with erythrocytosis or thrombocytosis in a general population. DESIGN: Cohort study. SETTING: Vicenza, Italy. PARTICIPANTS: 10 000 community dwellers age 18 to 65 years and enrolled in the Vicenza Thrombophilia and Atherosclerosis project. MEASUREMENTS: Platelet count and hematocrit at baseline in all participants and at second follow-up if baseline results were abnormal. Measurement of erythrocyte sedimentation rate, peripheral arterial saturation, serum erythropoietin level, and leukocyte alkaline phosphatase level; chest radiography; abdominal ultrasonography; and occult fecal blood testing were done in persons with confirmed high hematocrit or platelet counts. Polycythemia vera and essential thrombocythemia were diagnosed according to the Polycythemia Study Group criteria. RESULTS: At baseline examination, 1 person had polycythemia vera, 1 had essential thrombocythemia, 88 had erythrocytosis, and 99 had thrombocytosis. Second examination confirmed erythrocytosis in 40% (95% CI, 29% to 51%) and thrombocytosis in 8% (CI, 4% to 15%) of those with abnormal baseline results. Among persons with confirmed abnormalities, further evaluation revealed 11 with idiopathic erythrocytosis, 2 with polycythemia vera (3/10 000 [CI, 0.6 to 8.7/10 000]), and 3 with essential thrombocythemia (4/10 000 [CI, 1.09 to 10.2/10 000]). After 5 years of follow-up, 1 additional person with a high platelet count developed essential thrombocythemia, and no persons developed hemorrhagic or thrombotic complications. CONCLUSIONS: The prevalences of polycythemia vera and essential thrombocythemia were higher than expected in this general population. However, the risks for developing polycythemia vera, essential thrombocythemia, or associated vascular complications in persons with erythrocytosis or thrombocytosis were low.

Adolescent↗
PubMed2002

Therapeutic options for essential thrombocythemia and polycythemia vera.

Several options exist for treating essential thrombocythemia and polycythemia vera. One approach is to assign the patient to a risk category from which treatment recommendations follow. The principal risks of essential thrombocythemia include thrombosis, major hemorrhage, and conversion to leukemia or myelofibrosis. Risk factors for thrombosis include age and prior thrombosis. Smoking and obesity have been implicated in isolated series. High-risk patients with essential thrombocythemia can be defined as those 60 years of age or older or those who have had a thrombosis at any age. These patients should be treated with hydroxyurea. If hydroxyurea cannot be tolerated, anagrelide and interferon-alpha (IFN-alpha) are alternatives. Low-dose aspirin (40 to 325 mg) can be used for patients whose platelet counts are < 1,500 x10(9)/L. Low-risk patients are those less than 60 years old who have not had thrombosis, who have no cardiovascular risk factors, and whose platelet counts are < 1,500 x 10(9)/L. These patients can be observed or placed on low-dose aspirin. Intermediate-risk patients are those less than 60 years who have not had thromboses, but who have platelet counts > 1,500 x 10(9)/L or who have significant cardiovascular risk factors. These patients should have their risk factors treated and may be given low-dose aspirin if the platelet count is < 1,500 x 10(9)/L. They can be observed or treated with anagrelide, hydroxyurea, or IFN-alpha. The Mayo Clinic experience suggests that no specific treatment affects outcomes of pregnancies. In high-risk pregnant women who need treatment, IFN-alpha is used. The principal risks of polycythemia vera are thrombosis, postpolycythemia myeloid metaplasia, and acute leukemia. Risk factors for thrombosis include age, the use of phlebotomies, the rate of phlebotomies, and a prior history of thrombosis. Platelet counts have not been definitively linked to an increased risk of thrombosis. High-risk polycythemia vera patients are those 60 years of age or older (some groups use 70 years) or those of any age who have had thrombosis. They should be treated with phlebotomy and hydroxyurea or IFN-alpha. Selected patients may be treated with anagrelide. A typical target range for phlebotomy is a hematocrit of < 42% for women and < 45% for men. Low-dose aspirin can be used if the platelet count is < 1,500 x 10(9)/L. Low-risk patients are those less than 60 years old who have had no thrombosis, no cardiovascular risk factors, and whose platelets are < 1,500 x 10(9)/L. These patients can be managed with phlebotomy alone or phlebotomy and low-dose aspirin. Intermediate-risk patients are those who are less than 60 years old, who have not had thrombosis, but who have platelet counts > 1,500 x 10(9)/L or who have cardiovascular risk factors. The cardiovascular risk factors should be treated, along with phlebotomy alone or with IFN-alpha. Low-dose aspirin is reasonable for those with platelet counts < 1,500 x 10(9)/mL. Anagrelide can be used with phlebotomy in selected patients. Women of childbearing age who are in the low-risk or intermediate-risk group can be treated with phlebotomy alone and low-dose aspirin if the platelet count is < 1,500 x 10(9)/L. For high-risk patients or pregnant patients, IFN-alpha can be added.

Anti-Inflammatory Agents, Non-Steroidal↗